December 2024 Pulmonary Case of the Month: Two Birds in the Bush Is Better than One in the Hand

Susanna G. Von Essen MD

University of Nebraska Medical Center

Omaha, NE USA

History of Present Illness

A 48-year-old man is referred for dyspnea on exertion and a nonproductive cough. He was well until 6 months prior to this visit. He feels he has had “flu-like symptoms” over the past month.

PMH, SH, and FH

He has had intermittent atrial fibrillation controlled by digoxin but also clopidogrel as an anticoagulant. He has symptoms of hay fever and had asthma as a child.

He has never smoked and rarely drinks. Pets include two dogs and a cat. He is a university English literature professor and his office is an old building but the building is clean and well maintained.  Hobbies include playing guitar in a rock-n-roll band.

His family history is unremarkable.

Physical Examination

His physical examination including  lungs and cardiovascular examination is unremarkable.

Which of the following are indicated for further workup? (Click on the correct answer to be directed to the second of six pages.)

1. Chest X-ray
2. Electrocardiogram (ECG)
3. Pulmonary function testing (PFTs)
4. 1 and 3
5. All of the above

Cite as: VonEssen SG. December 2024 Pulmonary Case of the Month: Two Birds in the Bush Is Better than One in the Hand. Southwest J Pulm Crit Care Sleep. 2024;29(6):53-56. doi: https://doi.org/10.13175/swjpccs035-24 PDF.

June 2022 Pulmonary Case of the Month: A Hard Nut to Crack

Daniel Gergen MD¹

Anne Reihman MD¹

Carolyn Welsh MD^1,2

¹Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Colorado, Aurora, Colorado USA

²Eastern Colorado Veterans Affairs Medical Center, Aurora, Colorado USA

History of Present Illness: A 54-year-old man presented to clinic with chronic cough, dyspnea on exertion, unintentional weight loss, and night sweats. Seven months before, he developed dyspnea on exertion and symptoms did not improve with inhalers. Four months prior to presentation, he was treated for presumed community-acquired pneumonia of the right lower lobe. Neither symptoms nor chest radiograph improved with multiple courses of antibiotics. In the four weeks prior to presentation his symptoms progressed to the point that he was unable to walk in his house without significant dyspnea.

Review of systems: 10-pound unintentional weight loss and six weeks of night sweats.

Past Medical History, Social History and Family History: The patient had a 15-pack-year smoking history and quit 15 years prior to presentation. He had no other past medical history, surgical history, family history, nor medications.

Physical Examination: Vital signs were normal on presentation. Physical exam showed faint wheezing and decreased breath sounds over the right posterior lung fields.

Radiography: Chest radiograph demonstrated dense opacification in the superior segment of the right lower lobe (Figure 1).

Figure 1. Initial chest radiography. A: PA view. B: Lateral view.

What are diagnostic possibilities at this time?

1. Lung abscess
2. Lung cancer
3. Foreign body with post-obstructive pneumonia
4. Tuberculosis
5. 1 and 3
6. All the above

Cite as: Gergen D, Reihman A, Welsh C. June 2022 Pulmonary Case of the Month: A Hard Nut to Crack. Southwest J Pulm Crit Care Sleep. 2022;24(6):89-92. doi: https://doi.org/10.13175/swjpccs024-22 PDF

December 2019 Pulmonary Case of the Month: A 56-Year-Old Woman with Pneumonia

Lewis J. Wesselius, MD

Department of Pulmonary Medicine

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

A 56-year-old woman complained of 6 weeks of increasing cough and shortness of breath. She had been treated for pneumonia with antibiotics, but when she failed to improve, she was begun on prednisone. She was receiving oxygen at 4 L/min by nasal cannula at the time she was seen.

PMH, SH, and FH

Her past medical history, social history and family were unremarkable other than a previous history of silicone breast implants. She was a nonsmoker.

Physical Examination

Her physical examination showed bibasilar crackles but was otherwise unremarkable.

Radiography

Her chest x-ray is shown in Figure 1.

Figure 1. Patient’s chest x-ray taken 6 weeks after the beginning of her illness.

Which of the following should be done at this time? (Click on the correct answer to be directed to the second of seven pages)

1. Coccidioidomycosis serology
2. Sputum gram stain and culture
3. Thoracic CT scan
4. 1 and 3
5. All of the above

Cite as: Wesselius LJ. December 2019 Pulmonary case of the month: a 56-year-old woman with pneumonia. Southwest J Pulm Crit Care. 2019;19(6):149-55. doi: https://doi.org/10.13175/swjpcc067-19 PDF

December 2018 Pulmonary Case of the Month: A Young Man with Multiple Lung Masses

Lewis J. Wesselius, MD

Department of Pulmonary Medicine

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

A 28-year-old man from Tennessee has been feeling ill with malaise and weight loss for the past 3 months. He had been in the in the Palm Springs area a few weeks prior to becoming ill. He works as a musician.

Past Medical History, Social History and Family History

He has a history of Wolf-Parkinson-White syndrome and had a prior ablation procedure at age 16. He does not smoke tobacco but does smoke marijuana occasionally. Family history is noncontributory.

Physical Examination

Physical examination was unremarkable.

Which of the following are indicated at this time? (Click on the correct answer to be directed to the second of eight pages)

1. Bronchoscopy with EBUS
2. Chest X-ray
3. VATS
4. 1 and 3
5. All of the above

Cite as: Wesselius LJ. December 2018 pulmonary case of the month: a young man with multiple lung masses. Southwest J Pulm Crit Care. 2018;17(6):138-45. doi: https://doi.org/10.13175/swjpcc118-18 PDF 

August 2018 Pulmonary Case of the Month

Arooj Kayani, MD

Richard Sue, MD

Banner University Medical Center Phoenix

Phoenix, AZ USA

Pulmonary Case of the Month CME Information

Completion of an evaluation form is required to receive credit and a link is provided on the last page of the activity. 

0.25 AMA PRA Category 1 Credit(s)™

Estimated time to complete this activity: 0.25 hours

Lead Author(s): Arooj Kayani, MD. All Faculty, CME Planning Committee Members, and the CME Office Reviewers have disclosed that they do not have any relevant financial relationships with commercial interests that would constitute a conflict of interest concerning this CME activity.

Learning Objectives: As a result of completing this activity, participants will be better able to:

1. Interpret and identify clinical practices supported by the highest quality available evidence.
2. Establish the optimal evaluation leading to a correct diagnosis for patients with pulmonary, critical care and sleep disorders.
3. Translate the most current clinical information into the delivery of high quality care for patients.
4. Integrate new treatment options for patients with pulmonary, critical care and sleep related disorders.

Learning Format: Case-based, interactive online course, including mandatory assessment questions (number of questions varies by case). Please also read the Technical Requirements.

CME Sponsor: University of Arizona College of Medicine at Banner University Medical Center Tucson

Current Approval Period: January 1, 2017-December 31, 201

Financial Support Received: None

History of Present Illness

A 59-year-old woman referred because of worsening dyspnea over the past 2 months along with cough and wheezing. She has a history of chronic obstructive pulmonary disease (COPD) and is on continuous oxygen @ 2 L/min.

PMH, SH, and FH

In addition to her COPD she has a history of hypothyroidism, pneumonia, tonsillectomy, hip lipoma resection, hysterectomy, and a herniorrhaphy. She has a 30 pack-year history of smoking. She currently smokes half pack/day. No family history of lung disease or cancer.

Medications

• Fluticasone/salmeterol
• Tiotropium
• Albuterol
• Levothyroxine

Physical Examination

• Vitals: HR 79/min, BP 100/69 mmHg, RR 16/min, SpO2 92% on 2 L/min.
• General: Alert and oriented. Healthy appearing in no distress.
• Lungs: Expiratory stridor and expiratory wheezing loudest over left lung. No crackles.
• Cardiac: Regular rhythm with no murmurs. No edema.
• The remainder of physical examination was unremarkable.

Which of the following should be performed? (Click on the correct answer to proceed to the second of four pages)

1. Spirometry
2. Sputum Gram stain, AFB stain, and fungal stain with cultures
3. Thoracic CT scan
4. 1 and 3
5. All of the above

Cite as: Kayani A, Sue R. August 2018 pulmonary case of the month. Southwest J Pulm Crit Care. 2018;17(2):47-52. doi: https://doi.org/10.13175/swjpcc093-18 PDF 

June 2018 Pulmonary Case of the Month

Lewis J. Wesselius, MD

Department of Pulmonary Medicine

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

The patient is a 53-year-old man who presented in January 2018 for a second opinion on interstitial lung disease first diagnosed in 2011. He lives in Los Angeles and had one year of increasing dyspnea on exertion prior to diagnosis. He had an outside surgical lung biopsy and was treated with prednisone, then started on azathioprine and the prednisone tapered. He was followed regularly and had limited progression over next 7 years.  However, recently he had increasing shortness of breath.

Past Medical History, Social History, Family History

He has no significant past medical history. He is a nonsmoker and denies any significant occupational exposures.

Physical Examination

Physical examination was unremarkable without rales or clubbing.

Which of the following should be obtained at this time? (Click on the correct answer to proceed to the second of five pages)

1. Prior chest x-rays, CT scans, pulmonary function testing and lung biopsy
2. Repeat CT scan, pulmonary function testing
3. Rheumatological serologies
4. 1 and 3
5. All of the above

Cite as: Wesselius LJ. June 2018 pulmonary case of the month. Southwest J Pulm Crit Care. 2018;16(6):296-303. doi: https://doi.org/10.13175/swjpcc063-18 PDF 

February 2018 Pulmonary Case of the Month

Lewis J. Wesselius, MD

Department of Pulmonary Medicine

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

A 75-year-old woman was diagnosed with a thymic carcinoid tumor in April, 2015 (Figure 1).

Figure 1. Representative image from the preoperative CT scan performed in April 2015 showing an anterior mediastinal mass (arrow).

This was treated with surgical resection followed by radiation therapy.

She began having cough and dyspnea 1 to 2 months later and in August, 2015 had a thoracic CT scan of her chest (Figure 2).

Figure 2. Representative image in lung windows from the second thoracic CT scan performed in August 2015.

Which of the following are true? (Click on the correct answer to proceed to the second of six pages)

1. Bronchoscopy should be performed
2. She should be given an empiric course of antibiotics
3. The most like diagnosis is radiation pneumonitis
4. 1 and 3
5. All of the above

Cite as: Wesselius LJ. February 2018 pulmonary case of the month. Southwest J Pulm Crit Care. 2018;16(2):55-61. doi: https://doi.org/10.13175/swjpcc020-18 PDF 

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia in a Patient with Multiple Pulmonary Nodules: Case Report and Literature Review

Hasan S. Yamin, MD¹

Feras Hawarri, MD¹

Mutaz Labib, MD¹

Ehab Massad, MD²

Hussam Haddad, MD³

Departments of ¹Internal Medicine Pulmonary & Critical Care Division, ²Thoracic Surgery and ³Pathology

King Hussein Cancer Center

Amman, Jordan

Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disease, where carcinoid tumorlets invade the pulmonary parenchyma and bronchioles. These nests of cells release a variety of mediators including bombesin and gastrin releasing peptide that cause heterogeneous bronchoconstriction, creating a mosaic appearance on chest imaging studies, especially on expiratory scans. Clinically patients usually have long standing symptoms of shortness of breath (SOB) and cough that are difficult to distinguish from asthma. In this article we describe a case of DIPNECH in a patient with several years’ history of SOB and cough, and review 179 cases of DIPNECH reported in the literature since 1992.

Case Presentation

A 72-year-old, non-smoking lady was admitted to the hospital in preparation for bilateral mastectomy. She recently received a diagnosis of bilateral breast invasive ductal carcinoma grade 2, estrogen receptor/progesterone receptor/human epidermal growth factor receptor 2 (HER-2) positive in the left tumor but negative in the right tumor.

Her past medical history was significant for hypertension, long standing cough and dyspnea on exertion labeled as asthma poorly responsive to nebulizers. Socially, she was a house wife with no history of occupational exposure.

The patient was found to be tachypneic (respiratory rate 22 breaths/minute) and hypoxemic (oxygen saturation 86% on room air). Heart rate and blood pressure were within normal limits. She had bilateral decreased breath sounds and diffuse expiratory wheezes.

Chest CT scan revealed diffuse mosaic pattern and multiple pulmonary nodules in both lungs suggestive of metastases (Figure 1).

Figure 1. Representative images form chest CT scan showing a diffuse mosaic pattern and multiple pulmonary nodules in both lung fields suggestive of metastases.

These lesions did not take up fludeoxyglucose (FDG) on positron emission tomography (PET) scan. Her pulmonary function tests (PFT) were unremarkable except for reduction in expiratory reserve volume (ERV) at 22%, and increased residual volume to total lung capacity ratio (RV/TLC) at 136% probably related to air trapping. Diffusion lung capacity was within normal limits.

Video assisted thoracoscopic biopsy of one of the nodules in left lower lobe was done. Pathology showed both a carcinoid tumor and tumorlets invading lung bronchioles (Figure 2A & B) and these tumorlets were positive for chromogranin (Figure 2C & D) and pancytokeratin (Figure 2 E & F).

Figure 2. A & B: histology (H&E stain) showing carcinoid tumorlets invading lung bronchioles; C & D: positive staining for chromogranin; E &F: positive staining for pancytokeratin.

A diagnosis of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) was made, and the patient was treated with intravenous steroids and nebulizers. Her oxygen saturation improved to 94% on room air. She was later discharged on oral steroids. Her CT scan also showed no significant improvement in changes described above.

Review of the Literature

Methods

We searched PubMed for all cases of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia reported in the English literature since 1992 when the entity was first described. A total of 179 patients were identified in 55 articles, in the form of case reports and case series. In this article we contribute an additional patient (1-55).

Patient Characteristics

A total of 180 patients (including our patient) were identified. There were 161 females (89.5%) and only 19 males (10.5%). Mean age at diagnosis was 57.75 years (males tended to present at a younger age of 52 years, compared to 58.4 years in females). Most patients were never smokers 52.8%, smokers/exsmokers 27.2%, and in 20% smoking status was not mentioned.

The majority of patients presented with cough (91 patients, 50.5%), followed by exertional dyspnea (81 patients, 45%), and hemoptysis (6 patients, 3.3%). Incidental imaging findings led to diagnosis in 22 patients (12.2%). Mean duration of symptoms before diagnosis was 8.25 years (Table 1).

Table 1. Patients` characteristics and presenting symptoms.

Diagnosis, Therapy and Outcome

Most patients underwent imaging with chest CT scan, the most common findings were nodules in 148 patients (82.2%), ground glass opacities/mosaic pattern in 66 patients (36.6%), and bronchial wall thickening in 37 patients (20.5%). Most patients had an abnormal spirometry: obstructive pattern (48.9%), restrictive (5%), or mixed obstructive restrictive pattern (6.7%) (Table 2).

Table 2. Spirometry and imaging.

Because of their symptoms, and spirometry findings 45 patients (25%) were labeled with another disease including asthma in 29 patients (16.1%), COPD in 12 patients (6.6%) and bronchiolitis in 4 patients (2.2%).

The diagnosis was made using surgical lung biopsy in 148 patients (82.2%), bronchoscopic biopsy in 10 patients (8 transbronchial biopsy, 2 endobronchial biopsy) (5.6%), CT-guided biopsy in 7 patients (3.9%), postmortem diagnosis in 3 patients (1.7%), post lung transplantation in 2 patients (1.1%) and clinically in 2 patients (1.1%). The diagnostic method was not mentioned in 8 patients (4.4%).

Patients received a variety of therapies including inhaled bronchodilators, inhaled or systemic steroids, and somatostatin analogues among others. Response to treatment was mentioned for 89 patients, (59 patients reported that their symptoms remained stable, 11 patients improved with treatment, while 18 patients reported symptom progression and 2 patients died. (Table 3).

Table 3. List of DIPNECH articles ordered by publication year. This table shows number of patients in each article, diagnostic method, therapy given and outcome.

Of note, 15 out of 23 patients who received a somatostatin analogue reported stable, or improvement in their symptoms (65.2%), which did not necessarily translate into improvement in air flows on spirometry (27, 29, 46, 51).

Discussion

Pulmonary neuroendocrine cell hyperplasia was described early in the previous century (56), however the significance and role of the pathologic changes were not precisely determined. It was thought that they were secondary to other lung diseases such as interstitial lung disease, bronchiectasis, cystic fibrosis, smoking exposure, or in people who live at high altitude. In addition to the previously mentioned associations, hyperplasia of pulmonary neuroendocrine cells was also thought to be a pre-neoplastic process, since the lesions can potentially progress to carcinoid tumors even without causing symptoms or airflow limitation. In 2004 the changes were recognized by WHO as one end of the spectrum of pulmonary neuroendocrine tumors.

The relationship between carcinoid tumorlets and other pulmonary diseases and its role in precipitating respiratory symptoms remains puzzling. The term DIPNECH was coined in 1992 by Aguayo (1) who described a new entity where idiopathic hyperplasia or dysplasia of pulmonary neuroendocrine cells occurred in the absence of other lung disorders. The changes were associated with physiologic and radiologic airflow limitation similar to obliterative bronchiolitis. This was the first description of pulmonary neuroendocrine hyperplasia as a primary process.

Because of similar symptoms, an obstructive pattern on pulmonary function tests, and chest imaging suggestive of air trapping, many patients receive a diagnosis of asthma for several years before the correct diagnosis is made. This similarity to other obstructive lung diseases can be explained by the pathologic changes of airway obstruction seen on biopsy. Pulmonary neuroendocrine cells, or Kulchitsky cells, are normally present in small numbers in airways, where they release a myriad of bioactive amines and peptides like serotonin, chromogranin A, gastrin-releasing peptide (GRP), and calcitonin.

Airway obstruction is believed to occur both due to physical obstruction of bronchioles by tumorlets and smooth muscle constriction caused by active mediators released. Bombesin and related peptides like gastrin releasing peptide, neuromedin B and neuromedin C are thought to cause bronchoconstriction indirectly through the release of several other bronchoconstrictors that act on smooth muscle cells (57). However, in vitro studies in guinea pig lungs suggest that bombesin may act directly by binding to specific receptors on smooth muscle cells (58).

Pulmonary neuroendocrine pathology occurs in a spectrum of three forms: hyperplasia, tumorlets and carcinoid tumors. DIPNECH is characterized by proliferation of neuroendocrine cells initially limited to the basement membrane of airways, when disease extends beyond the lumen of airway it is called carcinoid tumorlets. Tumorlets larger than 0.5 cm become carcinoid tumors and appear as nodules on chest CT scans. Diagnosis requires lung biopsy, with a surgical biopsy procedure more likely to provide diagnostic tissue than bronchoscopic transbronchial biopsies.

According to Aguayo`s definition of DIPNECH, patients have pulmonary symptoms with radiographic and physiologic abnormalities suggestive of obstructive lung disease, but in our review 12.2% of patients had no symptoms at all, and 15.5% had normal spirometry. We believe hyperplasia, tumorlets and carcinoid tumors represent different aspects of the same disease, the occurrence of symptoms, radiologic and physiologic airflow limitation depends on the time frame at which diagnosis was made, should those patients be followed up, they could develop symptoms and airflow limitation in the future. Thus, we propose to expand the definition to include patients with no symptoms or spirometry abnormalities. However, it remains uncertain whether asymptomatic patients who are diagnosed at an earlier stage need specific treatment or not.

It is also clinically difficult to establish a causal relationship, or determine the direction of the relationship between pulmonary neuroendocrine cell hyperplasia and other concomitant lung disorders, or harmful exposures (1,59, 60). In our review 27.2% of patients were active or previous smokers, only one patient lived at high altitude (more than 2000m) (14), 29 patients had a history of previous or current malignancy including 8 lung cancers (not shown in table), 13 patients had evidence of bronchiectasis, and one patient had honeycombing on imaging. These findings are similar to data obtained from individual case reports and series (2, 6, 14, 17, 19, 22, 29, 33, 37, 46, 47 and 53).

When the diagnosis is made, therapeutic options may include observation for mild symptoms, inhaled or systemic steroids, in addition to bronchodilators, especially if patients who show reversible airway obstruction on PFT. Other potential therapies are somatostatin analogues, however more studies are needed to determine their precise role. (27, 29, 43, 46)

Conclusion

DIPNECH is a rare clinical entity that requires a high clinical suspicion. Because of clinical, spirometry, and imaging similarity to other obstructive lung diseases, and the requirement for lung biopsy to make the diagnosis, DIPNECH is probably an under-diagnosed entity, with still limited treatment options. The diagnosis should probably be considered in any patient with difficult to treat obstructive lung disease, unexplained bronchiolitis, particularly if there are multiple small lung nodules present on chest CT scan. We propose to expand the definition of DIPNECH to include patients with even no symptoms or spirometric evidence of airflow limitation, as development of these abnormalities depends on the time frame at which diagnosis is made. It is also difficult to establish a causal relationship with other concomitant lung conditions, the presence of which should not rule out a diagnosis of DIPNECH.

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40. Erdini F, Spaltro AA, Ruiu A, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) and multiple pulmonary epithelioid hemangioendothelioma (PEH): a case report. Pathologica. 2015 Mar;107(1):37-42. [PubMed]
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42. Pietrangeli V, Piciucchi S, Tomassetti S, et al. Diffuse neuroendocrine hyperplasia with obliterative bronchiolitis and usual interstitial pneumonia: an unusual "headcheese pattern" with nodules. Lung. 2015 Dec;193(6):1051-4. [CrossRef] [PubMed]
43. Chauhan A, Ramirez RA. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) and the role of somatostatin analogs: a case series. Lung. 2015 Oct;193(5):653-7. [CrossRef] [PubMed]
44. Hassan WA, Udaka N, Ueda A, et al. Neoplastic lesions in CADASIL syndrome: report of an autopsied Japanese case. Int J Clin Exp Pathol. 2015 Jun 1;8(6):7533-9. [PubMed]
45. Ofikwu G, Mani VR, Rajabalan A, et al. Case report: a rare case of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Case Rep Surg. 2015;2015:318175. [CrossRef] [PubMed]
46. Carr LL, Chung JH, Duarte Achcar R, et al. The clinical course of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Chest. 2015 Feb;147(2):415-2. [CrossRef] [PubMed]
47. Baniak NM, Wilde B, Kanthan R. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)--An uncommon precursor of a common cancer? Pathol Res Pract. 2016 Feb;212(2):125-9. [CrossRef] [PubMed]
48. Kuruva M, Shah HR, Dunn AL, et al. 111In-Pentetreotide imaging in diffuse idiopathic neuroendocrine hyperplasia of the lung. Clin Nucl Med. 2016 Mar;41(3):239-40. [CrossRef] [PubMed]
49. Cansız Ersöz C, Cangır AK, Dizbay Sak S. Diffuse neuroendocrine cell hyperplasia: report of two cases. Case Rep Pathol. 2016;2016:3419725. [CrossRef] [PubMed]
50. Ishizaki U, Itoh R, Matsuo Y, et al. Diffuse Idiopathic Pulmonary neuroendocrine cell hyperplasia: a case with long-term follow-up. J Thorac Imaging. 2016 Nov;31(6):W76-W79. [CrossRef] [PubMed]
51. Chatterjee K, Kamimoto JJ, Dunn A, et al. A case of DIPNECH presenting as usual interstitial pneumonia. Pneumonol Alergol Pol 2016;84(3):174-7. [CrossRef] [PubMed]
52. Escudero AG, Zarco ER, Arjona JC. Expression of developing neural transcription factors in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). Virchows Arch. 2016 Sep;469(3):357-63. [CrossRef] [PubMed]
53. Trisolini R, Valentini I, Tinelli C, et al. DIPNECH: association between histopathology and clinical presentation. Lung. 2016 Apr;194(2):243-7. [CrossRef] [PubMed]
54. Warren WA, Dalane SS, Warren BD, et al. Ten years of chronic cough in a 64-year-old man with multiple pulmonary nodules. Chest. 2016; 150(3):e81-e85. [CrossRef] [PubMed]
55. Hsu J, Jia L, Pucar D, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and granulomatous inflammation mimicking high-grade malignancy on FDG-PET/CT. Clin Nucl Med. 2017 Jan;42(1):47-49. [CrossRef] [PubMed]
56. Frölich F. Die Helle Zelle der Bronchialschleimhaut und ihre Beziehungen zum Problem der Chemoreceptoren. Frankf Z Pathol. 1949 Apr;60(3-4):517-59.
57. Barnes PJ. Pharmacology of airway smooth muscle. Am J Respir Crit Care Med. 1998 Nov;158(5 Pt 3):S123-32. [CrossRef] [PubMed]
58. Lach E, Haddad EB, Gies JP. Contractile effect of bombesin on guinea pig lung in vitro: involvement of gastrin-releasing peptide-preferring receptors. Am J Physiol. 1993 Jan; 264(1 Pt 1):L80-6. [PubMed]
59. Campanha RB, Matos C, Nogueira F. DIPNECH (diffuse idiopathic pulmonary neuroendocrine cell hyperplasia): a rare syndrome or undiagnosed? J Pulm Respir Med 2015;5:4. [CrossRef]
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Cite as: Yamin HS, Hawarri F, Labib M, Massad E, Haddad H. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia in a patient with multiple pulmonary nodules: case report and literature review. Southwest J Pulm Crit Care. 2017;15(6):282-93. doi: https://doi.org/10.13175/swjcc139-17 PDF

December 2017 Pulmonary Case of the Month

Lewis J. Wesselius, MD¹

Michael B. Gotway, MD²

Departments of ¹Pulmonary Medicine and ²Radiology

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

A 52-year-old woman from Iowa sought a second opinion for a left hilar mass. She travels to Phoenix regularly to visit family. She began feeling ill in late 2016 with cough and sputum production and was treated with multiple courses of antibiotics without improvement.

PMH, SH and FH

Past medical history is unremarkable. She is a nonsmoker. FH is noncontributory.

Physical Examination

Physical examination was normal.

Radiography

In March of this year she had chest radiograph in Phoenix which suggested left hilar adenopathy. A thoracic CT scan was performed (Figure 1).

Figure 1. Representative images from the thoracic CT scan in lung windows (A-E) and soft tissue windows (F).

Which of the following are diagnostic considerations? (Click on the correct answer to procced to the second of seven pages)

1. Lung cancer  
2. Lymphoma
3. Sarcoidosis
4. Tuberculosis
5. All of the above

Cite as: Wesselius LJ, Gotway MB. December 2017 pulmonary case of the month. Southwest J Pulm Crit Care. 2017;15(6):232-40. doi: https://doi.org/10.13175/swjpcc144-17 PDF

September 2017 Pulmonary Case of the Month

Lewis J. Wesselius, MD

Department of Pulmonary Medicine

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

A 67-year-old woman with history of chronic lymphocytic leukemia (CLL) was referred due to a 6-week history severe cough. Her CLL had recently relapsed and she was begun on ibrutinib (a small molecule drug that binds permanently to Bruton's tyrosine kinase) in addition to acyclovir, sulfamethoxazole/trimethoprim and allopurinol.

Past Medical History, Social History and Family History

Her CLL was initially diagnosed in 2009 and had responded to fludarabine, cyclophosphamide, and rituximab. She had no other chronic medical diseases. She smoked ½ pack per day but quit with the development of her cough. Family history was noncontributory.

Physical Examination

Her vital signs were unremarkable and she was afebrile but did cough frequently during the examination. There were shoddy small lymph nodes noted in both supraclavicular and axillary areas. Lungs were clear and the rest of the physical examination was unremarkable.

Laboratory Evaluation

Her complete blood count revealed her to be mildly anemic with a hemoglobin of 9.0 g/dL, an elevated white count of 33,700 cells/mcL with 88% lymphocytes, and a low platelet count of 60,000 cells/mcL. Her electrolytes were within normal limits and her blood urea nitrogen was 20 mg/dL, creatinine 1.1 mg/dL and uric acid 7.1 mg/dL.

Chest Radiography

A chest x-ray was performed (Figure 1).

Figure 1. Initial chest x-ray.

Which of the following is true? (Click on the correct answer to proceed to the second of five pages)

1. A pulmonary nodule is present in the left upper lobe (LUL)
2. Ibrutinib is well known to cause a chronic cough
3. Pneumonia is unlikely since she is afebrile
4. 1 and 3
5. All of the above

Cite as: Wesselius LJ. September 2017 pulmonary case of the month. Southwest J Pulm Crit Care. 2017;15(3):94-9. doi: https://doi.org/10.13175/swjpcc108-17 PDF

March 2017 Pulmonary Case of the Month

Maxwell L. Smith, MD 

Department of Laboratory Medicine and Pathology

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

The patient is 52-year-old man who complained of dyspnea on exertion and a dry cough.

Past Medical History, Social History and Family History

He had a history of gastroesophageal reflux disease (GERD) and was taking a proton pump inhibitor.

He never smoked and had no known exposures.

Family history was noncontributory.

Physical Examination

Physical Examination was unremarkable.

Chest X-ray

A chest x-ray was reported as normal.

Which of the following are indicated? (Click on the correct answer to proceed to the second of five pages)

1. Chest CT scan
2. Endoscopy/bronchoscopy
3. Pulmonary function testing
4. 1 and 3
5. All of the above 

Cite as: Smith ML. March 2017 pulmonary case of the month. Southwest J Pulm Crit Care. 2017;14(3):89-93. doi: https://doi.org/10.13175/swjpcc014-17 PDF

July 2016 Pulmonary Case of the Month

Kashif Yaqub, MD

Robert Viggiano, MD

Imran S. Malik, MD

Zayn A. Mian

Department of Pulmonary Medicine

Mayo Clinic Arizona

Scottsdale, AZ USA

Pulmonary Case of the Month CME Information

Members of the Arizona, New Mexico, Colorado and California Thoracic Societies and the Mayo Clinic are able to receive 0.25 AMA PRA Category 1 Credits™ for each case they complete. Completion of an evaluation form is required to receive credit and a link is provided on the last panel of the activity. 

0.25 AMA PRA Category 1 Credit(s)™

Estimated time to complete this activity: 0.25 hours

Lead Author(s): Kashif Yaqub, MD.  All Faculty, CME Planning Committee Members, and the CME Office Reviewers have disclosed that they do not have any relevant financial relationships with commercial interests that would constitute a conflict of interest concerning this CME activity.

Learning Objectives:
As a result of this activity I will be better able to:

1. Correctly interpret and identify clinical practices supported by the highest quality available evidence.
2. Will be better able to establsh the optimal evaluation leading to a correct diagnosis for patients with pulmonary, critical care and sleep disorders.
3. Will improve the translation of the most current clinical information into the delivery of high quality care for patients.
4. Will integrate new treatment options in discussing available treatment alternatives for patients with pulmonary, critical care and sleep related disorders.

Learning Format: Case-based, interactive online course, including mandatory assessment questions (number of questions varies by case). Please also read the Technical Requirements.

CME Sponsor: University of Arizona College of Medicine at Banner University Medical Center Tucson

Current Approval Period: January 1, 2015-December 31, 2016

Financial Support Received: None

History of Present Illness

A 53 year-old woman presented to the emergency department with dyspnea over 3 weeks. There was no cough, wheezing or other complaints.

Past Medical History, Social History and Family History

She has no significant past medical history. She was a nonsmoker. Family history was unremarkable.

Physical Examination

Decreased breath sounds over the left lower chest but otherwise unremarkable.

Laboratory Evaluation

• Elevated white blood cell count with a left shift
• Na+ 130 mEq/L
• 10-20 RBCs on urinalysis

Radiographic Evaluation

A CT angiogram of the chest was performed for possible pulmonary embolus (Figure 1).

Figure 1. Representative images from the thoracic CT in lung windows (A) and soft tissue windows (B).

Which of the following is appropriate at this time? (Click on the correct answer to proceed to the second of six panels)

1. Biopsy of left pleural mass
2. Bone marrow aspiration
3. Diuretics for congestive heart failure
4. Empiric antibiotics for empyema
5. Thoracentesis

Cite as: Yaqub K, Viggiano R, Malik IS, Mian AZ. July 2016 pulmonary case of the month. Southwest J Pulm Crit Care. 2016;13(1):1-8. doi: http://dx.doi.org/10.13175/swjpcc051-16 PDF

May 2016 Pulmonary Case of the Month

Jennifer M. Hall, DO

Banner University Medical Center Phoenix

Phoenix, AZ USA

Pulmonary Case of the Month CME Information

Members of the Arizona, New Mexico, Colorado and California Thoracic Societies and the Mayo Clinic are able to receive 0.25 AMA PRA Category 1 Credits™ for each case they complete. Completion of an evaluation form is required to receive credit and a link is provided on the last panel of the activity. 

0.25 AMA PRA Category 1 Credit(s)™

Estimated time to complete this activity: 0.25 hours

Lead Author(s): Jennifer M. Hall, DO.  All Faculty, CME Planning Committee Members, and the CME Office Reviewers have disclosed that they do not have any relevant financial relationships with commercial interests that would constitute a conflict of interest concerning this CME activity.

Learning Objectives:
As a result of this activity I will be better able to:

1. Correctly interpret and identify clinical practices supported by the highest quality available evidence.
2. Will be better able to establsh the optimal evaluation leading to a correct diagnosis for patients with pulmonary, critical care and sleep disorders.
3. Will improve the translation of the most current clinical information into the delivery of high quality care for patients.
4. Will integrate new treatment options in discussing available treatment alternatives for patients with pulmonary, critical care and sleep related disorders.

Learning Format: Case-based, interactive online course, including mandatory assessment questions (number of questions varies by case). Please also read the Technical Requirements.

CME Sponsor: University of Arizona College of Medicine at Banner University Medical Center Tucson

Current Approval Period: January 1, 2015-December 31, 2016

Financial Support Received: None

History of Present Illness

A 24-year-old woman was diagnosed with pneumonia while on her honeymoon in Europe. She received an unknown treatment as an outpatient. When she returned a repeat chest x-ray showed persistent lung infiltrates. At that time she was asymptomatic. She was referred to pulmonary for further evaluation.

Past Medical History, Family History, Social History

• Idiopathic thrombocytopenic purpura at age 8
• Recurrent “bronchitis” since childhood
• Lifelong non-smoker, occasional ETOH, no illicit drugs
• No significant family history, other than hypertension in her father

Physical Examination

She had bibasilar fine crackles (fine) otherwise her physical examination was unremarkable.

Radiography

A chest x-ray was performed and interpreted as showing bilateral basilar interstitial infiltrates (Figure 1).

Figure 1. Chest x-ray showing bibasilar interstitial infiltrates.

To better define the abnormalities on chest x-ray a thoracic CT scan was performed (Figure 2).

Figure 2. Representative images from the thoracic CT scan in lung windows.

Based on the CT scan, which of the following diagnosis is least likely? (Click on the correct answer to proceed to the second of five panels)

1. Hematogenous metastasis
2. Hypersensitivity pneumonitis
3. Lymphangitic metastasis
4. Miliary tuberculosis
5. Sarcoidosis

Cite as: Hall JM. May 2016 pulmonary case of the month. Southwest J Pulm Crit Care. 2016 May;12(5):165-70. doi: http://dx.doi.org/10.13175/swjpcc037-16 PDF 

March 2016 Pulmonary Case of the Month

Ramachandra R. Sista, MD

Maxwell L. Smith, MD

 Lewis J. Wesselius, MD

Departments of Pulmonary Medicine and Pathology

Mayo Clinic Arizona

Scottsdale, AZ

Pulmonary Case of the Month CME Information

Members of the Arizona, New Mexico, Colorado and California Thoracic Societies and the Mayo Clinic are able to receive 0.25 AMA PRA Category 1 Credits™ for each case they complete. Completion of an evaluation form is required to receive credit and a link is provided on the last panel of the activity. 

0.25 AMA PRA Category 1 Credit(s)™

Estimated time to complete this activity: 0.25 hours

Lead Author(s): Ramachandra R. Sista, MD. All Faculty, CME Planning Committee Members, and the CME Office Reviewers have disclosed that they do not have any relevant financial relationships with commercial interests that would constitute a conflict of interest concerning this CME activity.

Learning Objectives:
As a result of this activity I will be better able to:

1. Correctly interpret and identify clinical practices supported by the highest quality available evidence.
2. Will be better able to establsh the optimal evaluation leading to a correct diagnosis for patients with pulmonary, critical care and sleep disorders.
3. Will improve the translation of the most current clinical information into the delivery of high quality care for patients.
4. Will integrate new treatment options in discussing available treatment alternatives for patients with pulmonary, critical care and sleep related disorders.

Learning Format: Case-based, interactive online course, including mandatory assessment questions (number of questions varies by case). Please also read the Technical Requirements.

CME Sponsor: University of Arizona College of Medicine at Banner University Medical Center Tucson

Current Approval Period: January 1, 2015-December 31, 2016

Financial Support Received: None

History of Present Illness

A 74-year-old man was referred for a recently identified right pleural effusion and dyspnea on exertion.  

Past Medical History, Family History and Social History

He has a history of anemia, hypertension, and prostate cancer with a prostatectomy in 2015. He is a life-long nonsmoker and has no occupational exposures. Family history is noncontributory.

Physical Examination

He had diminished breath sounds at the right lung base and a palpable spleen. Otherwise the physical examination was unremarkable.

Laboratory

CBC: hemoglobin  8.5 g/dL, white blood count  7.7 X 109 cells/L,  platelets 357 X 109 cells/L.

Radiography

A chest X-ray showed a right pleural effusion. Representative images from the CT scan are shown in Figure 1.

Figure 1. Representative images from the CT scan.

Which of the following is the most likely diagnosis? (Click on the correct answer to proceed to the second of five panels)

1. Empyema
2. Lung cancer
3. Tuberculosis
4. Usual interstitial pneumonia
5. Valley fever (coccidioidomycosis)

Cite as: Sista RR, Smith ML, Wesselius LJ. March 2016 pulmonary case of the month. Southwest J Pulm Crit Care. 2016;12(3):74-80. doi: http://dx.doi.org/10.13175/swjpcc020-16 PDF

Why Chronic Constipation May be Harmful to Your Lungs: A Case Report and Review of Lipoid Pneumonia and Mycobacterium fortuitum Leading to Acute Respiratory Failure and Septic Shock

Sandra Till, DO

Manoj Mathew, MD 

Da-Wei Liao, MD

Christina Ramirez, MD 

Banner University Medical Center

Phoenix, AZ

Case Report

A 43 year-old female with a past medical history of right-sided hemiparesis secondary to motor vehicle accident 17 years prior presented a two week history of cough, fever and right-sided pleuritic chest pain. Her baseline status included using a wheelchair, living alone at home and working as a teacher.

On admission she had a temperature of 39.6º C, was tachycardia and hypotensive requiring vasopressors. Labs were remarkable for a white count of 25,000 cells/mcL. Chest x-ray showed right-sided infiltrate and pleural effusion (Figure 1).

Figure 1. Chest x-ray on presentation.

Bronchoscopy and thoracentesis was performed upon admission. The pleural fluid wasexudative with a glucose of 78 and no suggestion of loculations on chest x-ray or ultrasound. The patient was started on therapy for community-acquired pneumonia.

On day 4 after admission, the patient had increasing sinus tachycardia, hypotension and was worsening despite being on antimicrobial therapy. A CT angiogram of the chest was performed (Figure 2).

Figure 2. Initial CT scan on day 4 of admission. Panel A: axial view showing pneumonia and right pleural effusion. Panel B: coronal view.

CT angiogram was negative for pulmonary embolism and a percutaneous chest tube was placed on day 4 for drainage of pleural effusion due to development of loculations. On day 7, the pleural fluid from initial thoracentesis grew acid-fast bacteria identified as Mycobacterium fortuitum.

Bronchoscopy was performed on day 8 and there was no endobronchial obstruction.

Bronchoscopic alveolar lavage cultures grew Mycobacterium fortuitum. She had no history of bronchiectasis, skin infection, or immunoglobulin deficiency. Treatment with amikacin and levofloxacin was initiated based on susceptibilities.

The pleural chest tube was removed on day 14 (Figure 3). At this time the patient was transferred to a skilled nursing facility.

Figure 3. CT scan on day 13 prior to chest tube removal. Panel A: axial view. Panel B: coronal view.

The patient continued antibiotic treatment for Mycobacterium fortuitum with amikacin and levofloxacin, however, serial sputum cultures remained positive. On day 25, in the skilled nursing facility, the patient developed respiratory failure due to increased right effusion and worsening pneumonia. She was transferred to our facility were she was intubated and a new right-sided chest tube was placed. After placement of chest tube and drainage the right lung did not expand. Decompensation was felt to be related to the inadequate evacuation of the empyema with plans to solely continue antimicrobial therapies by the outside facility.

Figure 4. CT scan on day 30 showing trapped lung. Panel A: axial view. Panel B: coronal view. 

Repeat pleural fluid cultures and BAL once again grew Mycobacterium fortuitum. She was taken for decortication and right middle and lower lobe resection by thoracic surgery. Due to extensive disease the patient required right thoracotomy, decortication, parietal pleurectomy, right middle lobectomy, and wedge resection of a right lower lobe lung abscess.

The lung pathology is shown below and was consistent with lipoid pneumonia (Figure 5).

Figure 5. Panels A & B: CD 163 stains showing lipid present within histiocytes. Panels C & D: histology demonstrating severe lipoid pneumonia. Panels E & F: Granulomatous inflammation with giant cells. Panel G: pleura. Panel H: abscess.

There were no mycobacteria cultured on the lung biopsy. There were areas of both acute and chronic fibrosis noted on pathology report along with areas of acute interstitial pneumonitis and granulomatous inflammation.

During post-operative phase the patient confirmed that she was drinking mineral oil chronically for treatment of constipation. Repeat sputum cultures 7 days post operatively were negative for Mycobacterium fortuitum. She continued to improve with treatment of Mycobacterium fortuitum and postoperative cultures remained negative. She was able to liberate from the ventilator and returned home at after a prolonged course of rehabilitation.

Lipoid Pneumonia and Associated Mycobacterial Infection

The association between acid-fast bacteria and lipoid pneumonia was first reported in 1925 and since case reports have been noted. In 1953, a case report and literature review documented six cases of “saprophytic” mycobacteria was noted in conjunction with lipoid pneumonia. It was observed at this time that the fatty environment of lipoid pneumonia might assist with the growth of mycobacterium (1). Since then, intermittent case reports have been published reporting lipoid pneumonia with atypical mycobacteria.

There are two main categories of lipoid pneumonia, endogenous and exogenous. The endogenous form is also known as cholesterol pneumonia or golden pneumonia. It is associated with lysis of lung tissue distal to obstruction due to malignancy, fat storage disease such as Neiman-Pick or Gaucher's, medications and therapies including chemotherapeutic agents, amiodarone and radiation therapy. Pulmonary alveolar proteinosis has also been reported in idiopathic cases with granulomatosis with polyangiitis and connective tissue diseases (2-4). In polarized light microscopy after staining with sulfuric and acetic acid, the sample reveals cholesterol crystals, which is diagnostic of endogenous lipoid pneumonia (3).

Exogenous lipoid pneumonia occurs when external substances enter the lungs due to inhalation or aspiration (3). Cases have been reported from mineral oil, paraffin use, oil based nasal drops, total parenteral nutrition, mineral oil nose drops, black fat tobacco smoking, milk, and liquid hydrocarbons used by flame blowers (2-6). The pulmonary reaction to each substance varies. For example, mineral oils are fairly inert and less likely to produce alveolar inflammation, where milk fats are hydrolyzed by lung lipases leading to a significant inflammatory response (2).

The clinical presentation and appearance of lipoid pneumonia is variable from consolidation to effusion to nodule. Nodules from lipids may have elevated standardized uptake value (SUV) on positron emission tomography (PET) scan. The BAL from lipoid pneumonia may demonstrate lipid laden foamy macrophages (2). Mineral oil granuloma (paraffinoma) also can present as a spiculated mass mimicking malignancy.

Mineral oil is notorious for causing lipoid pneumonia by aspiration for several reasons. First, it floats on the column of undigested material in the esophagus so it is first to be aspirated (5); secondly, it impairs phagocytosis at the alveolar level; and lastly, it inhibits the cough reflex and motor function of ciliated mucosa (7).

The impairment of phagocytosis associated with lipoid pneumonia is thought to be a contributing factor in why atypical mycobacterium strives in the lipid rich environment of lipoid pneumonia (5,6). Malnutrition is also thought to be a component of risk as it due to impairment in cell mediated immunity (6). Lipid acts as mechanical protection for the mycobacteria favoring tissue necrosis facilitating secondary infection. Also it is thought that lipids may activate the cell walls of the atypical mycobacteria leading to increased virulence of the mycolic acids within the wall of the bacteria (8).

Mycobacterium fortuitum rarely causes pulmonary disease unless associated with lipoid pneumonia. This is often related to gastroesophageal disease and chronic vomiting and aspiration of contents. It is typically associated with skin and soft tissue infections and is a rapid growing mycobacterium and most frequently found in water and soil (2,8,9)

This case demonstrates an atypical presentation of lipoid pneumonia and Mycobacterium fortuitum infection leading to septic shock and ventilator failure. Although the association of lipoid pneumonia and mycobacterial infections is well documented, the rapid and acute decline in this patient’s clinical status is unusual. This can be attributed to incomplete drainage of the initial empyema prior to transfer to the skilled nursing facility.

The etiology of the lipoid pneumonia was chronic aspiration of mineral oil producing an ideal environment for growth of Mycobacterium fortuitum. The absence of bronchiectasis, immunoglobin deficiency, skin infections should prompt further evaluation for abnormal lung architecture serving as a nidus for Mycobacterium fortuitum Infection. In our case, failure to improve is attributed to a persistent nidus for infection. We advocate resection of diseased lung segments of lipoid pneumonia to facilitate successful treatment of Mycobacterium fortuitum. In conclusion, if a patient has lipoid pneumonia with signs of clinical infection, the possibility of rapidly growing mycobacterium such as M. fortuitum should be considered.

References

1. Gibson JB. Infection of the lungs by saprophytic mycobacteria in achalasia of the cardia, with report of a fatal case showing lipoid pneumonia due to milk. J Pathol Bacteriol. 1953;65(1):239-51. [CrossRef] [PubMed]
2. Hasan A, Swamy T. Nocardia and Mycobacterium fortuitum infection in a case of lipoid pneumonia. Respiratory Medicine CME 2011: 75-78. [CrossRef]
3. Betancourt SL, Martinez-Jimenez S, Rossi SE, Truong MT, Carrillo J, Erasmus JJ. Lipoid pneumonia: spectrum of clinical and radiologic manifestations. AJR Am J Roentgenol. 2010;194(1):103-9. [CrossRef] [PubMed]
4. Harris K, Chalhoub M, Maroun R, Abi-Fadel F, Zhao F. Lipoid pneumonia: a challenging diagnosis. Heart Lung. 2011;40(6):580-4. [CrossRef] [PubMed]
5. Hughes RL, Freilich RA, Bytell DE, Craig RM, Moran JM. Clinical conference in pulmonary disease. Aspiration and occult esophageal disorders. Chest. 1981;80(4):489-95. [CrossRef] [PubMed]
6. Tranovich VL, Buesching WJ, Becker WJ. Pathologic quiz case. Chronic pneumonia after gastrectomy. Pathologic diagnosis: chronic aspiration lipoid pneumonia with Mycobacterium abscessus. Arch Pathol Lab Med. 2001;125(7):976-8. [PubMed]
7. Jouannic I, Desrues B, Léna H, Quinquenel ML, Donnio PY, Delaval P. Exogenous lipoid pneumonia complicated by Mycobacterium fortuitum and Aspergillus fumigatus infections. Eur Respir J. 1996;9(1):172-4. [Pubmed]
8. Couto SS, Artacho CA. Mycobacterium fortuitum pneumonia in a cat and the role of lipid in the pathogenesis of atypical mycobacterial infections. Vet Pathol. 2007;44(4):543-6. [CrossRef] [PubMed]
9. Vadakekalam J, Ward MJ. Mycobacterium fortuitum lung abscess treated with ciprofloxacin. Thorax. 1991;46(10):737-8. [CrossRef] [PubMed] 

Cite as: Till S, Mathew M, Liao D-W, Ramirez C. Why chronic constipation may be harmful to your lungs: a case report and review of lipoid pneumonia and mycobacterium fortuitum leading to acute respiratory failure and septic shock. Southwest J Pulm Crit Care. 2015;11(4):193-9. doi: http://dx.doi.org/10.13175/swjpcc118-15 PDF 

October 2015 Pulmonary Case of the Month: I've Heard of Katy Perry

Kathryn E. Williams, MB

Maxwell L. Smith, MD

Philip J. Lyng, MD

Laszlo T. Vaszar, MD

 

Department of Pulmonary Medicine

Mayo Clinic Arizona

Scottsdale, AZ

History of Present Illness

A 45-year-old man with a history of dyslipidemia and a family history of early coronary artery disease (CAD) underwent coronary artery calcium scoring CT. He was a non-smoker and asymptomatic.

Past Medical History

In addition to his hyperlipidemia he has a history of obesity and impaired fasting glucose.

Physical Examination

His physical examination was unremarkable.

Radiography

The thoracic CT was interpreted as a low risk for CAD but there were incidental findings (Figure 1).

Figure 1. Panels A-C: Representative views from the thoracic CT scan in lung windows. Lower panel: video of thoracic CT in lung windows.

What incidental finding is not shown on thoracic CT scan. (Click on the correct answer to proceed to the second of six panels).

1. Honeycombing
2. Multiple small pulmonary nodules
3. Patchy ground glass opacities
4. Slightly enlarged mediastinal lymph nodes

Cite as: Williams KE, Smith ML, Lyng PJ, Vaszar LT. October 2015 pulmonary case of the month: I've heard of Katy Perry. Southwest J Pulm Crit Care. 2015;11(4):126-35. doi: http://dx.doi.org/10.13175/swjpcc123-15 PDF

August 2015 Pulmonary Case of the Month: Holy Sheep

Jennifer M. Hall, DO

David M. Baratz, MD

Banner University Medical Center Phoenix

Phoenix, AZ

History of Present Illness

A 42-year-old woman presented to the emergency department with chest pain and dyspnea. The onset of symptoms was acute, initially endorsing left-sided sharp chest pain which then progressed with dyspnea. Chest radiograph was read as normal. Laboratory evaluation was notable for an elevated D-Dimer which prompted a thoracic CT scan to be obtained.

Past Medical History, Family History, Social History

• She had well-controlled rheumatoid arthritis (on no medical therapy) and was diagnosed with emphysema by her PCP two years earlier.
• Her mother died from pulmonary embolism secondary to underlying lung cancer.
• She quit smoking 2 years ago with a total of 20-pack-years.

Physical Examination

Patient was in mild distress with heart rate of 105, respiratory rate of 22, but otherwise stable, SpO2 was 95% while breathing ambient air. She had diminished breath sounds in both bases, but otherwise her chest was clear to auscultation. The remainder of the exam was unremarkable.

Radiography 

A chest x-ray (Figure 1) and a thoracic CT scan (Figure 2) were performed.

Figure 1. Initial PA of the chest.

Figure 2. Thoracic CT scan in lung windows. Panels A-F: representative static images. Lower panel: video.

A chest tube was placed for the left-sided pneumothorax.

What is the next step in management? (Click on the correct answer to proceed to the second of five panels)

1. Bronchoscopy
2. Consider pleurodesis
3. Observation with repeat CT in 3 months
4. Open lung biopsy
5. 1 and 3
6. 2 and 4

Reference as: Hall JM, Baratz DM. August 2015 pulmonary case of the month: holy sheep. Southwest J Pulm Crit Care. 2015;11(2):53-8. doi: http://dx.doi.org/10.13175/swjpcc103-15 PDF

July 2015 Pulmonary Case of the Month: A Crazy Case

Lewis J. Wesselius, MD

Department of Pulmonary Medicine

Mayo Clinic Arizona

Scottsdale, AZ

History of Present Illness

A 23-year-old woman presented in 2008 at outside institution with dyspnea and diffuse pulmonary infiltrates. She required intubation. After a surgical lung biopsy, she was transferred to the Mayo Clinic Hospital for further care.

Past Medical History

She has had a history of progressive dyspnea for several months, otherwise negative 

Physical Examination

Vital signs are stable. SpO2 94% on FiO2 of 0.4. She is intubated and there is a chest tube in her right chest. Otherwise the physical examination is unremarkable.

Radiography

A thoracic CT scan was performed (Figure 1).

Figure 1. Representative images from the thoracic CT in lung windows.

Which of the following are present on the thoracic CT scan? (click on the correct answer to proceed to the second of five panels)

1. Diffuse ground-glass opacities
2. Interlobular septal thickening and intralobular reticular thickening
3. Right-sided pneumothorax
4. 1 and 3
5. All of the above

Reference as: Wesselius LJ. July 2015 pulmonary case of the month: a crazy case. Southwest J Pulm Crit Care. 2015;11(1):3-10. doi: http://dx.doi.org/10.13175/swjpcc079-15 PDF

Systemic Lupus Erythematosus Presenting As Cryptogenic Organizing Pneumonia: Case Report

Anthony Jedd, MD

Sashank Kolli, MD 

Thomas Liao, MD

Isabel Oliva, MD

Loyola University Of Chicago

Stritch School Of Medicine

Maywood, IL

and

University of Arizona

Tucson, AZ

Introduction

Systemic Lupus Erythematosus (SLE) is a systemic disease with multiorgan involvement. In the respiratory system, SLE can involve the lung parenchyma and pleura with intrathoracic manifestations of pleuritis, alveolar hemorrhage and pulmonary fibrosis. Cryptogenic organizing pneumonia (COP) is a rare complication of SLE. We describe a case of newly diagnosed lupus presenting as COP.

Case Report

An 18-year-old woman with no significant past medical history presented to the Emergency Department complaining of generalized malaise, cough and fever for 4 days. Her cough was productive with white to brownish sputum. She complained of chest heaviness/pressure with inability to take deep breaths. Her only reported sick contact was her mother who had the “flu” one week prior to the onset of her illness. She denied any illicit drug use, alcohol or smoking, as well as any recent travel or exotic pet exposure. On admission, her vital signs were: temperature 38.4°C, pulse rate 129 bpm, blood pressure 159/108 mmHg, respiratory rate 29 rpm and oxygen saturation 94% on room air. On physical exam, the patient was in moderate distress with tenderness to palpation over her muscles. Left anterior cervical adenopathy was present and lung auscultation revealed coarse bilateral crackles. She was alert and oriented times 3 with no neurological deficits. 

Laboratory data on admission: white blood cell count 5100/µL with 43% bands, hemoglobin 10.6 g/dL, platelets 125 x 10⁹/L, blood urea nitrogen 37mg/dL, and creatinine 2.0 mg/dL. Urinalysis revealed a large amount of microscopic blood, small amount of leukocyte esterase, moderate bacteria and protein > 300 mg/dl. ABG of pH 7.39/pCO2 28 mm Hg/pO2 of 68 mm Hg on 2L/min by nasal cannula. Initial chest radiograph (CXR) demonstrated bilateral perihilar infiltrates (Figure 1).   

  

Figure 1. CXR on admission.

She was started on ceftriaxone and azithromycin with a working diagnosis of sepsis secondary to community-acquired pneumonia with impending respiratory failure.

She continued to be febrile, hypoxic and tachycardic. A 4-day follow-up CXR demonstrated interval worsening of bilateral airspace disease (Figure 2).

Figure 2. CXR on hospital day 4.

Despite antibiotic therapy for 5 days she was intubated due to continued deterioration. Diagnostic bronchoscopy was performed showing a positive mycoplasma IgM, while samples for bacterial, viral and fungal sources as well blood cultures were negative. GMS stain for Pneumocystis was also negative. At this time infectious disease recommended switching the antibiotics to vancomycin and aztreonam.  

The patient continued to have persistent anemia, thrombocytopenia and urine containing large amounts of protein raising suspicion for an autoimmune hemolytic anemia with bone marrow failure. Rheumatologic panel revealed ANA titer of 2,560 (normal <40), anti-ds DNA antibody >300 IU/ml (normal <10) and normal complement C3,C4. HIV testing was non reactive. Per rheumatology's recommendations, she was started on methylprednisolone 1 g daily for 3 days and later switched to prednisone 100 mg daily and 600 mg cytoxan for the working diagnosis of systemic lupus erythematosus (SLE). After about 1 week of persistent extensive bilateral lung infiltrates and continued ventilator dependence, an open lung biopsy was performed which demonstrated bronchoalveolar tissue showing organizing pneumonia of unknown etiology (Figure 3). No histological findings for vasculitis or alveolar hemorrhage were identified.

 

Figure 3. Panel A: patchy fibroblastic plugs in bronchioles and alveolar ducts (bronchiolitis obliterans) (black arrows). Panel B: Organizing pneumonia within alveoli (black circle).

She was diagnosed with SLE involving multiple organs, which included lung, kidneys and bone marrow. Prednisone 100 mg daily was continued for 2 weeks. Broad spectrum antibiotics were discontinued, but she finished a 2 week coarse of azithromycin for the positive mycoplasma antibodies. Her respiratory status gradually improved and she was extubated on her 14th hospital day. As an outpatient, prednisone was tapered slowly over the next 2 months to 10 mg daily and then she was transitioned to mycophenolate. Follow-up CXR showed resolution of the airspace disease (Figure 4).

Figure 4. CXR 2 months after admission to ICU.

Discussion

Cryptogenic organizing pneumonia is a noninfectious inflammatory pulmonary process that leads to the formation of fibromyxoid connective tissue plugs that adhere to the walls of the alveolar ducts and alveoli (1,2,4). COP can be idiopathic or secondary to several etiologies, including drug toxicity, infection, connective tissue diseases (CTD), malignancy and bone marrow transplantation (6). The diagnosis of SLE-related COP is rare with no cohort studies showing a dominant type of CTD resulting in COP.

Oymak S et al. (8) reviewed etiologic and clinical features in 26 patients with COP and found that 58% were idiopathic. The other 42% were secondary, but the causes were not described. Yoo JW et al. (9) further compared cryptogenic organizing pneumonia and connective tissue disease-related organizing pneumonia (CTD-OP). The study showed rheumatoid arthritis, Sjogren’s syndrome and polymyositis/dermatomyositis were predominant types of CTD and no patients were mentioned with SLE (9). Other studies mention polymyalgia rheumatica and SLE as potential causes of COP, but there are no reported cases of the two entities presenting together in adults (10-12). 

The mechanism by which SLE can lead to the development of COP is unknown. Otsuka et al. (13) suggested that elevated antiphospholipid antibodies contribute to the development of Masson bodies, macrophages and fibrin found within pulmonary alveoli, due to an inhibited inflammatory repair mechanism within the airways, which may contribute to the development of COP. The hypothesis of epithelial damage by the immune system is supported by the response to steroid therapy, which prevents and/or resolves deposition of IgM, IgG and infiltration of plasma cells into the bronchiolar walls (14,15).

The development of SLE-related COP remains a rare entity. Our patient’s presentation was unique in that COP was the initial manifestation of her SLE. Additionally, the pattern of airspace disease on chest radiograph was atypical for organizing pneumonia, which usually presents as either peripheral or peribronchiolar areas of consolidation. As more cases arise, our understanding of the mechanism and timing of the disease will hopefully become more apparent.

References

1. Epler GR, Colby TV, McLoud TC, Carrigton CB, Gaensler EA. Bronchiolitis obliterans organizing pneumonia. N Engl J Med 1985;312:152-8. [CrossRef] [PubMed]
2. Colby TV. Pathologic aspects of bronchiolitis obliterans organizing pneumonia. Chest 1992;102:38S-43S. [CrossRef] [PubMed]
3. Epler GR. Bronchiolitis obliterans organizing pneumonia. Arch Intern Med. 2001;161:158-64. [CrossRef] [PubMed]
4. Moore SL. Bronchiolitis obliterans organzining pneumonia: a late complication of stem cell transplantation. Clin J Oncol Nurs. 2003;7(6):659-62. [CrossRef] [PubMed]
5. Cordier JF. Organizing pneumonia. Thorax 2000;55:318-28. [CrossRef] [PubMed]
6. Roberton B, Hansell D. Organizing pneumonia: a Kaleidoscope of concepts and morphologies. Eur Radiol 2011;21:2244-54. [CrossRef] [PubMed]
7. Takada H, Saito Y, Nomura A, Ohga S, Kuwano K, Nakashima N, Aishima S, Tsuru N, Hara T. Bronchiolitis obliterans organizing pneumonia as an initial manifestation in systemic lupus erythematosus. Pediatr Pulmonol. 2005;40:257-260. [CrossRef] [PubMed]
8. Oymak S, Demirbas HM. Mavili E, Akgun H, Gulmex I, Demir R, Ozesmi M. Bronchiolitis obliterans organizing pneumonia. Respiration. 2005;72:254-62. [CrossRef]  [PubMed]
9. Yoo JW, Song JW, Jang SJ, Lee CK, Kim MY, Lee HK, Jegal Y, Kim DS. Comparison between cryptogenic organizing pneumonia and connective tissue disease-related organizing pneumonia. Rheumatology 2011;50:932-8. [CrossRef] [PubMed]
10. Douglas WW, Tazelaar HD, Hartman TE, Hartman RP, Decker PA, Schroeder DR, Ryu JH. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med. 2001;164(7):1182-5. [CrossRef] [PubMed]
11. Katzenstein ALA, Myers J, Prophet WD, Corley LS 3rd, Shin MS. Bronchiolitis obliterans and usual interstitial pneumonia. Am J Surg Pathol. 1986;10:373-81. [CrossRef] [PubMed]
12. Lynch D. Lung disease related to collagen vascular disease. J Thorac Imaging. 2009;24(4):299-309. [CrossRef] [PubMed]
13. Otsuka F, Amano T, Hashimoto N, Takahashi M, Hayakawa N, Makino H, OtaZ, Ogura T. Bronchiolitis obliterans organizing pneumonia associated with systemic lupus erythematosus with antiphopholipid antibody. Intern Med. 1996;35:341-4. [CrossRef] [PubMed]
14. Myers JL, Katzenstein AL. Ultrastructural evidence of alveolar epithelial injury in idiopathic bronchiolitis obliterans-organizing pneumonia. Am J Pathol. 1988;132(1):102-9. [PubMed]
15. Ippolito JA, Palmer L, Spector S, Kane PB, Goveric PD. Bronchiolitis obliterans organizing pneumonia and rheumatoid arthritis. Semin Arthritis Rheum. 1993;23(1):70-8. [CrossRef] [PubMed]

Reference as: Jedd A, Kolli S, Liao T, Oliva I. Systemic lupus erythematosus presenting as cryptogenic organizing pneumonia: case report. Southwest J Pulm Crit Care. 2015;10(2):87-92. doi: http://dx.doi.org/10.13175/swjpcc164-14 PDF 

August 2014 Pulmonary Case of the Month: A Physician's Job is Never Done

Elijah Poulos, MD^*

Kristine Saunders, MD^†

Pulmonary and Critical Care Medicine*

Department of Pathology^†

Phoenix VA Medical Center

Phoenix, AZ

History of Present Illness

A 75-year-old man presented with recurrent minimally productive cough, dyspnea, fatigue, low-grade fevers, and weight loss in November 2013. The patient had been treated twice as an outpatient with antibiotics in the previous 6 weeks for pneumonia.

PMH, FH, SH

The patient has a history of obstructive sleep apnea but is not compliant with his prescribed continuous positive airway pressure. He also as a history of obesity, dyslipidemia, and peripheral vascular disease.

There is no significant family history.  

He is a retired brick layer with a 50 pack-year smoking history but quit a few weeks prior to admission.  He drinks a case of beer/week.

Physical Examination

VS stable. There were no significant findings on physical examination.

Radiography

A chest radiograph (Figure 1) was performed.

Figure 1. Admission PA (Panel A) and lateral (Panel B) chest radiograph.

What should be done next? (Click on the correct answer to proceed to the next panel)

1. Bronchoscopy with bronchoalveolar lavage
2. Bronchoscopy with transbronchial biopsy
3. Needle biopsy
4. Thoracentesis
5. Video-assisted thorascopic surgery (VATS)

Reference as: Poulos E, Saunders K. August 2014 pulmonary case of the month: a physician's job is never done. Southwest J Pulm Crit Care. 2014;9(2):59-67. doi: http://dx.doi.org/10.13175/swjpcc098-14 PDF