December 2022 Pulmonary Case of the Month: New Therapy for Mediastinal Disease

Lewis J. Wesselius MD

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

A 43-year-old woman complained of persistent cough over 1 year with mild increasing dyspnea on exertion. She denied fever, sweats or weight loss. She had noted fatigue and dry cough, as well as shortness of breath, particularly when supine.

Past Medical History (PMH), Social History (SH), Family History (FH)

• An outside bronchoscopy done in 2019 with washings and biopsy showing only some non-specific inflammation
• Life-long nonsmoker
• Not on any chronic medications
• Had only lived in Arizona, although has travelled in other states
• There is no significant family history

Physical Examination

• Prominent vascularity on anterior chest

What should be done at this time? (Click on the correct answer to be directed to the 2nd of 6 pages)

1. Chest X-ray
2. Obtain old x-rays
3. Pulmonary function testing
4. Serology for coccidioidomycosis
5. All of the above

Cite as: Wesselius LJ. December 2022 Pulmonary Case of the Month: New Therapy for Mediastinal Disease. 2022;25(6):92-96. doi: https://doi.org/10.13175/swjpccs054-22 PDF

Symptomatic Improvement in Cicatricial Pemphigoid of the Trachea Achieved with Laser Ablation Bronchoscopy

Elizabeth Benge MD¹, Vincent Tran MD², Nazanin Sheikhan MD¹, Sapna Bhatia MD³, Yi McWhorter DO⁴, John Collier MD³, Arnold Chung MD⁵

Departments of ¹Internal Medicine, ²Surgery, ³Pulmonology, ⁴Anesthesiology/Critical Care Medicine, and ⁵MountainView Cardiovascular and Thoracic Surgery Associates

HCA Healthcare MountainView Hospital 

Las Vegas, NV, USA 

Abstract

Cicatricial pemphigoid (CP) with tracheal involvement is a rare and potentially deadly condition. Here, we report the first case in which Nd:YAG laser (1064nm) laser ablation bronchoscopy was used to treat CP with tracheal involvement. Our patient is a 71-year-old male with a history of CP refractory to medical therapy affecting his trachea who presented to the emergency department with dyspnea. He ultimately underwent bronchoscopy with Nd: YAG laser (1064nm) laser ablation, which resulted in a temporary alleviation of his respiratory symptoms. A repeat laser ablation was planned in hopes of prolonging the patient’s remission, but due to interval changes in the patient’s airway anatomy, it was deemed unsafe. While our patient’s uniquely advanced disease was not amenable to further laser-mediated intervention, it is possible that patients with less advanced disease may experience better outcomes with similar therapy. This case shows the promise laser ablation could hold for patients with tracheal cicatricial pemphigoid.

Introduction

Cicatricial pemphigoid (CP) is a diverse group of subepithelial blistering disorders of the skin and mucous membranes (1,2). Tracheal involvement is a rare and deadly sequela of this disease class (3). We report the first case in which Nd:YAG laser (1064nm) laser ablation bronchoscopy was used as a treatment for CP with tracheal involvement. Of note, the terms cicatricial pemphigoid and mucous membrane pemphigoid are synonymous and are used interchangeably throughout this report.

Case Presentation

Our patient is a 71-year-old man with a history of CP affecting his left eye and trachea who presented to the emergency department with progressively worsening dyspnea.

The patient has a history of multiple bronchoscopies; the most recent one showed tracheal pemphigoid lesions partially obstructing his airway. His diagnosis of cicatricial pemphigoid had been made over fifteen years prior to the current presentation via biopsy and subsequent immunofluorescence staining. On admission, his respiratory rate was 21 breaths/min and his oxygen saturation was 97% on 50% Bipap: 14/8. He was admitted to the intensive care unit for evaluation and management of his acute hypoxic respiratory failure.

Initially, a fiberoptic bronchoscopy was performed under laryngeal mask airway (LMA) general anesthesia. Dense, dark-colored lesions were noted to be occluding most of the trachea, consistent with the patient’s history of tracheal CP (Figure 1).

Figure 1. Patient’s trachea demonstrating heavy burden of cicatricial pemphigoid lesions prior to any intervention

They were partially removed in a piecemeal manner with forceps instrumentation. After this procedure, the patient still required supplemental oxygen, oscillating between BiPAP and nasal cannula. Two days later, he was started on rituximab, which he had also received during previous relapses.

On hospital day four, our cardiothoracic surgery team performed bronchoscopy with laser ablation under LMA general anesthesia. After the procedure, the patient’s tracheal lesions had markedly decreased in size (Figure 2).

Figure 2. Patient’s trachea with reduced lesions status-post bronchoscopy with laser ablation.

He was also entirely weaned off supplemental oxygen.

In the following weeks, the patient’s symptom burden was significantly decreased. He reported an improvement in his quality of life and satisfaction with the procedure. A subsequent repeat laser ablation was planned at the three-month mark. This procedure was more technically challenging due to airway-narrowing caused by an increase in scar tissue from the initial laser ablation. Due to the risks imposed by the interval changes in the patient’s anatomy, we decided against further laser therapy. In the absence of laser treatments, the patient’s tracheal pemphigoid recurred and symptoms returned to their prior state. He currently receives interval fiberoptic bronchoscopies to partially remove his lesions when they threaten his airway.

Discussion 

In a study involving subjects with aggressive ocular CP, 81% of patients achieved clinical remission with rituximab therapy (4). Medical therapy had repeatedly failed to reduce our patient’s symptoms, making his case unique in both its rarity and refractory nature. With no other options, our team developed an innovative treatment modality in an attempt to offer our patient some symptomatic relief.

Previous case reports have shown the utility of low-level laser therapy in mucous membranous lesions (5-7). One study showed successful resection of an obstructive mass caused by CP and restoration of airway patency using a Holmium LASER (2100nm) (8-9). We decided to ablate/resect the inflammatory tissue using an Nd:YAG LASER (1064nm) given its medium penetration length (1-4mm), coagulopathic ability (high heme absorption), and decreased tissue destruction when compared to the Ho:YAG laser; which has a higher laser absorption coefficient with water.

To our knowledge, this is the first case report of successful treatment of cicatricial pemphigoid with Nd:YAG laser (1064nm) ablation therapy. This procedure resulted in immediate, although ultimately impermanent, improvement in our patient’s respiratory insufficiency. Our patient also reported an improved quality of life during the period of time the laser ablation therapy offered him symptomatic relief. He was able to attend his grandchildren’s’ soccer games and walk to the end of his driveway to get his newspaper, activities he had not be able to participate in for years.

While our patient’s improvement was temporary, his disease process was uniquely advanced. It is possible that patients with less advanced disease may experience longer periods of remission with laser-mediated therapy, or may be able to tolerate repeated laser ablation procedures. Importantly, our patient’s case demonstrates that laser therapy can significantly reduce the burden of pemphigoid lesions, and can lead to a better quality of life for a disease process with few alternative treatment modalities.

Conclusion 

Therapeutic fiberoptic bronchoscopy with laser ablation is a promising treatment for patients suffering from CP of the trachea. Future investigations should focus on optimizing the laser ablation technique to achieve safe and sustained results.

References

1. Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol. 2000 Oct;43(4):571-91. [CrossRef] [PubMed]
2. Minaie A, Surani SR. Mucous Membrane Pemphigoid with Tracheal Involvement. Case Rep Pulmonol. 2016;2016:5749784. [CrossRef] [PubMed]
3. Kato K, Moriyama Y, Saito H, Koga H, Hashimoto T. A case of mucous membrane pemphigoid involving the trachea and bronchus with autoantibodies to β3 subunit of laminin-332. Acta Derm Venereol. 2014 Mar;94(2):237-8. [CrossRef] [PubMed]
4. You C, Lamba N, Lasave AF, Ma L, Diaz MH, Foster CS. Rituximab in the treatment of ocular cicatricial pemphigoid: a retrospective cohort study. Graefes Arch Clin Exp Ophthalmol. 2017 Jun;255(6):1221-1228. [CrossRef] [PubMed]
5. Oliveira PC, Reis Junior JA, Lacerda JA, Silveira NT, Santos JM, Vitale MC, Pinheiro AL. Laser light may improve the symptoms of oral lesions of cicatricial pemphigoid: a case report. Photomed Laser Surg. 2009 Oct;27(5):825-8. [CrossRef] [PubMed]
6. Yilmaz HG, Kusakci-Seker B, Bayindir H, Tözüm TF. Low-level laser therapy in the treatment of mucous membrane pemphigoid: a promising procedure. J Periodontol. 2010 Aug;81(8):1226-30. [CrossRef] [PubMed]
7. Minicucci EM, Miot HA, Barraviera SR, Almeida-Lopes L. Low-level laser therapy on the treatment of oral and cutaneous pemphigus vulgaris: case report. Lasers Med Sci. 2012 Sep;27(5):1103-6. [CrossRef] [PubMed]
8. Jalil BA, Abdou YG, Rosen SA, Dammad T. Mucous Membrane Pemphigoid Causing Central Airway Obstruction. J Bronchology Interv Pulmonol. 2017 Oct;24(4):334-338. [CrossRef] [PubMed]
9. Benge E, Yamaguchi L, Tran V, Sheikhan N, Bhatia S, Mcwhorter Y, Collier J, Chung A. Successful Treatment of Cicatricial Pemphigoid of the Trachea with Laser Ablation Bronchoscopy. Chest. 2021 Oct 1;160(4):A1999-2000 [Abstract]. [CrossRef]

Abbreviations

• Bipap: bilevel positive airway pressure
• CP: cicatricial pemphigoid
• Ho:YAG: holmium-doped yttrium aluminum garnet
• Laser: light amplification by stimulated emission of radiation
• LMA: laryngeal mask airway
• Nd:YAG: neodymium-doped yttrium aluminum garnet

Disclosures

Conflicts of Interest: The above listed authors have no conflicts of interest to declare.
Funding: This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA Healthcare or any of its affiliated entities.
This case was presented at the CHEST Annual Meeting that took place from Oct 17, 2021 – Oct 20, 2021 in a virtual format.

Cite as: Benge E, Tran V, Sheikhan N, Bhatia S, McWhorter Y, Collier J, Chung A. Symptomatic Improvement in Cicatricial Pemphigoid of the Trachea Achieved with Laser Ablation Bronchoscopy. Southwest J Pulm Crit Care. 2022;24(1):8-11. doi: https://doi.org/10.13175/swjpcc058-21 PDF 

January 2015 Pulmonary Case of the Month: More Red Wine, Every Time

Uzair Ghori, MD (UGhori@salud.unm.edu)

Shozab Ahmed, MD  (Sahmed@salud.unm.edu)

University of New Mexico

Albuquerque, New Mexico

History of Present Illness

A 41-year-old man travelling from Texas to Las Vegas, Nevada presents to the Emergency Room in Albuquerque, New Mexico with petechial rash, photophobia and headache of 2 weeks duration. The patient complains of general malaise, arthralgia, trouble sleeping, shortness of breath associated with cough and intermittent bilateral lower extremity swelling of 3 weeks duration.

PMH, SH & FH

The patient was prescribed lisinopril and metformin for hypertension and diabetes mellitus, respectively. He admitted occasional drinking, smoking a variable quantity for 30 years but currently not smoking. He denied any illicit drug use.

Physical Exam

Vitals: Heart Rate-92, Blood Pressure-116/45 mm Hg, Respiratory Rate-44 breaths/min, Temperature- 37.2ºC, SpO2-98% on non-rebreather mask.

General: His mental status was not altered.

HEENT: No papilledema was appreciated on eye exam.

Neck: JVP not appreciated.

Lungs: he had diminished breath sounds bilaterally on auscultation.

Heart: His heart had a regular rate and rhythm with no murmurs rubs or gallops.

Abdomen: No abdominal distention or lower extremity edema appreciated.

Skin: A petechial rash was noted most prominently in the lower extremities.

Based on the initial presentation the most appropriate investigations would be? (Click on the correct answer to proceed to the 2nd of 6 panels)

1. CBC, CT head, echocardiogram, blood cultures, metabolic panel, inflammatory markers
2. CBC, UA, lumbar puncture, chest x-ray, inflammatory markers, metabolic panel
3. Echocardiogram, CBC, UA, venous blood gases, bronchoscopy, CT head
4. Stress test, CXR, inflammatory markers, lumbar puncture, ultrasound abdomen, metabolic panel
5. UA, lumbar Puncture, bronchoscopy, echocardiogram, CT head, inflammatory markers 

Reference as: Ghori U, Ahmed S. January 2015 pulmonary case of the month: more red wine, every time. Southwest J Pulm Crit Care. 2015;10(1):1-7. doi: http://dx.doi.org/10.13175/swjpcc155-14 PDF

November 2012 Pulmonary Case of the Month: The Wolves Are at the Door

Lewis J. Wesselius, MD

Department of Pulmonary Medicine

Mayo Clinic Arizona

Scottsdale, AZ

History of Present Illness

A 49 year old female was admitted for hypoxia, lethargy, and an abnormal chest x-ray. She was recently discharged after a 10 day outside hospital stay for a diagnosis of pneumonia treated initially with azithromycin, then clindamycin and discharged on levofloxacin. Corticosteroids given during that hospitalization and she was discharged on taper. As the steroids were tapered, she had increasing dyspnea, confusion, and lethargy. She presented to the emergency room with an abnormal CT chest x-ray and was started on meropenem, vancomycin and azithromycin, and was also given IV methylprednisolone (125 mg initial dose).

PMH, FH and SH

She had her first stroke at age 18 and walks with a cane and has some expressive aphasia. There were multiple prior episodes of pneumonia (25 in 5 years). She was diagnosed with systemic lupus erythematosis (SLE) with lupus pneumonitis (based on surgical lung biopsy) about 3-4 years prior to admission. She had a St. Jude mitral valve replacement 12 years ago and had suffered a hemorrhagic stroke presumed secondary to anticoagulation. There is also a history of nephrolithiasis and recurrent urinary tract infections and anemia with multiple prior transfusions.

Her mother died at 49 reportedly due to complications of SLE.

Physical Examination

• Temperature 37.1° C;  Blood Pressure127/75 mm Hg;  Pulse 80 beats/min; SaO2 96% on 3 LPM
• HEENT: no significant abnormalities identified
• Chest: clear to auscultation and percussion
• Cardiovascular: mechanical click, no murmur
• Extremities: trace edema

Laboratory Evaluation

• Hemoglobin 10.1 g/dL   WBC  11,900  cells/μL   Platelets 137,000 cells/μL  
• INR 2.62
• Creatinine 0.9 mg/dL  BUN 15 mmol/L
• N-terminal pro-brain natriuretic peptide (NT pro-BNP) 1,294 pg/ml
• C-reactive protein (CRP) 74.7 mg/L 
• Erythrocyte sedimentation rate (ESR) 14 mm/hr
• Drug Screen:  negative

Chest X-ray

Her chest x-ray is shown below (Figure 1).

Figure 1. Portable chest radiography at the time of admission.

Which of the following are pulmonary complications of SLE?

1. Pleuritis
2. Chronic interstitial pneumonitis
3. Acute lupus pneumonitis
4. Pulmonary hypertension
5. All of the above

Reference as: Wesselius LJ. November 2012 pulmonary case of the month: the wolves are at the door. Southwest J Pulm Crit Care 2012;5: 223-8. PDF