June 2014 Arizona Thoracic Society Notes

The June 2014 Arizona Thoracic Society meeting was held on Wednesday, 6/25/14 at the Bio5 building on the University of Arizona Medical Center campus in Tucson beginning at 5:30 PM. This was a dinner meeting with case presentations. There were about 33 in attendance representing the pulmonary, critical care, sleep, pathology and radiology communities.

Four cases were presented:

1. Eric Chase presented a 68 year old incarcerated man shortness of breath, chest pain and productive cough.  The patient was a  poor historian. He was supposed to be receiving morphine for back pain but this had been held. He also had a 45 pound weight loss over the past year. His PMH was positive for COPD, hypertension, congestive heart failure, chronic back pain and  hepatitis C. Past surgical history included a back operation and some sort of chest operation. On physical examination he was  tachypneic, tachycardic  and multiple scars over his neck, back and chest including a median sternotomy scan. Subcutaneous emphysema was present. Laboratory evaluation was most remarkable for a lactate of 4.6 mg/dL. Chest x-ray revealed subcutaneous and mediastinal air, LLL consolidation, and a left pleural effusion.  Thoracentesis of the pleural effusion showed a high amylase and a low pH. A chest tube was placed. Esophagram showed contrast draining through the left chest and chest tube. CT scan was consistent with a colonic interposition graft with a graft to pleural fistula. The patient was deemed to be a poor surgical candidate and a jejunostomy tube was placed.
2. Mohammad Dalabih presented a 72 year old woman with asthma who had no response to asthma medications. Spirometry was consistent with moderate restriction. A thoracic CT scan showed two small nodules along with mosaic attenuation. A lung wedge biopsy showed nonmalignant appearing cells with tumorlets and bronchitis. The cells were CD56 positive. A diagnosis of diffuse interstitial pulmonary neuroendocrine hyperplasia (DIPNECH). Dr. Dalabih reviewed DIPNECH which usually presents in middle aged women with symptoms of cough and dyspnea; obstructive abnormalities on pulmonary function testing; and radiographic imaging showing pulmonary nodules, ground-glass attenuation, and bronchiectasis. In general, the clinical course remains stable; however, progression to respiratory failure can occur. Long-term follow- up studies and the best treatment remains unknown. The April 2014 Pulmonary Case of the Month also presented a case of DIPNECH (1).
3. Mohammad Alzoubaidi presented the case of a 61 year old woman with right upper quadrant pain who was found to have a large liver lesion on abdominal CT scan. She suffered a cardiac arrest shortly after the CT scan and her hemoglobin decreased to 5.6 g/dL. Angiography revealed multiple pseudoaneursyms with the largest apparently bleeding. Coil embolization was performed but a couple of days later her shock recurred. A repeat angiogram showed enlargement of the known pseudoaneursyms and several new ones. She was begun on corticosteroids for a presumed vasculitis. Unfortunately, she continued to bleed and died. Autopsy was consistent with fibromuscular dysplasia.  Fibromuscular dysplasia is a non-atherosclerotic, non-inflammatory disease of the blood vessels resulting in constriction and dilatation (pseudoaneursyms) (2). The cause and best treatment are unknown.
4. John Bloom presented a 22 year old Somali man that grew up in India who came to the US about 15 months before presentation. He was relatively asymptomatic but was found to have supraclavicular adenopathy on a "wellness" physical examination. Biopsy of the lymph nodes was recommended but he refused. He presented about a month later with neck and back pain. Physical examination revealed by adenopathy and a fever of 38.2º C. His white blood cell count was 12,600 cells/µL. Thoracic CT showed a miliary pattern with vertebral destruction. Laminectomy with cord stabilization was performed. Biopsy was negative for acid fast bacilli but positive for GMS+ organisms consistent with coccidioidomycosis. A large cervical paraspinal abscess just below the skull was drained and a large mediastinal abscess was also seen on CT scan. Discussion ensued about whether drainage was appropriate for the mediastinal mass, but most thought not.  The case illustrates that Valley Fever is common and in most chest differential diagnosis in the Southwest.

There being no further business the meeting was adjourned about 6:45 PM. There will be no meeting in July. The next meeting in Phoenix will be a case presentation conference on August 27, 6:30 PM at Scottsdale Shea Hospital.

Richard A. Robbins, MD

References

1. Wesselius LJ. April 2014 pulmonary case of the month: DIP-what? Southwest J Pulm Crit Care. 2014;8(4):195-203. [CrossRef]
2. Slovut DP, Olin JW. Fibromuscular dysplasia. N Engl J Med. 2004;350(18):1862-71. [CrossRef] [PubMed] 

Reference as: Robbins RA. June 2014 Arizona thoracic society notes. Southwest J Pulm Crit Care. 2014;8(6):356-7. doi: http://dx.doi.org/10.13175/swjpcc084-14 PDF

January 2014 Arizona Thoracic Society Notes

The January Arizona Thoracic Society meeting was held on Wednesday, 1/22/2014 at Shea Hospital beginning at 6:30 PM. There were 11 in attendance representing the pulmonary, critical care, sleep, pathology and radiology communities.

A discussion was held how to encourage attendance of young physicians to the Arizona Thoracic Society. A short presentation was made by Rick Robbins on the SWJPCC reiterating the material published in the yearly report from the editor (1).

Three cases were presented:

Dr. Tom Colby from the Pathology at the Mayo Clinic Arizona presented the first case. The patient was a 62 year old with polycythemia vera and shortness of breath. CT scan showed diffuse ground glass densities. The right ventricle and the pulmonary artery were slightly enlarged. A VATS lung biopsy was performed. The biopsy showed an increase in megakarocytes, immature red blood cells and immature white cell precursors consistent with extramedullary hematopoiesis. There was no fibrosis. There was a marked increase in CD34 staining consistent with alveolar septal capillary proliferation. Review of three other similar cases revealed similar findings. Dr. Colby questioned whether the endothelial proliferation could contribute to the clinical and radiologic findings. Suggestions were made to obtain pulmonary function testing, echocardiography and arterial blood gases. Depending on results it was felt that pulmonary hypertension needed to be excluded and he might require a right heart catherization.

Lewis Wesselius also from the Mayo Clinic Arizona presented a 53 year old woman from Indiana with a history of chronic cough and progressive shortness of breath since May 2013. Echocardiography showed 16% left ventricular ejection fraction. She had a biventricular pacemaker placed. A clinical diagnosis of sarcoidosis with a dilated cardiomyopathy was made but the patient did no improve on corticosteroids and methotrexate.  CT scan of the chest showed some mosaic attenuation.  There were no perilymphatic abnormalities as often seen in sarcoidosis. A VATS biopsy was performed. Histology revealed a proliferation of neuroendocrine cells within the airways forming tumorlets. A diagnosis of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) or a diffuse carcinoid tumor was made. Previously, 24 cases of DIPNECH were reported in the American Journal of Respiratory and Critical Care by Nassar et al. (2) but none had cardiomyopathy. It was questioned whether her cardiomyopathy could be secondary to DIPNECH.

Jessica Hurley from Bethesda North Hospital presented a 27 year old man with asthma, recurrent pneumonia, dyspnea and progressive hypoxia. He had an elevated IgE but less than 1000. Thoracic CT scan showed diffuse consolidation. Cultures were negative. He was placed on high dose corticosteroids but his hypoxia progressively worsened. A lung biopsy was performed and revealed broncholitis. No definitive diagnosis was apparent and it was felt there was more going than asthma but there were differing opinions on how to proceed. The biopsy will be reviewed by the lung pathologists at Mayo Clinic Arizona.

There being no further business the meeting was adjourned at about 8:30 PM. The next meeting is scheduled for Wednesday, February 26, 6:30 PM at Scottsdale Shea hospital.

Richard A. Robbins, M.D.

References

1. Robbins RA. The tremedous threes! annual report from the editor. Southwest J Pulm Crit Care. 2014:8(1):1-3. [CrossRef]
2. Nassar AA, Jaroszewski DE, Helmers RA, Colby TV, Patel BM, Mookadam F. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a systematic overview. Am J Respir Crit Care Med. 2011;184(1):8-16. [CrossRef] [PubMed] 

Reference as: Robbins RA. January 2014 Arizona thoracic society notes. Southwest J Pulm Crit Care. 2014:8(1):66-7. doi: http://dx.doi.org/10.13175/swjpcc006-14 PDF