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

December 2013 Arizona Thoracic Society Notes

A breakfast meeting of the Arizona Thoracic Society and the Tucson winter lung series was held on Saturday, 12/14/2013 at Kiewit Auditorium on the University of Arizona Medical Center Campus beginning at 8:30 AM. There were 31 in attendance.

A lecture was presented by Joe G. N. "Skip" Garcia, MD, the senior vice president for health sciences at the University of Arizona (Figure 1).

Figure 1. Joe G. N. “Skip” Garcia, MD

The title of Garcia’s talk was “Personalizing Medicine in Cardiopulmonary Disorders: The Post ACA Landscape”.

Garcia began with reiterating that the Affordable Care Act (ACA, Obamacare) is fact and could pose a threat to academic medical centers. However, he views the ACA as an opportunity to develop personalized medicine which grew from the human genome project. Examples cited included the genetic variability among patients in determining the dose of warfarin and bronchodilator response to beta agonists in asthma (1,2).

Garcia’s laboratory has studied predominately 6 diseases including the adult respiratory distress syndrome (ARDS), idiopathic pulmonary fibrosis (IPF), sarcoidosis, asthma, pulmonary artery hypertension and sickle cell disease. Each has in common that there has been minimal progress made in the past generation and each has been shown to have racial or ethnic disparities in outcomes. He cited examples of how molecular testing could improve care.

Black and Hispanic patients with ARDS have a significantly higher risk of death compared with white patients (3). Garcia noted that the ventilator is not necessarily a friend and use of higher tidal volumes has been associated with increased mortality (4). He reasoned that the variation in susceptibility to ventilator induced lung injury could potentially explain the racial differences in mortality. Beginning with a dog model of ARDS, highly significant regional differences in gene expression were observed between lung apex/base regions. One of these potential targets was pre-B-cell colony enhancing factor (PBEF), a gene not previously associated with lung pathophysiology (5). Further work showed PBEF could induce changes seen in ARDS including a neutrophil alveolitis and increases in nuclear factor-κβ (NFKB) expression (6).

Few would question that there is a need for validated biomarkers in idiopathic pulmonary fibrosis. Using a similar approach to the investigation of PBEF in ARDS, peripheral blood mononuclear cell (PBMC) gene expression profiles predictive of poor outcomes in idiopathic pulmonary fibrosis (IPF) were examined by microarray. Microarray analyses suggest that 4 genes (CD28, ICOS, LCK, and ITK) are potential outcome biomarkers in IPF and should be further evaluated for patient prioritization for lung transplantation and stratification in drug studies (7). PBMC gene expression profiles were also examined in sarcoidosis.  There was a significant association of single nucleotide polymorphisms (SNPs) in signature genes with sarcoidosis susceptibility and severity (8). Further examples were presented on sickle cell disease.

Garcia concluded that molecular techniques represent powerful tools to investigate potential therapeutic approaches in respiratory diseases where little progress has been made.

Richard A. Robbins, MD

References

1. International Warfarin Pharmacogenetics Consortium, Klein TE, Altman RB, Eriksson N, Gage BF, Kimmel SE, Lee MT, Limdi NA, Page D, Roden DM, Wagner MJ, Caldwell MD, Johnson JA. Estimation of the warfarin dose with clinical and pharmacogenetic data. N Engl J Med. 2009;360(8):753-64. [CrossRef] [PubMed]
2. Duan QL, Lasky-Su J, Himes BE, Qiu W, Litonjua AA, Damask A, Lazarus R, Klanderman B, Irvin CG, Peters SP, Hanrahan JP, Lima JJ, Martinez FD, Mauger D, Chinchilli VM, Soto-Quiros M, Avila L, Celedón JC, Lange C, Weiss ST, Tantisira KG. A genome-wide association study of bronchodilator response in asthmatics. Pharmacogenomics J. 2013 Mar 19. [Epub ahead of print] [CrossRef] [PubMed]
3. Erickson SE, Shlipak MG, Martin GS, Wheeler AP, Ancukiewicz M, Matthay MA, Eisner MD; National Institutes of Health National Heart, Lung, and Blood Institute Acute Respiratory Distress Syndrome Network. Racial and ethnic disparities in mortality from acute lung injury. Crit Care Med. 2009 Jan;37(1):1-6. [CrossRef] [PubMed]
4. The Acute Respiratory Distress Syndrome Network. Ventilation with lower tidal volumes as compared with traditional tidal volumes for acute lung injury and the acute respiratory distress syndrome. N Engl J Med. 2000;342(18):1301-8. [CrossRef] [PubMed]
5. Simon BA, Easley RB, Grigoryev DN, Ma SF, Ye SQ, Lavoie T, Tuder RM, Garcia JG. Microarray analysis of regional cellular responses to local mechanical stress in acute lung injury. Am J Physiol Lung Cell Mol Physiol. 2006;291(5):L851-61. Herazo-Maya JD, Noth I, Duncan SR, Kim S, Ma SF, Tseng GC, Feingold E, Juan-Guardela BM, Richards TJ, Lussier Y, Huang Y, Vij R, Lindell KO, Xue J, Gibson KF, Shapiro SD, Garcia JG, Kaminski N. Peripheral blood mononuclear cell gene expression profiles predict poor outcome in idiopathic pulmonary fibrosis. Sci Transl Med. 2013 Oct 2;5(205):205ra136.
6. Hong SB, Huang Y, Moreno-Vinasco L, Sammani S, Moitra J, Barnard JW, Ma SF, Mirzapoiazova T, Evenoski C, Reeves RR, Chiang ET, Lang GD, Husain AN, Dudek SM, Jacobson JR, Ye SQ, Lussier YA, Garcia JG. Essential role of pre-B-cell colony enhancing factor in ventilator-induced lung injury. Am J Respir Crit Care Med. 2008;178(6):605-17. [CrossRef] [PubMed]  
7. Zhou T, Zhang W, Sweiss NJ, Chen ES, Moller DR, Knox KS, Ma SF, Wade MS, Noth I, Machado RF, Garcia JG. Peripheral blood gene expression as a novel genomic biomarker in complicated sarcoidosis. PLoS One. 2012;7(9):e44818. [CrossRef] [PubMed] 

Reference as: Robbins RA. December 2013 Arizona thoracic society notes. Southwest J Pulm Crit Care. 2013;7(6):360-2. doi: http://dx.doi.org/10.13175/swjpcc175-13 PDF

June 2012 Arizona Thoracic Society Notes

The June 2012 Arizona Thoracic Society meeting was held on 6/27/2012 at Scottsdale Shea beginning at 6:30 PM. There were 23 in attendance representing the pulmonary, critical care, sleep, pathology, infectious disease, radiology, and thoracic surgery communities.

Discussions were held regarding offering CME and partnering with other thoracic societies in the Southwest Journal of Pulmonary and Critical Care. This was endorsed by the membership. There was also discussion regarding what to discuss with Rep. David Schweikert on August 8.

Seven cases were presented:

1. Thomas Colby, a pulmonary pathologist from the Mayo Clinic, presented a case of a 45 yo woman with a history of asthma and systemic lupus erythematosis who was found to have cysts on CT scanning. The CT scan was considered consistent with lymphangioleiomyomatosis (LAM). A lung biopsy showed only changes consistent with asthma in addition to the cysts. This case was published along with 4 similar cases by Rowan C, et al. Am J of Surg Pathol 2012;36:228–34.
2. Jonathan Ruzi, a pulmonologist and sleep medicine specialist in Scottsdale showed an unusual flow-volume loop in an asymptomatic patient (Figure 1 below). Figure 1. Flow-volume loop of patient presented in case 2.         The cause of the obstruction was unknown but most thought this represented a type of upper airway obstruction from redundant tissue such as seen in obstructive sleep apnea or an enlarged tongue.
3. Henry Luedy, a pulmonary fellow, presented a case of an 82 yo with cough who was a former smoker with COPD who presented with a cough. The patient presented with a consolidative process in the lingula and underwent bronchoscopy which revealed bronchial inflammation and a trace of blood in the lingula. Biopsy revealed an adenocarcinoma. Unfortunately, the pathology was not presented due to Dr. Luedy being unable to obtain the slides or images from the VA due to a clerk citing HIPAA regulations as the reason. A discussion was led by Dr. Colby on how the pathology affects the classification of these tumors as bronchoalveolar or adenocarcinoma and how there is much overlap between the classification. It was noted that educational activities are excluded from HIPAA regulations as long as the data is de-identified and there are not identifiers on a pathology slide.
4. Tonya Whiting, a pulmonary fellow, and Manny Mathew, a pulmonologist based at Good Samaritan, presented a case of a man who developed shortness of breath while camping in the White Mountains. CT scanning revealed dense consolidation especially of the left upper lobe. He was referred for bronchoscopy but both his symptoms and consolidation resolved within 24 hours. It was felt this was a case of high altitude pulmonary edema which was somewhat unusual because high altitude pulmonary edema is unusual below 12000 feet (the patient was camping at about 9000 feet).
5. Tonya Whiting and Allen Thomas, a pulmonologist at the VA, presented a 61 year old man with a history of polysubstance abuse and multiple lung nodules. Bronchoscopy with bronchoalveolar lavage and transbronchial biopsy was negative. Open lung biopsy revealed brochiolcentric inflammation with acute lung injury. The patient was treated with corticosteroids and the nodules resolved in 2-3 weeks. The feeling was that this represented cryptogenic organizing pneumonia (COP) presenting with multiple nodules which is a rare presentation for COP.
6. Andrew Goldstein, a thoracic surgeon, presented a case of a 50 year old asymptomatic, nonsmoker with a huge, > 10 cm, lung tumor. The lesion was round and smooth and did not invade the chest wall on CT scan. Dr. Goldstein pointed out that pain is sensitive in predicting chest wall invasion. The tumor was resected and proved to be a carcinoid tumor.
7. Tim Kurberski, an infectious disease specialist from Maricopa Medical Center, presented a 39 year old with a history of systemic lupus erythematosis on corticosteroids who presented with shortness and breath thought to be secondary to pulmonary edema from a cardiomyopathy. The CT scan revealed diffuse ground glass opacities. The patient also had a rash near the buttocks which was thought to be possible shingles and the chest findings possible chickenpox pneumonia. The steroids were increased but the patient failed to improve. She underwent bronchoscopy with bronchoalveolar lavage which revealed larvae consistent with Strongyloidiasis.

There being no further cases, the meeting was adjourned at 8:30 with the next meeting being a special meeting on August 8 when Rep. David Schweikert is scheduled to attend. 

Richard A. Robbins, M.D.

CCR Representative

Arizona Thoracic Society

Reference as: Robbins RA. June 2012 Arizona Thoracic Society notes. Southwest J Pulm Crit Care 2012;4:211-3. (Click here for a PDF version of the Notes)