A Mistaken Case of Sarcoid

Abstract

SESSION TITLE: Fellow Case Report Poster - Diffuse Lung Disease II SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Chronic hypersensitivity pneumonitis (HP) is a diffuse parenchymal lung disease caused by repeat exposure to a variety of antigens. It can mimic several interstitial lung diseases radiologically and pathologically making it difficult to differentiate. CASE PRESENTATION: A 40 year old female was referred to our pulmonary clinic for ILD evaluation after recently immigrating from Pakistan. She reported years of debilitating cough progressive dyspnea. She lived in an urban region and denied any significant organic or chemical exposures. Her initial pulmonary function testing (PFTs) suggested severe restrictive lung disease and her CT Chest revealed coarse upper lobe reticulation and architectural distortion. Despite non-revealing trans-bronchial and EBUS biopsies at the time, she was started on prednisone therapy for presumed sarcoidosis. Notably, her steroids were begun just months before her move and she reported persistent improvement in her symptoms since, despite the lack of objective changes in PFTs and CT chest on our assessment. Extensive serologic evaluation for an etiology revealed only antibodies to pigeon droppings which were not consistent with her history. She underwent VATs biopsy which revealed many poorly formed granulomas, severe diffuse interstitial fibrosis, cholesterol clefts, and Schumann bodies. Her final diagnosis was deemed to be chronic HP with fibrosing NSIP pattern. DISCUSSION: A definitive diagnosis for HP requires exposure to a known antigen and the assemblage of clinical, radiologic, laboratory, and pathologic findings. Due to the similar pathological features of NSIP and Chronic HP, the diagnosis remains a challenge. While acute HP may respond to immunosuppressive therapy, it is less clear whether patients with severe disease with an unclear precipitant would benefit from such therapy. In our case, it is unclear if the steroids or the environmental change (or both) may have halted the progression of her already severe disease. Thereby making the decision for long term immunosuppression a more difficult one. CONCLUSIONS: There remain large gaps in our knowledge with regards to the management of chronic HP, necessitating the need for larger case series or prospective trials. Reference #1: Churg A, Sin DD.Pathologic patterns and survival in chronic HP. Am J Sur Path.2009Dec;33(12)1765-70. Reference #2: Selman M, Pardo A. HP:insights in diagnosis and pathobiology. Am J Respir Crit Care Med 2012;186:314-324. Reference #3: Vourlekis JS, Schwarz MI.NSIP as the sole histologic expression of HP.Am J Med 2002;112:490-493. DISCLOSURE: The following authors have nothing to disclose: Mohammed Siddiqui, Omid Savari, Yasir Tarabichi No Product/Research Disclosure Information