Diffuse Lung Disease: Student/Resident Case Report Poster - Diffuse Lung Disease |

To Biopsy or Not to Biopsy FREE TO VIEW

Nanda Din, MD; Wai Wai Lin, MD; Xavier Fonseca, MD; Noeen Ahmad, DO
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NYU Langone Medical Center, New York, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):528A. doi:10.1016/j.chest.2016.08.542
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SESSION TITLE: Student/Resident Case Report Poster - Diffuse Lung Disease

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: According to the American Thoracic Society guidelines, Idiopathic Pulmonary Fibrosis (IPF) can be diagnosed by typical UIP pattern on HRCT without lung biopsy. We present a case with a clinical picture of interstitial lung disease (ILD) but not definite IPF which surprised us with an unexpected diagnosis.

CASE PRESENTATION: A 63-year-old Hispanic male was referred to pulmonary clinic after hospitalization for worsening dyspnea. Past medical history included HTN, DM and smoking. Physical exam was significant for Velcro crackles in the lower lung fields. Spirometry revealed a restrictive pattern. HRCT showed subpleural honeycombing with upper lobe predominance and mediastinal lymphadenopathy. Initial differentials included sarcoidosis, hypersensitivity pneumonitis, malignancy or connective tissue disease. Autoimmune work-up was negative. ACE level was normal. Echocardiogram showed LVEF of 61% and sPAP of 41mmHg. His dyspnea worsened despite supportive management. Repeat HRCT failed to prove a confident diagnosis of IPF. After a multidisciplinary discussion, lung biopsy was pursued for definitive diagnosis and targeted therapy. Post-op course was morbid with prolonged mechanical ventilation and kidney injury. To our surprise, pathology revealed 2-3 layers of pulmonary capillary proliferation consistent with Pulmonary Capillary Hemangiomatosis (PCH).

DISCUSSION: PCH is a rare vascular disease caused by inappropriate proliferation of pulmonary capillaries within the lung. Only a few cases exist in the literature. In a series of 37 cases, only 3 were diagnosed by lung biopsy and the rest diagnosed on autopsy or in the explanted lung during lung transplant1. Clinical picture varies depending on the affected lung structures. Prognosis is poor and median survival is 3 years. In our patient, PCH was not part of our differential. We were reasonably convinced of the diagnosis of IPF, and planned for targeted therapy. We questioned if biopsy should not have been pursued given the morbid post-op course. The most serious complication of lung biopsy is mortality within post-op 30 days2. Risks and benefits should be considered when deciding to perform lung biopsy. Targeted therapy for IPF does not require a lung biopsy for diagnosis. Had this patient not received a lung biopsy, radiographic and clinical features were fairly suggestive of IPF, and he might have erroneously been started on the ineffective medication. No definitive treatment is available yet for PCH, except lung transplantation. We started patient on imatinib and will observe treatment response.

CONCLUSIONS: PCH is a rare pulmonary condition which can present as ILD and associated with poor prognosis. Lung biopsy is the only definitive diagnostic modality. Considering procedure associated complications, to biopsy or not to biopsy is a dilemma in patients with atypical clinical presentation of ILD as in our case.

Reference #1: Medicine (Baltimore) 2002 Nov;81(6):417-24.

Reference #2: Eur Respir J 2001, 17:175-179.

DISCLOSURE: The following authors have nothing to disclose: Nanda Din, Wai Wai Lin, Xavier Fonseca, Noeen Ahmad

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