Diffuse Lung Disease: Fellow Case Report Poster - Diffuse Lung Disease II |

Early Onset Idiopthic Pulmonary Fibrosis FREE TO VIEW

Mehboob Kalani, MD; Viral Gandhi, MBBS; Candice Brem, MD; Tariq Cheema, MD
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Allegheny General Hospital, Pittsburgh, PA

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

Chest. 2016;150(4_S):499A. doi:10.1016/j.chest.2016.08.513
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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: We present a rare case of idiopathic pulmonary fibrosis (IPF) in a 31-year-old patient.

CASE PRESENTATION: A 31 year-old Caucasian male with 20 pack-year smoking history was referred for evaluation of shortness of breath. Symptoms started 6 months ago. It was associated with progressive weight loss and non-productive cough. No occupational exposure. No family history of pulmonary fibrosis. Physical exam showed occasional inspiratory rales. No clubbing. Oxygen saturation was 93% on room air. Chest x-ray showed bilateral symmetric lower lobe nodular interstitial thickening. CT Chest showed bilateral lower lobe predominant reticular thickening and mild traction bronchiectasis with sparing of subpleural space. (Figure 1) Connective tissue work-up was negative. Pulmonary function test showed moderate restriction and moderate decrement in diffusion capacity. Open lung biopsy showed fibroblastic foci with usual interstitial pneumonia (UIP) pattern consistent with IPF. (Figure 2)

DISCUSSION: IPF is a chronic, progressive fibrosing interstitial pneumonia of unknown cause and associated with histopathologic and/or radiologic pattern of UIP. (1) The mean age at diagnosis is 66 years. It is uncommon in patients younger than 50 years. Historical studies have shown a survival advantage for patients with other forms of IIP when compared to IPF. Our patient presented at a young age with smoking history and bilateral lower lobe predominant reticular opacities favoring a diagnosis of smoking related IIP namely desquamative Interstitial Pneumonia or Respiratory Bronchiolitis - Interstitial lung disease. Sparing of sub-pleural space is characteristic for non-specific interstitial pneumonia. IPF is characterized by peripheral, subpleural reticular opacities with traction bronchiectasis and honeycombing. Biopsy proves pivotal in diagnosis of IIP; which was the case in our patient. Biopsy showed fibroblastic foci which is characteristic for IPF. The treatment of IPF is evolving and changing in recent times with guidelines signifying the role of Pirfenidone and Nintedanib. However, the mortality and survival of this disease entity still remains elusive.

CONCLUSIONS: Patient was diagnosed with IPF after multidisciplinary committee meeting. He was started on Pirfenidone and referred for lung transplant.

Reference #1: An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management Ganesh Raghu, et al. American Journal of Respiratory and Critical Care Medicine 2011 183:6, 788-824.

DISCLOSURE: The following authors have nothing to disclose: Mehboob Kalani, Viral Gandhi, Candice Brem, Tariq Cheema

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