12. Diffuse Parenchymal Lung Diseases: ILD, Sarcoidosis, IPF, LAM

A4937 - If You Know You Know: Diagnosing Childhood Idiopathic Pulmonary Fibrosis in a Resource Poor Setting

Presenter:
Date
05/21/2019
Room
Area E (Hall F, Level 2), KBHCCD
Session Name
C46 - CASE REPORTS: PEDIATRIC DIFFUSE LUNG DISEASE
Session Type
Thematic Poster Session

Abstract

INTRODUCTION: Idiopathic Pulmonary fibrosis(IPF) is rare in children with limited data across the world and more so in a developing country like ours.It is a chronic interstitial lung disorder, inflammatory in nature marked by pulmonary fibrosis and common histological features. It is believed that trigger factors initiate inflammatory reactions that progress to pulmonary fibrosis. This report aims to document IPF in a child in this environment and our approach to diagnosis. CASE: A 16 year old University undergraduate presented with 10 years history of recurrent breathlessness, three weeks’ dry non-productive cough and fever and two weeks’ vomiting.She had visited several other facilities to no avail. She was chronically ill looking and surprisingly not dyspnoic but had finger clubbing and SPO2 87%. Vital signs and physical examination were otherwise normal. Her weight was ˂3rd percentile while her height was 80th percentile for age. Diagnosis didn’t come easy but after excluding, Asthma and PTB, we settled on Pulmonary fibrosis, possibly IPF. CXR showed bilateral inhomogenous opacities with thin-walled cystic changes and Interstitial lung disease was suspected.CT Scan showed widespread cystic changes with subtle nodularities and areas of bronchiolar cystic dialation. Percutaneous lung biopsy from an area identified by CT scan showed alveoli lined by flattened epithelium and dense fibrosis in interalveolar spaces. Other foci of perivascular fibrosis with mixed inflammatory cells, mostly lymphocytes, consistent with bronchiectasis. Repeated lung function tests showed moderate to severe restrictive lung disease. She responded well to Prednisone and initial ibuprofen and had interval improvement in CXRs and LFTs. She reported back to school but couldn’t complete a full year when she relapsed and was rushed to a tertiary institution in another state and succumbed to the illness DISCUSSION: Diagnosing IPF needs a high index of suspicion , coupled with available expertise and affordable diagnostic technologies. This patient could afford several CXRs and LFT and a CT scan which thousands of children can not afford. Th bottom line is suspecting it clinically and making use of the expertise of lung tissue biopsy and imaging techniques. Unfortunately steroids was the available drug which she responded to but not without its attendant side effects.Funding Source: None
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