National Registry of Interstitial Lung Disease from Pakistan

Abstract

Introduction Interstitial lung disease (ILD) is a heterogeneous group of over 200 parenchymal lung diseases with a myriad of etiologies. Interstitial lung disease registries from around the world show varying prevalence and incidence of these diseases. The aim of this study was to determine the epidemiology and characteristics of ILD in Pakistan. Methods This web-based registry, which is the first multicenter registry of ILD from Pakistan, recruited patients from 10 centers of five major cities between January 2016 and March 2019. Results A total of 744 patients were enrolled in the registry. The five most frequent ILDs were idiopathic pulmonary fibrosis (IPF) 34.4%, hypersensitivity pneumonitis (HP) - 17.7%, idiopathic nonspecific interstitial pneumonitis (iNSIP) - 16.8%, connective tissue disease-associated ILD (CTD-ILD) - 16.3%, and sarcoidosis - 9.1%. Conclusion Idiopathic pulmonary fibrosis is the most prevalent ILD in Pakistan, followed by HP and iNSIP. An ongoing prospective registry with longitudinal follow-up will help us further elaborate on the clinical characteristics, treatment, and survival outcome of patients with ILD.

Keywords: interstitial lung disease; lung fibrosis; pulmonary fibrosis; rare lung diseases.

Figure 1. Recruitment from Different Centres of Pakistan

AKUH=Aga Khan University Hospital, DP=Doctors Plaza, DUHS=Dow University of Health Sciences, SZH=Sheikh Zayed Hospital, KEMU=King Edward Medical University, NMCU=Nishtar Medical College, GDCH=Gulab Devi Chest Hospital, KTH=Khyber Teaching Hospital, LRH=Lady Reading Hospital, FJCGH=Fatema Jinnah Chest & General Hospital

Figure 2. Gender-wise Exposure in Patients with Hypersensitivity Pneumonitis

Figure 3. Box and Whisker Plot of Forced Vital Capacity (% of predicted) in Different ILDs

FVC=Forced Vital Capacity, ILD=Interstitial Lung Disease