NATIONAL REGISTRY OF INTERSTITIAL LUNG DISEASE FROM PAKISTAN

Abstract

SESSION TITLE: Diffuse Lung Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: The aim of this study was to determine the epidemiology and characteristics of interstitial lung disease from Pakistan. METHODS: This web-based registry which is the first multicenter registry of ILD from Pakistan recruited patients from 10 centers of 5 major cities between January 2016 and March 2019.The ILD diagnosis was based on high-resolution computed tomography (HRCT) and histopathology in cases where biopsy was performed. The opinion of rheumatologist was also sought in cases of CTD related ILDs. The purpose of this multidisciplinary discussion (MDD) resource group was to maintain uniformity in the diagnostic criteria. RESULTS: A total of 744 patients were enrolled in the registry. The final ILD diagnoses were as follows: IPF 256 (34.4%), HP 132 (17.7%), iNSIP 125 (16.8%), CTD-ILD 121 (16.3%), sarcoidosis 68 (9.1%) and 42 (5.7%) were other ILDs. The median age was 57.5 years with interquartile range 46–65 years and gender was predominantly female (65.6%). A mean age greater than 60 years and history of smoking was most significant in IPF patients (P<0.001). The female gender was significant in all ILD's except IPF (P<0.001). The most common clinical symptoms were cough in 674 (90.6%) and dyspnea in 519 (69.75%). On physical examination, crepitations were audible in 674 (90.6%) and rhonchi in 83 (11.2%) while clubbing was seen in 247 (33.2%) which were mostly IPF. Gastroesophageal reflux disease (GERD) was present in 195 (26.2%) patients and was statistically significant with iNSIP and IPF (P=0.021). Echocardiography was done in 432 (58.1%) patients, of which 206 (47.7%) showed pulmonary arterial hypertension (PAH); 44.7% had mild PAH, 30.1% had moderate PAH and 25.2% had severe PAH. The overall predicted forced vital capacity (FVC) was 58.2 ±18.7%. FVC % of predicted was presented as box-and-whiskers plot showing the median, interquartile range and outliers (if any) for ILD patients. The median FVC for IPF was 59 (range: 25-99.99), for iNSIP was 54 (range: 25-99.99), for CTD-ILD was 60.5 (range: 26-99.99), for sarcoidosis it was 67 (range: 26-99.99). The HP patients had the lowest FVC % of predicted median that was 45.5 (range: 25-82) where four cases had ≥85 FVC and considered as outliers (Figure 2). An avian exposure was reported in 105 (79.5%) HP patients and out of which 86.8% were females. CONCLUSIONS: IPF 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 the clinical characteristics, treatment and survival outcome of patients with ILD. CLINICAL IMPLICATIONS: An expansion of the ILD registry to other cities and rural areas of the country with longitudinal follow-up of currently enrolled patients and more stringent validation of data by MDD in tertiary care and National Coordinating Centers could further expand the network and refine disease information. DISCLOSURES: no disclosure on file for Mosavir Ansarie; No relevant relationships by Ali Zubairi, source=Web Response