Study of idiopathic pulmonary fibrosis in a tertiary care centre

Abstract

Introduction: Idiopathic interstitial pulmonary fibrosis is an important debilitating disease among the interstitial lung diseases, frequently diagnosed with the help of HRCT among the symptomatics. Materials and Methods: We have studied a total of 48 cases of IPF over a span of two and half years. Among a total of 104 ILDs attending our tertiary care centre IPF constituted 48 cases accounting for 46%. IPF cases are diagnosed on the basis of clinical history and hRCT findings. Results: There is a female preponderance of 1.4:1. Mean age of IPF patients in our study is 57.9 years. Mean age in females is 60.71 and 53.9 in males.Among our 48 cases of radiologically diagnosed IPF forty patients had definite UIP Pattern and 8 patients have possible UIP pattern as defined by the radiologist. The frequency of difinite IPF cases increased with severity of symptoms as measured by MMRC scale and the association is statistically significant. More than 90% of patients expressed tiresomeness and breathlessness. Cough was seen in 60% and pain chest was found in 45% of IPF patients. There is a statistically significant correlation of disease severity measured by MMRC scale with decrease in DLCO. BAL fluid lavage showed predominant neutrophils in >90% of IPF patients. Severity of IPF measured by MMRC correlated with decrease in 6 minute walk distance(p Summary and Conclusion: Idiopathic interstitial pulmonary fibrosis is a debilitating disease. Constitutes significant proportion of ILDs. Females outnumbered males. Tiresomeness and breathlessness are predominat symptoms. Disease occurred at a much earlier age in males. Active and passive smoking possibly contributes to the severity of IPF. MMRC grade correlates significantly with DLCO, 6 minute walk distance and definite UIP. There is no statistically significant correlation of MMRC grade with severity of PAH and FVC values. Keywords: Interstitial Lung Disease (ILD); Idiopathic Pulmonary Fibrosis (IPF); MMRC Grade, Desaturation; Br