Idiopathic pulmonary fibrosis: A Study of 46 patients from Western India: Clinical presentations and survival

Abstract

OBJECTIVES: Idiopathic pulmonary fibrosis (IPF) is the most common form of interstitial lung disease (ILD) diagnosed all over the world as well as in India. The objective was to study the clinical presentations, treatment options, and survival of patients diagnosed with IPF. MATERIALS AND METHODS: This was a 6-year retrospective observational study. All patients diagnosed with ILD underwent a comprehensive evaluation to confirm the diagnosis of IPF and were subsequently included in the study. Clinical data and laboratory data were recorded. Patients were treated as per current guidelines. RESULTS: Out of the total number of patients (n=123) diagnosed with ILD, 46 (37.4%) patients were included in the study [Males, n=19 (41%) and females, n=27 (59%)]. Sixty-seven percent (n=31) of the patients were in the age group of 61-80 years. The mean age of males and females was 68.36 and 66.25 years, respectively. Clubbing was observed in 67% (n=29) of the patients. The mean forced vital capacity (FVC) was 52%. High-resolution computed tomography (HRCT) of the chest showed a definite usual interstitial pneumonia (UIP) pattern of honeycombing in 60% (n=28) of the patients. Desaturation on a 6 min walk distance was noted in 60% (n=28) of the patients. Ten patients wrongly received-anti tuberculosis treatment (21%) before they were diagnosed with IPF. The median delay in diagnosis was 20 months. The 5-year survival rate was 25%; the average survival was 39 months. Patients receiving pirfenidone showed a survival benefit compared with those having received triple therapy. Survival in IPF was significantly low compared with that in other ILDs. Twenty-four (52%) patients died during the study. CONCLUSION: IPF is a disease which affects the fourth to seventh decade of the Indian population with low survival.