Abstract

Introduction: Although PPF clinical trials provide valuable information, patients enrolled are selective due to restrictive inclusion and exclusion criteria. This evidence suggests that there is a need to investigate baseline characteristics and treatment outcomes in real-world populations of ILD especially when clinical practice is heterogenous globally due to the complexity of inflammatory and fibrotic interplay within ILD and drug reimbursement restrictions.

Methods: A cross-sectional retrospective study of all patients with ILD excluding IPF with data on immunosuppressant and antifibrotic treatment lines, conducted in the Institut Perubatan Respiratori, Kuala Lumpur, Malaysia during a 7-year period between 2016 ? 1st February 2023. 

Result: Out of the 106 cases reported, 55% were females with mean age of diagnosis was 62 years and  majority being non-smokers, 79%. The ILD by race was higher amongst the Indian at 48% followed by Malay and Chinese. The mean percent predicted forced vital capacity was at 55% and diagnosis of ILD was autoimmune ILD at 52% and HP at 26% were common. The most common immunosuppressant used was Azathioprine, 79% followed by mycophenolate mofetil, 19%. 

The percentage of patients deemed as PPF after 1st line immunosuppressant treatment was 44% whilst, the same percentage measure after 2nd line immunosuppressant treatment was at 25%.75% showed disease stabilization after intrioduction of 2nd line immunosuppresant therapy . 

Conclusion: The study suggests majority achieved disease stablization with immunosuppressant therapy.This provided the clinical rational of reserving antifibrotic in the third line setting and healthcare cost.