Approaches in diagnosis, follow-up and treatment of patients with progressive pulmonary fibrosis: a questionnaire study

<bold>Introduction:</bold> Progressive pulmonary fibrosis (PPF) depends on the underlying disease; there is no complete consensus on diagnosis, follow-up, and treatment. We aim to evaluate strategies for PPF patients through a questionnaire. <bold>Methods:</bold> The link to a web-based questionnaire has been prepared to assess the diagnosis, follow-up, and treatment of patients with PPF. <bold>Results:</bold> In this study, 91 pulmonologists and 39 rheumatologists completed an online survey. Doing a multidisciplinary council(MDC) in their hospitals’ the rate was 63%; working with a thoracic radiologist, the rate was 71%, and working with pathologists studying interstitial lung diseases’ the rate was 40%. The most common underlying primary diseases were rheumatoid arthritis-associated ILD (46.2%) and systemic sclerosis-associated ILD (45.4%). The methods used during follow-ups were respiratory function tests (90%), DLCO (84%), HRCT (79%), and pulmonary symptoms (79%). Used first-line medications were steroids (85%), and second-line medications were MMF(58.5%). Antifibrotic drug treatment was given by 85% of participants, and 78.5% of them would use a combination of antifibrotic and immunosuppressive agents. While 28% of the participants did not experience any hesitation in diagnosing and treating PPF, the absence of a multidisciplinary team (35%) and the interpretation of radiological findings (31.5%) were the most hesitant situations. <bold>Conclusions:</bold> In this study, MDC were important for physicians in PPF patients. Although the management of PPF patients varied, the physicians’ approach to diagnosis, follow-up, and treatment of PPF patients was similar to the PPF guidelines’ recommendations.