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

Abstract

BACKGROUND AND AIM: Progressive pulmonary fibrosis (PPF), depending on the underlying disease, lacks a complete consensus on diagnosis, follow-up, and treatment approaches, both in our country and worldwide. This study aims to evaluate the approaches of pulmonologists and rheumatologists to PPF patients using a questionnaire. METHODS: A web-based questionnaire consisting of 23 questions was prepared to assess the facilities of physicians' departments and their approaches to the diagnosis, follow-up, and treatment of patients with PPF. The questionnaire was sent to doctors' personal email addresses and the participants' responses were analyzed. RESULTS: A total of 91 pulmonologists and 39 rheumatologists completed the online survey. Among the participants, 44% had less than 10 years, 35% had 10-25 years, and 21% had more than 25 years of professional experience. Multidisciplinary councils were conducted in 63% of hospitals, 71% had thoracic radiologists, and 40% collaborated with pathologists specializing in interstitial lung diseases (ILD). The most common underlying primary diseases were rheumatoid arthritis-associated ILD (46.2%) and systemic sclerosis-associated ILD (45.4%). During follow-ups, the most commonly used methods included respiratory function tests (90%), carbon monoxide diffusion tests (84%), high-resolution computed tomography (79%), and pulmonary symptoms evaluations (79%). First-line medications for the underlying disease were steroids (85%), while second-line medications were mycophenolate mofetil (58.5%). Antifibrotic drug treatment was prescribed by 85% of participants, and 78.5% of them reported that they would use a combination of antifibrotic and immunosuppressive agents. While 28% of participants reported no hesitation in the diagnosis and treatment of PPF, the absence of a multidisciplinary team (35%) and challenges in interpreting radiological findings (31.5%) were the most commonly cited obstacles. CONCLUSIONS: This study highlights the importance of multidisciplinary councils for physicians managing patients with PPF. Although the management of PPF patients varied, the physicians' approaches to diagnosis, follow-up, and treatment of PPF patients aligned closely with recommendations in PPF guidelines.