How Do We Identify and Manage Progressive CTD-ILD?-Update in Treatment and Guideline

Abstract

The lung is a frequent target of autoimmune-mediated injury in patients with rheumatic diseases. Not infrequently, respiratory involvement may be the presenting manifestation of connective tissue diseases (CTDs). The prevalence of interstitial lung disease (ILD) in rheumatoid arthritis is about 20 to 30% of patients, while ILD may occur in 20 to 50% of patients with idiopathic inflammatory myositis, 40-50% or more of patients with systemic sclerosis, 2 to 5% of patients with systemic lupus erythematosus, 20 to 60% of patients with mixed connective tissue disease, and up to 25% of patients with Sjögren’s syndrome. Rheumatic disease-associated lung involvement is a major cause of morbidity and mortality in these patients. It shows a considerable heterogeneity in incidence and prevalence, clinical course, and the involved lung structure depending on the underlying rheumatic disease. Each rheumatic disease is associated with a characteristic pattern of their lung disease. The standard therapy has traditionally been immunosuppressant. With the evolvement of treatment, anti-fibrotic is now indicated for chronic fibrosing ILDs with a progressive phenotype, including autoimmune disease-associated ILD. By targeting the underlying mechanism of pulmonary fibrosis, it is shown that the annual rate of FVC decline can be reduced by more than 50%. In this lecture, Prof. Oliver Distler and Prof. Toby Maher will review the importance of timely treatment, the management practices and treatment guidelines for CTD-ILD.