Connective tissue disease (CTD) comprising a various range of autoimmune disorders is often accompanied by lung involvement, which can lead to life-threatening complications. The main types of CTDs that can present as interstitial lung disease (ILD) include rheumatoid arthritis, systemic sclerosis, Sjögren's syndrome, mixed connective tissue disease, idiopathic inflammatory myopathies, and systemic lupus erythematosus. CTD-ILD poses a significant challenge in clinical diagnosis and management due to its heterogeneous nature and variable prognosis. Early diagnosis by clinical, serological, and radiographic assessments is crucial to differentiate CTD-ILD from idiopathic forms and to adopt appropriate therapeutic strategies. Therefore, we have reviewed the numerous clinical manifestations and diagnostic approaches for each type of CTD-ILD, considering the diversity and complexity of CTD-ILD. The significance of a multidisciplinary approach in optimizing the management of CTD-ILD has been underscored by recent therapeutic advancements, which include the use of immunosuppressive agents, antifibrotic therapies, and newer biological agents targeting specific pathways implicated in the pathogenesis. Therapeutic strategies should be tailored to each condition, considering the type of CTD, the extent of lung involvement, and the presence of extrapulmonary manifestations. Furthermore, we aimed to provide clinical guidance including therapeutic recommendations for the effective management of CTD-ILD, based on PICO analysis.
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