Connective tissue diseases (CTDs) such as systemic sclerosis (SSc), rheumatoid arthritis (RA), Sjögren’s syndrome (SS), idiopathic inflammatory myopathies (IIMs), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD), can be associated with a range of pulmonary manifestations including pleural involvement (pleuritis, pleural effusion, and pleural thickening), airways disease, vascular involvement (vasculitis), pulmonary hypertension, diffuse alveolar hemorrhage, and interstitial lung disease (ILD). In the course of systemic autoimmune disease ILD often appears early, representing even the first and/or the only manifestation. Up to 15% of the patients initially diagnosed with idiopathic nonspecific interstitial pneumonia (iNSIP) are found to have an underlying systemic rheumatic disease upon further investigation. Notably, ILD represents the leading cause of death in systemic autoimmune diseases patients, as marked in the EUSTAR database, being responsible for the death of 16.8% of SSc patients, followed by pulmonary arterial hypertension (PAH) (14.7%) and cancer (13.1%).