Damage to the respiratory system worsens prognosis and increases mortality in all systemic immunoinflammatory rheumatic diseases (SIRDs). Leading pulmonary manifestations in each disease are different, so the typical picture of each nosological entity is very peculiar. In all SIRDs, the disease starts with lung involvement in 10—20% of cases, preceding the expanded clinical presentation of a systemic disease. At a certain stage, lung damage may be the only manifestation of the disease or predominate in its clinical picture, determining the severity of a condition. Among the patients referred to consultation with a pulmonologist for interstitial lung disease, about 15% have manifestations of SIRDs that is confirmed by a special survey. The paper discusses the diagnostic features of SIRDs with lung involvement, as well as indications for consultation with a rheumatologist. The key clinical symptoms indicating a possible rheumatic disease in patients with lung involvement are considered. Evidence for the main circulating autoantibodies identifiable in high titers in patients with SIRDs in a serological test is given and its positivity is interpreted. The issues associated with the term «undifferentiated connective tissue diseases-associated interstitial lung disease» are discussed; the expediency of using the term «interstitial pneumonia with autoimmune signs (AIS)» is emphasized. The clinical characteristics of and classification criteria for AIS are presented. The prognosis and outcomes of interstitial pneumonias of varying etiologies, as well as the importance of the multidisciplinary approach to managing patients with SIRDs and lung involvement, which improves the quality of diagnosis, adequacy of treatment, and quality of life, are dealt with.