The review focused on managing patients with pulmonary sarcoidosis against the backdrop of systemic vasculitis and the influence of a set of triggers which can be manifested by basal lymphadenopathy, infiltration of lung tissue, and damage to the eyes and skin, histologically characterized by the formation of non-caseous epithelioid-cell granulomas. A clinical case for managing patients with pulmonary sarcoidosis against systemic vasculitis was proposed. In this clinical case, it is crucial to consider the multifactorial nature of the pathology in the patient with the dominance of exogenous triggers (autoimmune pathology, sensitization, exposure to infectious factors, chemicals, stress), which can trigger the development of PS, complicate the course of accompanying pathology, and deteriorate the quality of life. Additionally, diagnosing PS in patients with other lung pathology, such as bronchial asthma, is often tricky and can affect the course of the underlying disease. Long-term immunosuppressive therapy may cause a secondary infection (viral, bacterial, fungal), which may require adequate etiotropic therapy. Keywords: pulmonary sarcoidosis, systemic vasculitis, diagnosis, immunosuppressive therapy