NOVEL APPROACHES TO MANAGEMENT OF PATIENTS WITH PULMONARY SARCOIDOSIS

Abstract

Sarcoidosis is an inflammatory multi-systemic disease of unknown origin with wide spectrum of clinical manifestations. Sarcoidosis may affect practically any organ — predominantly lungs, lymphatic system, skin, eyes alone or in combination. The disease is characterized by formation of non-caseous granuloma. In 1999 American Thoracic society (ATS), European Respiratory society (ERS) and World Association of Sarcoidosis and Other Granulomatous diseases (WASOG) adopted an international consensus statement on diagnosis and treatment of sarcoidosis «Statement on Sarcoidosis». Based on ATS, ERS and WASOG 1999 Statement the national documents such as “Evidence-based adapted clinical guideline on sarcoidosis” and “Unified clinical protocol of primary, secondary (specialized) and tertiary (highly-specialized) medical care for sarcoidosis” approved by MOH of Ukraine (decree # 634 dated 08 Sep 2014) were developed by current authors in Ukraine. In 2020 ATS experts published an updated «Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline», according to which the diagnosis of sarcoidosis was based on 3 criteria: compatible clinical symptoms, presence of non-necrotizing granulomatous inflammation in one or more tissue samples (not always required) and exclusion of alternative causes of granulomatous disorder. Finally, in 2021 ERS task force report was published “ERS clinical practice guidelines on treatment of sarcoidosis”, which outlined completely new approaches to management of sarcoidosis patients. According to this document, a decision about treatment of patient depends on 2 major factors: risk of death or organ failure and deterioration of quality of life of a patient. In addition, main principle of management of sarcoidosis patients is a balance between: a) minimization of risk of disability or death due to lung injury and decrease of quality of life, and b) risk of comorbidity and reduction of quality of life due to corticosteroid or other therapies. Current review focuses on analysis of major statements of these documents, disputable questions of initial therapy choice in patients with pulmonary sarcoidosis. Key words: pulmonary sarcoidosis, diagnosis, treatment, glucocorticosteroids, immunosuppressants, cytokine inhibitors.