New in understanding of the pathophysiology, clinical features and treatment of systemic vasculitis: a literature review 2018–2019

Головач И.Ю., Егудина Е.Д., Тер-Вартаньян С.Х.

Summary. Systemic vasculitis is a systemic autoimmune disease. This pathology is characterized by a wide range of clinical manifestations and complications, with the potential involvement of any organ and system in the pathological process and leading to disability of patients. The purpose of the study: to find out whether the available literature data over the past two years can open up new possibilities in the precision and individualized treatment of systemic vasculitis. Material and methods. This work provides a review of the literature on pathophysiology, clinical features and treatment of systemic vasculitis of small and large caliber vessels, cryoglobulinemic vasculitis, based on a search of English-language articles in the Medline and PubMed databases published from January 2018 to June 2019. Results and discussions. Fundamental studies of the last 12 months confirm previous evidence that anti-neutrophilic cytoplasmic antibodies (ANCA), causing excessive formation of an extracellular neutrophilic trap and activation of complement, play a leading role in the pathogenesis of ANCA-associated vasculitis. The key role of CD4⁺ T cells in the development of granulomatous inflammation and tissue damage in vasculitis is emphasized. Data from new studies on the pathogenesis of vasculitis have contributed to the emergence of new options for therapeutic strategies aimed at improving the long-term results of treatment of patients. Tocilizumab is increasingly being used to treat large caliber vasculitis, and growing evidence confirms its effectiveness in routine clinical practice. Promising data have been obtained on the use of leflunomide as a steroid-saving steroid agent for giant cell arteritis and ustekinum in patients with refractory type of this vasculitis. Micophenolate mofetil was not inferior to cyclophosphamide in the induction of remission. Conclusions. Over the previous year, tremendous progress has been made in understanding the pathophysiology of vasculitis, developing and testing new therapeutic agents. This review of the literature showed that knowledge of the pathogenesis and clinical phenotypes of vasculitis is a prerequisite for the transition to precision and individualized medicine for vasculitis.