Background The vasculitides are a heterogenous group of diseases involving inflammation of blood vessels with subsequent tissue and/or organ failure.1 Systemic vasculitis may be idiopathic or secondary associated with variety of diseases of known etiology, as one of the most severe clinical features are the necrotic changes of the extremitas and/or internal organs.2
Objectives The objective of this study was to analyse a group of patients with different forms vasculitides, presenting with necrotic changes in relation to nosological belonging, association with other vasculitis lesions and immunological profile.
Methods The study included 76 patients (19 men and 57 women) with vasculitic necrotic lesions, as the mean age was 59.7 years. The diagnoses were determined using the diagnostic criteria for systemic lupus erythematodes (SLE), progressive systemic sclerosis (PSS), rheumatoid artritis (RA), ANCA-associated vasculitis (AAV). Sera were tested for panel antinuclear antibodies (ANA) (anti-RibP, dsDNA, PCNA, Ro60, La, CENT-B, Sm, RNP/Sm, nucleosomes, Jo-1, Scl-70, Pm/Scl), for some antiphospholipid antibodies (anti-anticardiolipin, b2GPI, protrombin) and antineutrophil cytoplasmic antibodies (ANCA).
Results The most frequent nosological entities associated with vasculitis, presenting with necrotic lesions, were SLE and PSS (44%). Hypersensitivity vasculitis with known and unknown etiology was diagnosed in 35.5%. AAV was determined only in 6.58% from the group with necrosis. It is interesting that rheumatoid artritis was associated with necrotic vasculitic changes in 7.9% of cases. Idiopathic vasculitis with necrosis wasn’t observed in the study. Analising other vasculitic lesions we found association with Raynaud’s phenomenon in 85.53%, cyanotic spots – 40.79%, livedo reticularis in 7.89% of patients, periungual vasculitis – 23.86%. The most frequently expressed antinuclear antibodies were anti-topoisomerase Abs (anti-Scl-70) – in 45% of patients. Other ANA were rarer detected. Antiphospholipid Abs were present in 33.3% of necrotizing vasculitis. ANCA were observed only in 3.75% of patients with necrosis.
Conclusions Vasculitis with necrosis is developed in the course of a variety of diseases, as it is associated with other vasculitic lesions in the significant part of patients. It is supposed different antibodies participate in the pathogenetic mechanisms connecting with the damage of the endothelium, following endothelial dysfunction, which promotes vascular inflammation.
Merkel P et Mahr AD: Classification and epidemiology of vasculitis. Rheumatology, ELSEVIER, Toronto, Canada, 2011: 1501
Churg J. Systemic necrotizing vasculitis. Cardiovascular pathology, 1994, 3: 197-204.
Disclosure of Interest None Declared