Application of plasma exchange in systemic vasculitis with dual seropositivity. Review and case report

Systemic vasculitis (SV) is a heterogeneous group of diseases. Their main morphological feature is inflammation of the vascular wall. The clinical manifestations depend on the type, diameter, and localization of the affected vessels and the activity of systemic inflammation. Vasculitis of small vessels is sometimes associated with antineutrophil cytoplasmic antibodies (ANCA-SV). The etiology of ANCA-SV is unknown although their development is associated with genetic factors, environmental exposure, medications, and infections. The key role was assigned to antineutrophil cytoplasmic autoantibodies to proteinase 3 and myeloperoxidase contained in primary neutrophil granules and monocyte lysosomes. Recently, the effector function of neutrophils was described: the release of neutrophil extracellular traps (NETs). NETs may contribute to the initiation of the autoimmune process. The role of an alternative complement pathway, in particular, anaphylatoxin C5A, and the C5A receptor (CD88) in the pathogenesis of ANCA-SV was demonstrated. B cells play a major role in the pathogenesis of ANCA-SV since they produce ANCA. ANCA-SV treatment is primarily aimed at suppressing the autoimmune and inflammatory process and removing ANCA using extracorporeal hemocorrection methods. Despite significant advances in the treatment of ANCA-SV the effectiveness of extracorporeal hemocorrection remains controversial. Some local small studies show positive results of plasma exchange. Nevertheless, large-scale studies have not revealed the long-term benefits of plasma exchange therapy. The effective use of plasma exchange in the treatment of anti-glomerular basement membrane (GBM) disease has been proven. The results of previous studies have shown that 40% of patients with the anti-GBM disease have ANCA circulating in the blood, in the predominant number of cases to myeloperoxidase. Only a few clinical cases describing the treatment of patients with double seropositivity have been reported. Here we present a clinical case of successful application of extracorporeal hemocorrection in the treatment of a patient with double seropositivity for ANCA and GBM antibodies.

АНЦА-ассоциированный васкулит, анти-БМК заболевание, плазмообмен, ANCA-associated vasculitis, anti-glomerular basement membrane disease, plasma exchange

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