nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-3808Research ArticleШКОЛА НЕФРОЛОГАEDUCATIONAL MATERIALSСмешанная криоглобулинемия, гломерулонефрит и ANCA: эссенциальный криоглобулинемический или АНКА-ассоциированный васкулит?Mixed cryoglobulinaemia, glomerulonephritis and ANCA: essential cryoglobulinaemic vasculitis or ANCA-associated vasculitis?ЛампрехтП. .LamprechtP. .noemail@neicon.ruШмиттВ. Г.SchmittW. H.noemail@neicon.ruГроссВ. Л.GrossW. L.noemail@neicon.ruРевматологическая клиника Бад Врамштадт и ревматологическая поликлиника, Медицинский Университет Любека, ГерманияRussian Federation19992806199912-3173181Copyright © Лампрехт П..., Шмитт В.Г., Гросс В.Л., 19991999Лампрехт П..., Шмитт В.Г., Гросс В.Л.Lamprecht P..., Schmitt W.H., Gross W.L.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/3808

Антитела к цитоплазме нейтрофилов (АНКА) традиционно ассоциируются с некоторыми первичными системными васкулитами (ПСВ), такими как гранулематоз Вегенера (ГВ), микроскопический полиартериит (МПА) и синдром Чардж-Стросса (ЧСС), имеющими сходную клиническую симптоматику и являющимися «малоиммунными» по иммуногистологическим проявлениям. Эта группа первичных системных васкулитов определяется как «АНКА-ассоциированные васкулиты» (AAВ). Эссенциальные смешанные криоглобулинемические васкулиты (ЭСК), напротив, клинически гетерогенны и иммунологически характеризуются потреблением комплемента и наличием иммунокомплексных депозитов. Кроме того, они АНКА-негативны. В настоящем сообщении мы представляем двух больных с системным васкулитом, у которых выявлялись цАНКА (протеиназа 3-AНКA в одном случае) в сочетании с гломерулонефритом и различными экстраренальными проявлениями васкулита. В обоих случаях предполагался AAВ, однако обнаружение криоглобулинемии II типа в сочетании с гипокомплементемией и гистологически доказанными иммунными комплексами в клубочках позволили поставить диагноз смешанного криоглобулинемического васкулита, который в одном из этих случаев был ассоциирован с гепатитом С. Не утверждая понятия «ложно-положительных» цAНКА при ЭСК, мы обсуждаем методы дифференциальной диагностики и различные современные подходы к лечению. * Публикуется по согласованию с автором и с разрешения Oxford University Press

Antineutrophil cytoplasmic antibodies (ANCA) are generally believed to be strongly associated with some primary systemic vasculitis (PSV), such as Wegeener’s granulomatosis (WG), microscopic polyangiitis (MPA), and Churg-Strauss syndrom (CSS), wich have some clinical manifestations in common and are ‘pauci-immune’ by immunohistology. This group of PVS has thus been termed ‘ANCA-associated vasculitis’ (AAV). By contrast, essential mixed cryoglobulinaemic vasculitis (EMC) are clinically heterogeneous and characterized immunologically by complement consumption and by immunocomplex depositions; they are also characteristically ANCA-negative. We report here on two SV-patients in whom the detection of cANCA (proteinase 3-ANCA in one case) in conjunction with glomerulonephritis and various extrarenal vasculitic lesions was suggestive of an AAV. However, demonstration of type II cryoglobulinaemia, and histological proof of immunocomplexes in the glomerulus led to the diagnosis of an EMC, which was associated with hepatitis C virus (HCV) infection in one of the cases. Against the setting of ‘false positive’ cANCA in EMC, we discuss the differential diagnostic steps as well as current differential therapeutic approaches.

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The authors declare that there are no conflicts of interest present.