nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.24884/1561-6274-2010-14-1-11-28nefr-1200Research ArticleПЕРЕДОВАЯ СТАТЬЯLEADING ARTICLEСМЕШАННАЯ КРИОГЛОБУЛИНЕМИЯMIXED CRYOGLOBULINEMIAФерриС.FerriC.

кафедра ревматологии

Университет Модены и области ЭмилияItaly2010100120101411128Copyright © Ферри С., 20102010Ферри С.Ferri C.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephrolog.ru/jour/article/view/1200

Смешанная криоглобулинемия (СК), тип II и тип III, – это заболевание, при котором в сыворотке крови выявляют циркулирующие криопреципитирующие иммунные комплексы и которое проявляется классической триадой симптомов: пурпурой, слабостью и артралгиями. Считается, что СК – редкое заболевание, однако, истинная заболеваемость остается неизвестной. Заболевание чаще встречается в Южной Европе по сравнению с Северной Европой или с Северной Америкой. Распространенность «эссенциальной» криоглубулинемии составляет приблизительно 1:100 000 (соотношение женщин и мужчин при этом 3:1), однако, этот термин в настоящее время используют только в отношении небольшого числа случаев СК. СК характеризуется вовлечением в процесс различных органов: кожа (ортостатическая пурпура, язвы), хронический гепатит, мембрано-знопролиферативный гломерулонефрит, периферическая нейропатия, диффузный васкулит и, реже, интерстициальные поражения легких и эндокринные нарушения. Как позднее осложнение, у некоторых пациентов могут развиваться злокачественные поражения лимфатической системы и печени. СК может быть ассоциирована с различными инфекциями и иммунными заболеваниями. При отсутствии подобной ассоциации СК представляет собой самостоятельное заболевание, так называемую «эссенциальную» СК. Этиопатогенез СК окончательно не ясен. Предполагают, что инфицирование вирусным гепатитом С (HCV) играет причинную роль, однако, с вкладом генетических и/или средовых факторов. Более того, СК может быть связана и с другими инфекционными агентами и иммунными расстройствами, такими как вирус иммунодефицита человека (ВИЧ) или первичный синдром Шегрена. Диагностика основывается на клинических и лабораторных данных. Циркулирующие смешанные криоглобулины, низкий уровень С4 и ортостатическая кожная пурпура являются классическими признаками болезни. Типичной патоморфологической находкой является лейкоцитокластический васкулит с вовлечением сосудов среднего и, более часто, мелкого диаметра, который выявляется при биопсии свежих кожных высыпаний. Дифференциальный диагноз необходимо проводить с широким спектром системных, инфекционных, неопластических процессов, главным образом с аутоиммунным гепатитом, синдромом Шегрена, полиартритом и В-клеточными лимфомами. Терапия СК первой линии должна включать эрадикацию вируса гепатита С путем комбинированного применения интерферона и рибавирина. Патогенетическая терапия (иммуносупрессия, ГКС и/или плазмаферез) должна соответствовать тяжести клинических проявлений и их прогрессирования в каждом конкретном случае. При СК необходимо длительное наблюдение для своевременной диагностики и лечения угрожающих жизни осложнений. Общий прогноз хуже у пациентов с вовлечением почек, печеночной недостаточностью, лимфопролиферативными заболеваниями, злокачественными образованиями.

Mixed cryoglobulinemia (MC), type II and type III, refers to the presence of circulating cryoprecipitable immune complexes in the serum and manifests clinically by a classical triad of purpura, weakness and arthralgias. It is considered to be a rare disorder, but its true prevalence remains unknown. The disease is more common in Southern Europe than in Northern Europe or Northern America. The prevalence of ‘essential’ MC is reported as approximately 1:100,000 (with a female-to-male ratio 3:1), but this term is nowused to refer to a minority of MC patients only. MC is characterized by variable organ involvement including skin lesions (orthostatic purpura, ulcers), chronic hepatitis, membranoproliferative glomerulonephritis, peripheral neuropathy, diffuse vasculitis, and, less frequently, interstitial lung involvement and endocrine disorders. Some patients may develop lymphatic and hepatic malignancies, usually as a late complication. MC may be associated with numerous infectious or immunological diseases. When isolated, MC may represent a distinct disease, the so-called ‘essential’ MC. The etiopathogenesis of MC is not completely understood. Hepatitis C virus (HCV) infection is suggested to play a causative role, with the contribution of genetic and/or environmental factors. Moreover, MC may be associated with other infectious agents or immunological disorders, such as human immunodeficiency virus (HIV) infection or primary Sjцgren’s syndrome. Diagnosis is based on clinical and laboratory findings. Circulating mixed cryoglobulins, low C4 levels and orthostatic skin purpura are the hallmarks of the disease. Leukocytoclastic vasculitis involving medium- and, more often, small-sized blood vessels is the typical pathological finding, easily detectable by means of skin biopsy of recent vasculitic lesions. Differential diagnoses include a wide range of systemic, infectious and neoplastic disorders, mainly autoimmune hepatitis, Sjцgren’s syndrome, polyarthritis, and B-cell lymphomas. The first-line treatment of MC should focus on eradication of HCV by combined interferon-ribavirin treatment. Pathogenetic treatments (immunosuppressors, corticosteroids, and/or plasmapheresis) should be tailored to each patient according to the progression and severity of the clinical manifestations. Long-term monitoring is recommended in all MC patients to assure timely diagnosis and treatment of the life-threatening complications. The overall prognosis is poorer in patients with renal disease, liver failure, lymphoproliferative disease and malignancies.

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