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[冷球蛋白血症的肾脏受累]

[Renal involvement of cryoglobulinemia].

作者信息

Karras Alexandre

机构信息

Service de néphrologie, hôpital européen Georges-Pompidou, 20, rue Leblanc, 75015 Paris, France; Faculté de médecine, université Paris Descartes, 12, rue de l'École-de-Médecine, 75006 Paris, France.

出版信息

Nephrol Ther. 2018 Apr;14(2):118-126. doi: 10.1016/j.nephro.2018.02.003. Epub 2018 Mar 12.

Abstract

Cryoglobulins are immunoglobulins that undergo reversible precipitation at low temperatures. They can induce systemic vasculitis, characterized by purpuric cutaneous lesions, arthritis, peripheral neuropathy, hypocomplementemia and glomerular disease. Renal pathology reveals membranoproliferative glomerulonephritis, with particularly intense mesangial cell proliferation and infiltration by macrophages, associated with intracapillary thrombi. This renal disease presents as a nephritic syndrome, with heavy proteinuria, haematuria severe hypertension and rapidly progressive kidney failure that can lead to end-stage renal disease. Hepatitis C is the main cause of mixed (type 2 or 3) cryoglobulinemia and requires the initiation of a specific antiviral therapy, together with immunosuppressive drugs. Rituximab is now considered as the best immunosuppressive therapy in this situation, inducing B-cell depletion, clearance of circulating cryoglobulin and resolution of renal symptoms. Monoclonal (type 1) cryoglobulinemia, is a rare condition, but it usually reveals an B-cell or a plasma cell proliferation, that require a specific hematological treatment to obtain remission of the renal disease.

摘要

冷球蛋白是在低温下会发生可逆沉淀的免疫球蛋白。它们可诱发系统性血管炎,其特征为紫癜性皮肤损害、关节炎、周围神经病变、补体血症及肾小球疾病。肾脏病理显示膜增生性肾小球肾炎,伴有特别强烈的系膜细胞增殖和巨噬细胞浸润,并伴有毛细血管内血栓形成。这种肾脏疾病表现为肾炎综合征,伴有大量蛋白尿、血尿、严重高血压及快速进展性肾衰竭,可导致终末期肾病。丙型肝炎是混合性(2型或3型)冷球蛋白血症的主要病因,需要启动特定的抗病毒治疗,并联合使用免疫抑制药物。利妥昔单抗目前被认为是这种情况下最佳的免疫抑制疗法,可诱导B细胞耗竭、清除循环冷球蛋白并缓解肾脏症状。单克隆(1型)冷球蛋白血症是一种罕见病症,但通常提示B细胞或浆细胞增殖,需要进行特定的血液学治疗以缓解肾脏疾病。

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