Sunderkötter C, de Groot K
Klinik und Poliklinik für Dermatologie, Universitätsklinikum Münster.
Hautarzt. 2008 May;59(5):382-93. doi: 10.1007/s00105-008-1530-2.
Treatment and course of leukocytoclastic immune-complex vasculitis (LcV) depend on absence or presence of IgA in immune complexes [Henoch-Schoenlein-Purpura (PSH)]. LcV due to IgG- or IgM-containing immune complexes has a better prognosis. If triggers cannot be detected or avoided, symptomatic treatments are usually sufficient due to a usually favourable course. When hemorrhagic blisters suggest incipient skin necrosis corticosteroids are indicated. For chronic or relapsing LcV we suggest colchicine or dapsone. In adults with PSH and severe glomerulonephritis there is insufficient evidence for the efficacy of glucocorticoids; but e.g. ACE inhibitors can be helpful depending on symptoms. In cryoglobulinemic vasculitis underlying diseases (often plasmocytoma or hepatitis C) should be treated, sometimes supplemented by plasmapheresis. Dapsone or colchicine are usually started for urticarial vasculitis. ANCA-associated systemic vasculitis requires rapid and aggressive induction therapy, usually with glucocorticoids and cyclophosphamide. In classic polyarteriitis nodosa glucocorticoids improve prognosis, in polyarteriitis nodosa cutanea colchicine or dapsone are more appropriate. Giant cell arteriitis requires rapid therapy with glucocorticoids. For livedo vasculopathy antithrombotic measures are required with low molecular heparin or antagonists to vitamin K, for maintenance dipyridamol und aspirin.
白细胞破碎性免疫复合物性血管炎(LcV)的治疗及病程取决于免疫复合物中是否存在IgA[过敏性紫癜(PSH)]。由含IgG或IgM的免疫复合物引起的LcV预后较好。如果无法检测到或避免触发因素,由于病程通常良好,对症治疗通常就足够了。当出现出血性水疱提示早期皮肤坏死时,需使用糖皮质激素。对于慢性或复发性LcV,我们建议使用秋水仙碱或氨苯砜。在患有PSH和严重肾小球肾炎的成人中,糖皮质激素疗效的证据不足;但例如,根据症状,血管紧张素转换酶抑制剂可能会有帮助。在冷球蛋白血症性血管炎中,潜在疾病(通常为浆细胞瘤或丙型肝炎)应予以治疗,有时辅以血浆置换。氨苯砜或秋水仙碱通常用于治疗荨麻疹性血管炎。抗中性粒细胞胞浆抗体相关性系统性血管炎需要快速且积极的诱导治疗,通常使用糖皮质激素和环磷酰胺。在经典结节性多动脉炎中,糖皮质激素可改善预后,在皮肤型结节性多动脉炎中,秋水仙碱或氨苯砜更为合适。巨细胞动脉炎需要用糖皮质激素进行快速治疗。对于青斑样血管病,需要采取抗血栓措施,使用低分子肝素或维生素K拮抗剂,维持治疗时使用双嘧达莫和阿司匹林。