Agarwal A, Clements J, Sedmak D D, Imler D, Nahman N S, Orsinelli D A, Hebert L A
Department of Internal Medicine, Ohio State University Hospital, Columbus 43210, USA.
J Am Soc Nephrol. 1997 Dec;8(12):1971-6. doi: 10.1681/ASN.V8121971.
An adult man presented severely ill with vasculitis of his lower extremities and with impaired kidney function. After detailed evaluation at a local hospital, a diagnosis of essential type III cryoglobulinemia was made. High-dose steroid and cyclophosphamide therapy was begun. The patient improved dramatically. However, 6 wk later when his steroid dose was reduced to 30 mg daily, vasculitis recurred. Intensifying his immunosuppressive therapy only worsened his condition. He was than transferred to the Ohio State University Medical Center for consideration for plasmapheresis for the presumed essential type III cryoglobulinemia. However, our evaluation showed that he had bacterial endocarditis causing his type III cryoglobulinemia. When immunosuppressive drugs were stopped and antibiotics were begun, his condition resolved completely. This case illustrates the difficulty of identifying infectious causes of cryoglobulinemia and emphasizes that an initial, highly favorable response of vasculitis to immunosuppressive therapy does not exclude an infectious cause for the vasculitis.
一名成年男性因下肢血管炎和肾功能受损而病情严重。在当地医院进行详细评估后,诊断为原发性III型冷球蛋白血症。开始了大剂量类固醇和环磷酰胺治疗。患者病情显著改善。然而,6周后当他的类固醇剂量减至每日30毫克时,血管炎复发。加强免疫抑制治疗只会使他的病情恶化。随后他被转至俄亥俄州立大学医学中心,考虑对推测的原发性III型冷球蛋白血症进行血浆置换。然而,我们的评估显示他患有细菌性心内膜炎,导致其III型冷球蛋白血症。当停用免疫抑制药物并开始使用抗生素后,他的病情完全缓解。该病例说明了确定冷球蛋白血症感染病因的困难,并强调血管炎对免疫抑制治疗最初的高度良好反应并不排除血管炎存在感染病因。