Rollino Cristiana, Coppo Rosanna, Giacchino Franca, Savoldi Silvana, Manganaro Marco, Amore Alessandro, Colla Loredana, Ferro Michela, Demicheli Giovanni, Berutti Silvia, Burdese Manuel, Paternoster Giuseppe, Cravero Raffaella, Benozzi Luisa, Vagelli Giuseppe, Messuerotti Alessandra, Licata Carolina, Bainotti Serena, Patti Rosaria Rita, Quaglia Marco, Costantini Luigia, Stratta Piero, Segoloni Giuseppe
Nefrologia e Dialisi, Ospedale S.G. Bosco, Torino, Italy.
G Ital Nefrol. 2010 Nov-Dec;27(6):639-48.
The treatment of membranous glomerulonephritis (MGN) is controversial, especially in cases of no response to first-line treatment or multiple relapses. The Clinical Nephrology Group of Piedmont carried out a multicenter analysis of the treatment of patients affected by MGN in 15 nephrology units in Piedmont. The first treatment is usually started after a waiting period of 3-6 months in case of proteinuria in the nephrotic range but normal or slightly impaired renal function. A history of cancer, the presence of infectious disease, and secondary forms of MGN are criteria for exclusion from treatment. As first-line treatment, Piedmont nephrologists prescribe corticosteroids alternated with immunosuppressive drugs, generally preferring cyclophosphamide to chlorambucil. Only one nephrology unit uses cyclosporin A (CyA) as the first choice. In case of no response to treatment, a second therapeutic approach is undertaken after 2-12 months. Second-line treatment consists of CyA if immunosuppressive drugs were given before, and corticosteroids/ immunosuppressive drugs if CyA was the first treatment. A further choice may be ACTH or rituximab. In case of multiple relapses the treatment options are the same but previous immunosuppressive treatment, patient age, and the duration of kidney disease with a greater probability of renal failure and progression towards sclerosis require careful attention. Concern has been expressed regarding the potentially severe side effects of ACTH including myopathy, cataract and diabetes. In conclusion, the applied therapeutic approaches in Piedmont reflect the difficulty reported in the literature in identifying simple recommendations. ACTH and rituximab are increasingly preferred for the treatment of MGN and there is a need for prospective studies to determine the best protocol for rituximab and the safety profile of ACTH.
膜性肾小球肾炎(MGN)的治疗存在争议,尤其是在对一线治疗无反应或多次复发的情况下。皮埃蒙特临床肾脏病小组对皮埃蒙特15个肾脏病科室中受MGN影响的患者治疗情况进行了多中心分析。对于肾病范围蛋白尿但肾功能正常或轻度受损的情况,通常在等待3 - 6个月后开始首次治疗。癌症病史、传染病的存在以及MGN的继发性形式是排除治疗的标准。作为一线治疗,皮埃蒙特的肾病专家开处皮质类固醇与免疫抑制药物交替使用,一般更倾向于环磷酰胺而非苯丁酸氮芥。只有一个肾脏病科室将环孢素A(CyA)作为首选。如果治疗无反应,在2 - 12个月后采取第二种治疗方法。二线治疗在之前使用免疫抑制药物的情况下为CyA,如果首次治疗为CyA则为皮质类固醇/免疫抑制药物。进一步的选择可能是促肾上腺皮质激素(ACTH)或利妥昔单抗。在多次复发的情况下,治疗选择相同,但先前的免疫抑制治疗、患者年龄以及肾病持续时间(肾衰竭和向硬化进展的可能性更大)需要仔细关注。人们对ACTH可能的严重副作用表示担忧,包括肌病、白内障和糖尿病。总之,皮埃蒙特应用的治疗方法反映了文献中报道的难以确定简单建议的情况。ACTH和利妥昔单抗在MGN治疗中越来越受青睐,需要进行前瞻性研究以确定利妥昔单抗的最佳方案和ACTH的安全性。