Rauen T, Michaelis A, Floege J, Mertens P R
Department of Nephrology and Clinical Immunology, RWTH-University Hospital, Aachen.
Clin Nephrol. 2009 Jun;71(6):637-42. doi: 10.5414/cnp71637.
In patients with idiopathic membranous nephropathy (IMN), immunosuppressive therapy is usually considered when severe nephrotic syndrome or risk for progressive renal failure exist. Recently, several studies showing beneficial effects of synthetic adrenocorticotropic hormone (ACTH) under such circumstances have been published. The objective of the present case series was to evaluate long-term ACTH effects on proteinuria and renal function.
Four patients with biopsy-proven membranous nephropathy and nephrotic syndrome were enrolled (median age 50 years (range 38 - 61), median GFR 39.5 ml/min (range 20 - 62), median proteinuria 9.6 g/d (range 6.0 - 20.0). Prior immunosuppressive treatment regimens included steroids, cyclosporine A, cyclophosphamide, mycophenolate mofetil or azathioprine. The patients received a synthetic ACTH analogue intramuscularly for a median duration of 8 months (range 3 - 24). ACTH dosage was adjusted according to side effects between 0.25 and 2.25 mg/week. Follow-up lasted between 24 and 82 months after therapy initiation.
All 4 patients exhibited partial (n = 2) or complete (n = 2) remissions of their nephrotic syndrome within the first year. After discontinuation of ACTH therapy, proteinuria remained low in 3 of 4 cases, whereas 1 patient exhibited undulating proteinuria. Glomerular function (as assessed by glomerular filtration rate, GFR) was maintained in all patients. Side effects were minor and included weight gain, elevated blood pressure and hyperglycemia.
In all 4 cases with IMN, ACTH treatment induced a lasting disease remission with relatively few side effects.
在特发性膜性肾病(IMN)患者中,当存在严重肾病综合征或进行性肾衰竭风险时,通常会考虑免疫抑制治疗。最近,有几项研究发表,显示在这种情况下合成促肾上腺皮质激素(ACTH)具有有益作用。本病例系列的目的是评估ACTH对蛋白尿和肾功能的长期影响。
纳入4例经活检证实为膜性肾病和肾病综合征的患者(中位年龄50岁(范围38 - 61岁),中位肾小球滤过率(GFR)39.5 ml/分钟(范围20 - 62),中位蛋白尿9.6 g/天(范围6.0 - 20.0)。先前的免疫抑制治疗方案包括类固醇、环孢素A、环磷酰胺、霉酚酸酯或硫唑嘌呤。患者接受合成ACTH类似物肌肉注射,中位持续时间为8个月(范围3 - 24个月)。ACTH剂量根据副作用在0.25至2.25 mg/周之间进行调整。治疗开始后随访持续24至82个月。
所有4例患者在第一年内均出现肾病综合征部分缓解(n = 2)或完全缓解(n = 2)。停用ACTH治疗后,4例中有3例蛋白尿维持在低水平,而1例患者蛋白尿呈波动状态。所有患者的肾小球功能(通过肾小球滤过率GFR评估)均得以维持。副作用较小,包括体重增加、血压升高和高血糖。
在所有4例IMN患者中,ACTH治疗诱导了持久的疾病缓解,且副作用相对较少。