Effective Treatment of Rapidly Deteriorated Renal Function with Methylprednisolone Pulse Therapy and High Dose Mycophenolate Mofetil in A Patient with Lupus Nephritis

使用甲基類固醇脈衝療法和高劑量Mycophenolate Mofetil有效治療一位狼瘡腎炎病人之迅速惡化腎功能

王明熙(Ming-Hsi Wang)

《內科學誌》 23卷1期 (2012/02) Pp. 59-65

https://doi.org/10.6314/JIMT.2012.23(1).08

摘要

三十歲非洲裔美國女性病患過去曾有Graves'疾病及未明確性結締組織疾病，因呼吸困難及全身性水腫住院。生化檢查發現血清尿素氮為60mg/dL，肌酸酐為4.6mg/dL，血清白蛋白2.1g/dL，每天尿蛋白流失4.6公克。血清補體C3 78mg/dL輕度下降，C4 36mg/dL在正常範圍值。抗核抗體效價為1：640呈現班點狀型態，而且抗雙鏈DNA抗體為陽性。血中狼瘡抗凝劑試驗為陰性。腎臟切片在光學顯微鏡下呈現局部分段式增生性狼瘡腎炎，螢光顯微鏡檢發現full house免疫沈積，電子顯微鏡發現廣泛性增大型足細胞以及足禿的退化跡象，以及在內皮細胞內發現極具有特徵性管泡狀網狀內含體。患者接受甲基類固醇脈衝治療，每天1公克注射3天後，維持口服類固醇每天60毫克治療。同時合併施與mycophenolate mofetil口服1000毫克每天兩次，隨後逐漸減少劑量。治療後第五天尿蛋白流失情況及血清肌酸酐明顯改善，病患之腎變病綜合症也於治療後三周改善消失。本文將進一步探討此臨床病案之鑑別診斷，疾病相關之危險因子，狼瘡腎炎之臨床表現與病理診斷分類及特徵，以及討論狼瘡腎炎的治療方式與預後相關因子。

並列摘要

A 30 year old African American female with Graves' disease and unspecified connective tissue disorder was admitted because of orthopnea and general edema. Laboratory testing showed blood urea nitrogen levels of 60 mg/dL, creatinine of 4.6 mg/dL, serum albumin of 2.1 g/dL and the daily urinary protein loss was 4.6 g. C3 level was 78 mg/dL and C4 level was 36 mg/dL. The antinuclear antibody titer was 1:640, with a speckled pattern, and anti-ds DNA was positive. She was negative for lupus anticoagulant. Renal biopsy revealed focal segmental proliferative lupus nephritis, and immunofluorescent microscopy showed full house immune deposits and electron microscopy revealed diffusely enlarged podocytes with foot process effacement and characteristic tubuloreticular inclusions in endothelial cells. She received 1 g/ day of pulse methylprednisolone intravenously for 3 days, followed by 60 mg/day of prednisone. She was also started with mycophenolate mofetil 1000mg twice a day and followed by decreased dosage. Heavy proteinuria and serum creatinine level started improving after 5 days of treatment and nephrotic syndrome remitted completely in 3 weeks. The differential diagnosis, clinical risk factors and manifestations, prognostic predictors and treatment are discussed.

並列關鍵字

Lupus nephritis ； Systemic lupus erythematosus ； Nephrotic syndrome