El-Husseini Amr, Sabucedo Alberto J, Lamarche Jorge, Courville Craig, Peguero Alfredo
James A. Haley Veterans Hospital, University of South Florida, Tampa, USA.
Division of Nephrology, Bone and Mineral Metabolism, University of Kentucky Chandler Medical Center, 800 Rose Street, MN-564, Lexington, KY, 40536-0298, USA.
CEN Case Rep. 2013 May;2(1):102-106. doi: 10.1007/s13730-012-0052-z. Epub 2012 Dec 11.
Sarcoidosis is a multi-organ disease of unknown etiology characterized by non-caseating granulomas. Here we report the case of a 78-year-old white male with a past medical history of diabetes mellitus, hypertension, and chronic kidney disease stage III with a baseline serum creatinine of 2.5 mg/dl. The patient had a prior admission history for acute kidney injury (AKI) attributed to dehydration and medication-induced nephro-toxicities. He presented to the renal clinic for follow-up with acute worsening of chronic kidney failure with a serum creatinine level of 3.5 mg/dl. Examination revealed that he was anemic and mildly hypercalcemic with suppressed parathyroid hormone and had proteinuria of 1.3 g per day. The computed tomography scan of the abdomen revealed right renal pelvic non-obstructing calculi. Serum protein electrophoresis revealed gammopathy with two distinct monoclonal peaks consisting of immunoglobulin G (IgG) kappa and IgG lambda, respectively. The kappa/lambda ratio was within normal limits, and urine protein electrophoresis showed no evidence of a monoclonal peak or Bence Jones proteins. Further workup for multiple myeloma, including bone marrow (BM) biopsy, revealed polyclonal plasma cells and B cells with no clonality. No morphological and immune-phenotypic evidence of plasma cell dyscrasia was found, but BM biopsy did show numerous non-caseating granulomas consistent with sarcoidosis. Skin biopsy from non-scaly 6-mm skin colored papule also showed non-caseating granulomas. The patient had elevated angiotensin-converting enzyme levels (165 ug/l) and an erythrocyte sedimentation rate of 27 mm/h. Kidney biopsy did not show granulomas. The hypercalcemia, proteinuria, and AKI responded well after 2 weeks of 60 mg oral prednisone daily.
结节病是一种病因不明的多器官疾病,其特征为非干酪样肉芽肿。在此,我们报告一例78岁白人男性病例,其既往有糖尿病、高血压病史,慢性肾脏病Ⅲ期,基线血清肌酐为2.5mg/dl。该患者既往曾因脱水和药物性肾毒性导致急性肾损伤(AKI)入院。他因慢性肾衰竭急性加重前来肾脏科门诊随访,血清肌酐水平为3.5mg/dl。检查发现他贫血,轻度高钙血症,甲状旁腺激素受抑制,每日蛋白尿1.3g。腹部计算机断层扫描显示右肾盂无梗阻性结石。血清蛋白电泳显示丙种球蛋白病,有两个不同的单克隆峰,分别由免疫球蛋白G(IgG)κ和IgGλ组成。κ/λ比值在正常范围内,尿蛋白电泳未显示单克隆峰或本周氏蛋白的证据。对多发性骨髓瘤的进一步检查,包括骨髓(BM)活检,发现多克隆浆细胞和B细胞,无克隆性。未发现浆细胞发育异常的形态学和免疫表型证据,但BM活检确实显示了许多与结节病一致的非干酪样肉芽肿。取自无鳞屑的6mm皮肤色丘疹的皮肤活检也显示非干酪样肉芽肿。患者血管紧张素转换酶水平升高(165μg/l),红细胞沉降率为27mm/h。肾脏活检未显示肉芽肿。每日口服60mg泼尼松治疗2周后,高钙血症、蛋白尿和AKI均有良好反应。