Isolated renal sarcoidosis: a rare presentation of a rare disease treated with infliximab
Isolated renal sarcoidosis: a rare presentation of a rare disease treated with infliximab
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DOI：
10.1007/s10067-006-0357-4
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发表时间：
2007-08-01
影响因子：
3.4
通讯作者：
Dossabhoy, Neville R.
中科院分区：
文献类型：
--
作者：
Ahmed, M. Mubashir;Mubashir, Eisha;Dossabhoy, Neville R.
结节病是一种多系统疾病，以非干酪化性肉芽肿反应为特征，常累及淋巴结、肺、肝、皮肤和眼睛。急性肾衰竭（ARF）作为结节病的一个孤立表现是罕见的。我们描述一例结节病表现为暂时性多关节炎和急性肾功能衰竭，由于孤立的肉芽肿性浸润肾实质。肾活检显示肉芽肿性间质性肾炎伴非干酪化性肉芽肿，符合结节病。排除细菌、真菌和分枝杆菌感染。没有证据表明肾外结节病累及。泼尼松60毫克，每日导致肾功能显着改善。由于类固醇减量和类固醇毒性的复发性发作，开始使用英夫利西单抗（一种抗肿瘤坏死因子-α（TNF-α）抗体）治疗，尽管类固醇减量，但仍导致肾功能稳定。虽然不常见，但在病因不明的急性或慢性肾衰竭的鉴别诊断中应考虑肾结节病，因为早期诊断和治疗可导致肾功能恢复并防止间质纤维化。皮质类固醇是治疗的主要药物。类固醇依赖性或难治性病例可能对其他免疫抑制剂（包括抗TNF-α药物）有反应。
Sarcoidosis is a multisystem disease characterized by noncaseating granulomatous reaction frequently involving the lymph nodes, lungs, liver, skin, and eyes. Acute renal failure (ARF) as an isolated manifestation of sarcoidosis is rare. We describe a case of sarcoidosis presenting as transient polyarthritis and ARF due to isolated granulomatous infiltration of the renal parenchyma. Renal biopsy showed granulomatous interstitial nephritis with noncaseating granulomas consistent with sarcoidosis. Bacterial, fungal, and mycobacterial infections were excluded. There was no evidence of extrarenal sarcoid involvement. Prednisone of 60 mg daily resulted in significant improvement in renal function. Because of recurrent flares on steroid taper and steroid toxicity, treatment with infliximab, an anti-tumor necrosis factor-alpha (TNF-alpha) antibody, was instituted and resulted in stabilization of renal function despite steroid taper. Although uncommon, renal sarcoidosis should be considered in the differential diagnosis of acute or chronic renal failure of uncertain etiology, as early diagnosis and treatment can lead to recovery of renal function and prevent interstitial fibrosis. Corticosteroids are mainstay of therapy. Steroid-dependant or refractory cases may respond to other immunosuppressants including anti-TNF-alpha agents.