Medizinische Klinik A, Klinikum der Stadt Ludwigshafen.
V. Medizinische Klinik, Universitätsklinikum Mannheim, Germany.
Curr Opin Pulm Med. 2018 Sep;24(5):513-520. doi: 10.1097/MCP.0000000000000504.
The purpose of this article is to provide understanding of renal sarcoidosis, the different types of renal sarcoidosis, disease burden of renal involvement, and treatment options.
The frequency of renal involvement seems to be underestimated, but renal sarcoidosis represents a relevant group of organ manifestations and significantly adds to the patient's morbidity. Because histopathological analysis of renal biopsy specimens can reveal various entities, a diagnostic workup is necessary in every patient with sarcoidosis.
If systematically screened for renal manifestations are likely to occur in up to 25-30% of all sarcoidosis patients. The most common histological form of renal sarcoidosis is the granulomatous interstitial nephritis; however, granulomas can be absent. Furthermore, one can find various forms of secondary glomerulonephritis. In cases with dysregulated calcium homeostasis, nephrocalcinosis and nephrolithiasis are commonly detectable kidney diseases. AA amyloidosis or renal masses because of granuloma formation are considered to be rare manifestations. In addition to glucocorticoids various immunosuppressive treatments such as tumor necrosis factor alpha inhibitors have proven to be effective based on case series.
本文旨在帮助理解肾结节病，包括不同类型的肾结节病、肾受累的疾病负担以及治疗选择。
肾受累的频率似乎被低估了，但肾结节病是一组重要的器官表现，显著增加了患者的发病率。因为肾活检标本的组织病理学分析可能揭示各种病变，所以对每个结节病患者都需要进行诊断性评估。
如果系统筛查，肾表现可能发生在高达 25-30%的所有结节病患者中。最常见的肾结节病组织学形式是肉芽肿性间质性肾炎；然而，也可能不存在肉芽肿。此外，还可以发现各种形式的继发性肾小球肾炎。在钙稳态失调的情况下，常可检测到肾钙质沉着症和肾结石等肾脏疾病。AA 淀粉样变性或因肉芽肿形成导致的肾肿块被认为是罕见的表现。除了糖皮质激素，基于病例系列研究，各种免疫抑制剂如肿瘤坏死因子-α抑制剂已被证明是有效的。
