Clinicopathological Characteristics and Long-Term Kidney Outcomes in Biopsy-Proven Renal Sarcoidosis.

IF 3.2 Q1 UROLOGY & NEPHROLOGY
Kidney360 Pub Date : 2025-06-02 DOI:10.34067/KID.0000000842
Jing Miao, Charat Thongprayoon, Wisit Cheungpasitporn, Alessia Buglioni, Ladan Zand, Fernando Fervenza
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Abstract

Background: Renal sarcoidosis (RS) is a rare but potentially severe manifestation of sarcoidosis, primarily affecting the kidneys through granulomatous interstitial nephritis (GIN) and calcium metabolic disturbances. This study evaluates the clinicopathological features and renal outcomes of biopsy-proven RS, focusing on identifying predictors of renal recovery and disease progression.

Methods: This retrospective study included 43 biopsy-proven RS at Mayo Clinic (2012-2024). Demographic, clinical, laboratory, and histopathological data were analyzed. Renal recovery within six months was classified as complete, partial, or no recovery based on serum creatinine (sCr) changes. Long-term adverse composite outcomes included the chronic kidney disease (CKD) onset, CKD progression, end-stage kidney disease, dialysis initiation, or death. Logistic regression and Cox proportional hazards regression were used to assess predictors of renal outcomes.

Results: Among cohort, 84% exhibited interstitial nephritis, with GIN in 33%. Calcium phosphate deposits were found in 58% of cases. Hypercalciuria (90%) was more common than hypercalcemia (74%). Within six months, 86% achieved recovery (74% complete, 12% partial), while 40% experienced long-term adverse composite outcomes. Higher sCr at biopsy (≥ 2.4 mg/dL) and moderate-to-severe interstitial fibrosis and tubular atrophy (IFTA) were associated with reduced complete recovery and worse long-term outcomes; while pre-existing CKD significantly increased the risk of adverse long-term outcomes. Steroid therapy significantly increased the likelihood of complete recovery and reduced the risk of adverse long-term outcomes.

Conclusions: Pre-existing CKD, elevated sCr at biopsy, IFTA, and steroid therapy are key predictors of renal outcomes in RS. Early diagnosis and intervention are crucial to preventing irreversible kidney damage.

活检证实的肾结节病的临床病理特征和长期肾脏预后。
背景:肾结节病(RS)是一种罕见但潜在严重的结节病表现,主要通过肉芽肿性间质性肾炎(GIN)和钙代谢紊乱影响肾脏。本研究评估活检证实的RS的临床病理特征和肾脏预后,重点是确定肾脏恢复和疾病进展的预测因素。方法:本回顾性研究包括2012-2024年在梅奥诊所活检证实的43例RS。对人口统计学、临床、实验室和组织病理学数据进行分析。根据血清肌酐(sCr)变化,6个月内肾脏恢复分为完全恢复、部分恢复或无恢复。长期不良综合结局包括慢性肾脏疾病(CKD)发病、CKD进展、终末期肾脏疾病、透析开始或死亡。采用Logistic回归和Cox比例风险回归评估肾脏预后的预测因素。结果:在队列中,84%表现为间质性肾炎,33%表现为GIN。58%的病例发现磷酸钙沉积。高钙尿(90%)比高钙血症(74%)更为常见。在6个月内,86%的患者实现了康复(74%完全康复,12%部分康复),而40%的患者出现了长期的不良综合结果。活检时较高的sCr(≥2.4 mg/dL)和中重度间质纤维化和小管萎缩(IFTA)与完全恢复减少和较差的长期预后相关;而先前存在的CKD显著增加了不良长期预后的风险。类固醇治疗显著增加了完全恢复的可能性,降低了不良长期结果的风险。结论:预先存在的CKD,活检时sCr升高,IFTA和类固醇治疗是RS肾脏预后的关键预测因素,早期诊断和干预对于预防不可逆肾损害至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Kidney360
Kidney360 UROLOGY & NEPHROLOGY-
CiteScore
3.90
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0.00%
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