Bindal Tanvi, Govindarajan Srinivasavaradan, Barwad Adarsh, Naranje Priyanka, Damle Nishikant Avinash, Hari Pankaj, Sinha Aditi, Bagga Arvind
Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India.
Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.
Pediatr Nephrol. 2025 Sep 9. doi: 10.1007/s00467-025-06939-3.
Kidney involvement in pediatric sarcoidosis is rare and often underrecognized, leading to diagnostic delays and treatment challenges. We report six patients with renal sarcoidosis to highlight their diverse presentations and outcomes and challenges in management.
Medical records of patients diagnosed with renal sarcoidosis during 2020-24 were reviewed. Sarcoidosis was diagnosed based on clinical and histological features and exclusion of alternative causes and managed according to unit protocols. Information on clinical features, laboratory and radiologic findings, histopathology, treatment, and follow-up were compiled.
We present six patients with sarcoidosis, presenting with kidney involvement at the age of 1.5-14 years, and followed up for 7-138 months. All patients had acute kidney injury (AKI) of whom two required hemodialysis. Proteinuria was present in all patients, while four patients had microscopic hematuria or leukocyturia. Hypercalcemia with hypercalciuria, distal renal tubular acidosis, and nephrocalcinosis were seen in five, two, and one case, respectively. Granulomatous interstitial nephritis was confirmed histologically in all cases. While initial therapy with corticosteroids led to clinical remission in all cases, five patients had nine relapses, necessitating second-line immunosuppression with mycophenolate mofetil, azathioprine, or methotrexate; one patient received antitumor necrosis factor therapy. Median eGFR at last follow up was 59.7 (range 12.7-132) ml/min/1.73 m; three progressed to chronic kidney disease (CKD) stages G3-G5.
Kidney involvement in pediatric sarcoidosis manifests in diverse forms, ranging from isolated biochemical abnormalities to severe AKI. While prompt immunosuppression might preserve kidney function, patients require close monitoring for relapses, and progression to CKD.
小儿结节病累及肾脏较为罕见，且常未得到充分认识，导致诊断延误和治疗困难。我们报告6例肾结节病患者，以突出其多样的临床表现、结局及管理挑战。
回顾2020年至2024年期间诊断为肾结节病患者的病历。结节病根据临床和组织学特征诊断，并排除其他病因，按照科室方案进行管理。收集临床特征、实验室及影像学检查结果、组织病理学、治疗及随访信息。
我们报告6例结节病患者，年龄1.5至14岁，肾脏受累，随访7至138个月。所有患者均有急性肾损伤（AKI），其中2例需要血液透析。所有患者均有蛋白尿，4例有镜下血尿或白细胞尿。分别有5例、2例和1例出现高钙血症伴高钙尿症、远端肾小管酸中毒和肾钙质沉着症。所有病例经组织学证实为肉芽肿性间质性肾炎。虽然初始使用糖皮质激素治疗使所有病例临床缓解，但5例患者复发9次，需要使用霉酚酸酯、硫唑嘌呤或甲氨蝶呤进行二线免疫抑制；1例患者接受抗肿瘤坏死因子治疗。末次随访时估算肾小球滤过率（eGFR）中位数为59.7（范围12.7至132）ml/min/1.73 m²；3例进展为慢性肾脏病（CKD）G3至G5期。
小儿结节病累及肾脏表现形式多样，从孤立的生化异常到严重的AKI。虽然及时的免疫抑制可能保留肾功能，但患者需要密切监测复发情况及是否进展为CKD。
