Dirim Ahmet Burak, Süleymanova Vafa, Hürdoğan Özge, Oto Özgür Akın, Artan Ayşe Serra, Öztürk Savaş, Özlük Yasemin, Kiliçaslan Işın, Yazici Halil
Division of Nephrology, Department of Internal Medicine, Faculty of Medicine, İstanbul University, İstanbul, Turkiye.
Department of Pathology, Faculty of Medicine, İstanbul University, İstanbul, Turkiye.
Turk J Med Sci. 2024 Oct 8;54(6):1252-1264. doi: 10.55730/1300-0144.5907. eCollection 2024.
BACKGROUND/AIM: Sarcoidosis is a multisystem disorder that affects many organs, including the kidneys. This single-center retrospective study investigated the clinical, pathological, and laboratory findings of patients with kidney sarcoidosis who were treated with immunosuppressives.
Twenty-three patients with biopsy-confirmed kidney sarcoidosis were included. Demographic, clinical, pathological, and laboratory findings, in addition to the treatments and outcomes of 20 patients with at least one month of follow-up were evaluated.
The median age of the patients at the time of biopsy was 47 years (60.9% were female). The median baseline estimated glomerular filtration rate (eGFR) and proteinuria were 21.5 mL/min and 1 g/g or g/day, respectively. Nineteen of the 23 patients were diagnosed with nonglomerular disease (four had glomerular diseases). Extrarenal sarcoidosis was present in 86.7% of the patients. Granulomatous interstitial nephritis (56.5 %) and nephrosclerosis with intratubular calcific casts (17.4 %) were the two most common diagnoses. All the patients initially received 1 mg/kg/day steroids for kidney involvement. Although no statistical difference was observed in kidney function during the follow-up, steroids improved the eGFR in the first month compared with baseline in patients with nonglomerular diseases (p = 0.049). Eventually, 45% of the patients developed end-stage kidney disease, and 45% of cohort had a treatment response. Patients with higher baseline calcium levels (p = 0.03) and lower degrees of interstitial fibrosis/tubular atrophy (p = 0.043) had better kidney outcomes. Moreover, none of the patients with sarcoidosis-related secondary glomerular disease had a treatment response (p = 0.043).
Hypercalcemia and lower interstitial fibrosis and tubular atrophy rates might be associated with better outcomes in sarcoidosis-related kidney involvement under immunosuppressive treatment. Moreover, late diagnosis, irregular follow-up, and glomerular disorders could be poor prognostic factors.
背景/目的：结节病是一种多系统疾病，可累及包括肾脏在内的多个器官。这项单中心回顾性研究调查了接受免疫抑制剂治疗的肾结节病患者的临床、病理和实验室检查结果。
纳入23例经活检确诊的肾结节病患者。评估了患者的人口统计学、临床、病理和实验室检查结果，以及20例至少随访1个月的患者的治疗情况和结局。
活检时患者的中位年龄为47岁（60.9%为女性）。基线估计肾小球滤过率（eGFR）和蛋白尿的中位数分别为21.5 mL/分钟和1 g/g或g/天。23例患者中有19例被诊断为非肾小球疾病（4例患有肾小球疾病）。86.7%的患者存在肾外结节病。肉芽肿性间质性肾炎（56.5%）和伴有肾小管内钙化管型的肾硬化（17.4%）是最常见的两种诊断。所有患者最初均接受1 mg/kg/天的类固醇治疗肾脏受累情况。尽管随访期间肾功能无统计学差异，但与非肾小球疾病患者的基线相比，类固醇在第一个月改善了eGFR（p = 0.049）。最终，45%的患者发展为终末期肾病，45%的队列有治疗反应。基线钙水平较高（p = 0.03）和间质纤维化/肾小管萎缩程度较低（p = 0.043）的患者肾脏结局较好。此外，结节病相关继发性肾小球疾病患者均无治疗反应（p = 0.043）。
高钙血症以及较低的间质纤维化和肾小管萎缩率可能与免疫抑制治疗下结节病相关肾脏受累的较好结局相关。此外，诊断延迟、随访不规律和肾小球疾病可能是不良预后因素。
