Renal Division, Department of Medicine, Peking University First Hospital, Institute of Nephrology, Peking University, Beijing, China
Nephrol Dial Transplant. 2012 Oct;27 Suppl 3:iii119-25. doi: 10.1093/ndt/gfr245. Epub 2011 May 19.
The association of kidney disease with Castleman disease (CD) is uncommon. To date, most studies have been based on single-case reports. Here, we describe renal involvement in CD in a large Chinese cohort.
Seventy-six CD patients were identified in one clinical center. Clinical and pathological characteristics of patients with renal involvement were described, which were also compared with cases identified through a systematic literature review.
Nineteen patients (25%) exhibited renal involvement. Patients with multicentric clinical type (59 versus 0%) or plasma cell (PC)/mixed cellularity histological variant (61.5 versus 6%) were more likely to have renal involvement (P < 0.001). Proteinuria (with 7/19 reaching nephrotic range) and acute renal failure (12/19, 63%) were the main clinical presentations. Kidney biopsy revealed various glomerular diseases (10/11) and interstitial nephritis (1/11), while with 'thrombotic microangiopathy-like' lesions were the most common pathological characteristics (6/11, 55%). This contrasted significantly with the literature in which amyloidosis was the most reported. Renal outcomes responded well to chemotherapy. Nine (9/12, 75%) patients with acute renal failure recovered completely, one recovered partially. Overall, only three (3/19, 16%) patients progressed to end-stage kidney disease. Renal involvement did not influence survival rate (log-rank test, P = 0.73) in the follow-up.
CD with multicentric type and PC or mixed cellularity variant are often associated with renal complications. Thrombotic microangiopathy-like lesions are the most common pathological characteristics. Chemotherapy can reverse kidney damage in most cases.
肾脏疾病与 Castleman 病（CD）的关联并不常见。迄今为止，大多数研究都是基于单个病例报告。在此，我们描述了一个大型中国队列中 CD 患者的肾脏受累情况。
在一个临床中心确定了 76 名 CD 患者。描述了有肾脏受累的患者的临床和病理特征，并与系统文献复习中确定的病例进行了比较。
19 名患者（25%）存在肾脏受累。多中心临床型（59%比 0%）或浆细胞（PC）/混合细胞型组织学变异型（61.5%比 6%）的患者更有可能发生肾脏受累（P<0.001）。蛋白尿（19 例中有 7 例达到肾病范围）和急性肾衰竭（19 例中有 12 例，63%）是主要的临床表现。肾脏活检显示各种肾小球疾病（11 例中的 10 例）和间质性肾炎（11 例中的 1 例），而“血栓性微血管病样”病变是最常见的病理特征（11 例中的 6 例，55%）。这与文献报道的淀粉样变性形成鲜明对比。肾脏结局对化疗反应良好。急性肾衰竭的 9 例（12 例中的 9 例，75%）患者完全恢复，1 例部分恢复。总的来说，只有 3 例（19 例中的 3 例，16%）患者进展为终末期肾病。肾脏受累在随访中并未影响生存率（对数秩检验，P=0.73）。
多中心型和 PC 或混合细胞型 CD 常伴有肾脏并发症。血栓性微血管病样病变是最常见的病理特征。大多数情况下，化疗可逆转肾脏损伤。
