Department of Nephrology, Peking University Third Hospital, 49 Huayuanbei Road, Beijing, 100191, PR China.
BMC Nephrol. 2020 Dec 4;21(1):528. doi: 10.1186/s12882-020-02191-z.
Idiopathic multicentric Castleman disease (iMCD) is an uncommon lymphoproliferative disorder and lacks treatment consensus. Herein, we report a case of iMCD complicated with Sjögren's syndrome (SS) and secondary membranous nephropathy (SMN).
A 45-year-old female with dry mouth for 3 months and anasarca and proteinuria for 2 months was admitted. She also experienced chest tightness, wheezing, fever, weight loss, moderate proteinuria and hypoalbuminemia. A computed tomography (CT) scan revealed a tissue mass in the thymus area and enlarged multiple lymph nodes. Her symptoms did not improve after resection of the thymus mass. The pathological findings were "reactive hyperplasia of the mediastinal lymph nodes and thymic hyperplasia". Lymph node biopsy findings confirmed iMCD with human herpes virus-8 (HHV-8) negativity. Based on anti-nuclear antibody (ANA) 1:320, anti-SSA and anti-SSB antibody positivity, salivary flow less than 0.1 ml/min and lip biopsy with focal lymphocytic sialadenitis, SS was diagnosed. Kidney biopsy showed secondary membranous nephropathy with endocapillary cell proliferation and infiltration of plasma cells and lymphocytes in the tubulointerstitium. Serum interleukin-6 (IL-6) levels were significantly increased, and therapy with tocilizumab (anti-IL-6 receptor antibody) worked well. The combination of cyclophosphamide (CyS) with methylprednisolone (MP) maintained satisfactory remission.
Our case of iMCD with SS and SMN is rare. There is a need for increased awareness of the disease to avoid unnecessary procedures and misdiagnoses. IL-6 was extremely high, and there was a rapid response to anti-IL-6 receptor agents. The combination of CyS with MP maintained complete remission.
特发性多中心 Castleman 病（iMCD）是一种罕见的淋巴组织增生性疾病，目前尚无治疗共识。在此，我们报告一例 iMCD 合并干燥综合征（SS）和继发性膜性肾病（SMN）。
一名 45 岁女性，因口干 3 个月，浮肿和蛋白尿 2 个月入院。她还伴有胸闷、喘息、发热、体重减轻、中度蛋白尿和低白蛋白血症。计算机断层扫描（CT）显示胸腺区有组织肿块和多个淋巴结肿大。切除胸腺肿块后，她的症状并未改善。病理检查结果为“纵隔淋巴结反应性增生和胸腺增生”。淋巴结活检结果证实为 iMCD，人类疱疹病毒-8（HHV-8）阴性。基于抗核抗体（ANA）1:320、抗 SS-A 和抗 SS-B 抗体阳性、唾液流量小于 0.1ml/min 和唇活检有局灶性淋巴细胞性涎腺炎，诊断为 SS。肾活检显示继发性膜性肾病，伴有毛细血管内细胞增殖和肾小管间质中浆细胞和淋巴细胞浸润。血清白细胞介素-6（IL-6）水平显著升高，使用托珠单抗（抗 IL-6 受体抗体）治疗效果良好。环磷酰胺（CyS）联合甲基强的松龙（MP）的联合治疗维持了令人满意的缓解。
我们报告的 iMCD 合并 SS 和 SMN 病例较为罕见。需要提高对该病的认识，以避免不必要的检查和误诊。IL-6 极高，对抗 IL-6 受体药物有快速反应。CyS 联合 MP 可维持完全缓解。
