Internal Medicine, Albany Medical Center, Albany, New York, USA
Pathology, Albany Medical Center, Albany, New York, USA.
BMJ Case Rep. 2022 Aug 12;15(8):e250706. doi: 10.1136/bcr-2022-250706.
A woman in her 20s with no medical history presented with progressive abdominal distension, right-sided abdominal discomfort, fatigue and nausea. Examination showed multifocal lymphadenopathy and hepatomegaly with tense ascites. Investigations revealed a multisystem inflammatory condition characterised by elevated acute phase reactants, anaemia, thrombocytopenia, acute kidney injury, lymphocytic ascites, hypoalbuminaemia and hypergammaglobulinaemia. HIV and human herpes virus-8 tests were both negative. In the presence of elevated ANA and SS-A/Ro antibodies, the patient was suspected to be carrying a connective tissue disease, most likely systemic lupus erythematosus (SLE). Clinical and laboratory findings fulfilled the diagnostic criteria for SLE. However, lymph node biopsy showed interfollicular plasmacytosis, associated with high interleukin 6 (IL-6) and vascular endothelial growth factor titers, together hinting towards a rare diagnosis of multicentric Castleman's disease (MCD). As we investigated further, renal biopsy was consistent with thrombotic microangiopathy which has been previously reported in MCD. Furthermore, immune staining on the renal biopsy was negative for 'full-house' immunoglobulin and complement staining pattern, which is specific for lupus nephritis, helping us exclude SLE. In light of these new findings, the patient was started on anti-IL-6 therapy which provided a successful outcome.
一位 20 多岁的女性，无病史，出现进行性腹胀、右侧腹部不适、乏力和恶心。检查发现多发性淋巴结病和肝大伴紧张性腹水。检查显示多系统炎症状态，表现为急性期反应物升高、贫血、血小板减少、急性肾损伤、淋巴细胞性腹水、低白蛋白血症和高球蛋白血症。HIV 和人类疱疹病毒-8 检测均为阴性。鉴于抗核抗体和 SS-A/Ro 抗体升高，患者被怀疑患有结缔组织病，最有可能是系统性红斑狼疮（SLE）。临床和实验室发现符合 SLE 的诊断标准。然而，淋巴结活检显示滤泡间浆细胞增多，伴有高白细胞介素 6（IL-6）和血管内皮生长因子滴度，共同提示罕见的多中心 Castleman 病（MCD）诊断。随着我们进一步调查，肾活检符合血栓性微血管病，此前曾在 MCD 中报道过。此外，肾活检的免疫染色对“全套”免疫球蛋白和补体染色模式呈阴性，这是狼疮肾炎的特征，有助于我们排除 SLE。鉴于这些新发现，患者开始接受抗 IL-6 治疗，取得了成功的结果。
