一名20多岁无病史的女性出现进行性腹胀,右侧腹部不适,疲劳和恶心。检查显示多灶性淋巴结肿大,肝肿大伴紧张腹水。研究表明,多系统炎症状态的特征是急性期反应物升高,贫血,血小板减少症,急性肾损伤,淋巴细胞性腹水,低白蛋白血症和高丙种球蛋白血症。HIV和人类疱疹病毒8测试均为阴性。在ANA和SS-A/Ro抗体升高的情况下,该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)。临床和实验室检查结果符合SLE的诊断标准。然而,淋巴结活检显示滤泡间浆细胞增多,与高白细胞介素6(IL-6)和血管内皮生长因子滴度相关,共同暗示对多中心Castleman病(MCD)的罕见诊断。随着我们进一步调查,肾活检与MCD先前报道的血栓性微血管病一致.此外,肾活检免疫染色为“全室”免疫球蛋白和补体染色模式阴性,对狼疮性肾炎有特异性,帮助我们排除SLE.鉴于这些新发现,患者开始接受抗IL-6治疗,结果成功.
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.