Gao Fei, Xu YongMei, Yang GuoWang
Department of Hematology & Oncology, Beijing Hospital of Traditional Chinese Medicine, Clinical Medical College of Traditional Chinese Medicine, Capital Medical University, Beijing, China.
Medicine (Baltimore). 2019 Apr;98(17):e15393. doi: 10.1097/MD.0000000000015393.
Combination of polyserositis and elevated serum CA 125 is common in tumor or infectious disease, but this clinical combination is also found in other diseases. It could be the initial manifestation of pseudo-pseudo Meigs' syndrome (PPMS), which is characterized by the presence of polyserositis and raised CA-125 level in systemic lupus erythematosus (SLE).
PATIENT'S CONCERNS: A 44-year-old Chinese female was admitted with three months history of painless abdominal distension accompanied by watery diarrhea 5-6 times daily, shortness of breath, fatigue, lower limb swelling, and 10 kg weight loss. The test results showed peripheral cytopenias, hypoproteinemia, renal dysfunction and elevated CA 125, antidouble-stranded DNA antibodies, and anti-Sjogren's syndrome A antigen antibody was positive. There is no evidence for the diagnosis of solid tumor according to the results of imaging modality and pathological examination.
The patient was diagnosed as pseudo-pseudo Meigs syndrome.
The patient received hormone, leflunomide, and Plaquenil therapy.
The patient's symptoms were relieved and the laboratory index was improved after the treatment of hormone and immunosuppressant.
PPMS is characterized by the combination of serous effusion and elevated serum CA 125 with no evidence of tumor among SLE patients. Clinicians should be aware of the diagnosis of PPMS avoiding unnecessary anxiety or surgical interventions.
多浆膜炎和血清CA 125升高在肿瘤或感染性疾病中很常见,但这种临床组合也见于其他疾病。它可能是假性梅格斯综合征(PPMS)的初始表现,其特征是在系统性红斑狼疮(SLE)中存在多浆膜炎和CA-125水平升高。
一名44岁中国女性因无痛性腹胀3个月入院,伴有每日5至6次水样腹泻、呼吸急促、疲劳、下肢肿胀和体重减轻10公斤。检查结果显示外周血细胞减少、低蛋白血症、肾功能不全以及CA 125升高,抗双链DNA抗体和抗干燥综合征A抗原抗体呈阳性。根据影像学检查和病理检查结果,没有实体瘤的诊断证据。
患者被诊断为假性梅格斯综合征。
患者接受了激素、来氟米特和羟氯喹治疗。
经激素和免疫抑制剂治疗后,患者症状缓解,实验室指标改善。
PPMS的特征是SLE患者出现浆液性积液和血清CA 125升高且无肿瘤证据。临床医生应了解PPMS的诊断,避免不必要的焦虑或手术干预。