Deniz Rabia, Hacımurtazaoğlu-Demir Gülşah, Karaalioğlu Bilgin, Özgür Duygu Sevinç, Akkuzu Gamze, Yıldırım Fatih, Bes Cemal
Department of Rheumatology, Başakşehir Çam and Sakura City Hospital, University of Health Sciences, Istanbul, Turkey.
Department of Internal Medicine, University of Health Sciences Bağcılar Education and Research Hospital, Istanbul, Turkey.
J Rheum Dis. 2024 Apr 1;31(2):116-119. doi: 10.4078/jrd.2023.0052. Epub 2024 Jan 17.
The triad of ascites, pleural effusion, and elevated cancer antigen-125 (CA-125) levels in the absence of ovarian malignancy in systemic lupus erythematosus patients is specifically named pseudo-pseudo Meigs' syndrome (PPMS) or Tjalma syndrome. In this case we reported a 33 years female patient with pleural effusion lasting for 3 years and new onset progressive massive ascites and increased level of CA-125. After she was evaluated for an underlying benign and malign ovarian tumor or any other malignancies, serologic tests were requested with respect to progressive renal dysfunction, proteinuria, lymphopenia, anemia, and effusion. She was diagnosed with systemic lupus erythamatosus (SLE) and renal biopsy showed class-V lupus nephritis. Immunosuppressive treatment led to improvement in both SLE activity and components of PPMS, including massive ascites and pleural effusion and without the need of diuretics. Co-existence of unexplained CA-125 increase, pleural effusion, and ascites might be related to PPMS and detailed examination to exclude malignancy and early and effective treatment of SLE are the mainstay of management.
系统性红斑狼疮患者在无卵巢恶性肿瘤的情况下出现腹水、胸腔积液和癌抗原125（CA - 125）水平升高的三联征被特别命名为假性梅格斯综合征（PPMS）或恰尔马综合征。在本病例中，我们报告了一名33岁女性患者，胸腔积液持续3年，新发进行性大量腹水且CA - 125水平升高。在对其进行潜在的良性和恶性卵巢肿瘤或任何其他恶性肿瘤评估后，针对进行性肾功能不全、蛋白尿、淋巴细胞减少、贫血和积液进行了血清学检查。她被诊断为系统性红斑狼疮（SLE），肾活检显示为Ⅴ型狼疮性肾炎。免疫抑制治疗使SLE活动度以及PPMS的各项表现得到改善，包括大量腹水和胸腔积液，且无需使用利尿剂。无法解释的CA - 125升高、胸腔积液和腹水并存可能与PPMS有关，详细检查以排除恶性肿瘤以及对SLE进行早期有效治疗是管理的主要内容。
