Kudsi Maysoun, Khalayli Naram, Hola Leen, Aldeeb Maria, Aziz Aghiad
Faculty of Medicine, Damascus University, Damascus, Syria.
Ann Med Surg (Lond). 2023 Nov 7;86(1):467-471. doi: 10.1097/MS9.0000000000001462. eCollection 2024 Jan.
Mixed connective tissue disease (MCTD) is characterized by high titres of distinct antibodies: U1 ribonucleoprotein with variable clinical features seen in rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis, and dermatomyositis. Limited case reports revealed the association between MCTD and cancer, like lymphoma, lung cancers, and others.
A 22-year-old female presented with enlargement of the abdomen and oedema of the lower extremities, gradually started 25 days The patient had been diagnosed to have rheumatoid arthritis. She was treated with 7.5 mg/week MTX for 6 months. Physical examination revealed: pallor, lower limb oedema, with synovitis and deformities of hands. The laboratory tests showed anaemia, elevated levels of creatine phosphokinase ESR, positivity of antinuclear antibody, anti-ds DNA, and antinuclear ribonucleoprotein. Urinary protein excretion was 1625 mg/24 h. Chest X-ray showed bilateral pleural effusion. Echocardiography revealed pericardial effusion Thoracic-abdominal and pelvic tomography showed a heterogeneous mass with a diameter of 5 × 6 cm at the expense of the right ovary. The mass was removed surgically, and a biopsy was taken, and was compatible with ovarian high-grade serous adenocarcinoma. A course of solumedrol 1 g/IV/3 days was applied, and then continue with 60 mg/day oral predlone. Later on discharge, she was taken 25 mg/day predlone, and methotrexate 10 mg.
Our case showed that the patient had no risk factors for developing ovary cancer. On the contrary, our patient was a young, non-smoker, without any previous treatment before the RA diagnosis was taken, and finally, she had 3 children with full-term pregnancy, and well health. This case highlights the importance of maintaining a high index of suspicion for malignancy in MCTD patients. However, further investigation on the role of the immune system in the development of ovarian cancer in women with autoimmune diseases including MCTD remains necessary.
混合性结缔组织病(MCTD)的特征是存在高滴度的特异性抗体:U1核糖核蛋白,伴有类风湿关节炎、系统性红斑狼疮、硬皮病、多发性肌炎和皮肌炎中可见的多种临床特征。有限的病例报告显示MCTD与癌症之间存在关联,如淋巴瘤、肺癌等。
一名22岁女性出现腹部增大和下肢水肿,症状于25天前逐渐开始。该患者此前被诊断为类风湿关节炎,接受了每周7.5毫克甲氨蝶呤治疗6个月。体格检查发现:面色苍白、下肢水肿、手部有滑膜炎和畸形。实验室检查显示贫血、肌酸磷酸激酶、血沉水平升高,抗核抗体、抗双链DNA和抗核核糖核蛋白呈阳性。尿蛋白排泄量为1625毫克/24小时。胸部X光显示双侧胸腔积液。超声心动图显示心包积液。胸腹部及盆腔断层扫描显示右侧卵巢有一个直径为5×6厘米的不均匀肿块。该肿块通过手术切除,并进行了活检,结果符合卵巢高级别浆液性腺癌。给予甲强龙1克静脉注射,每3天一次,然后继续口服泼尼松60毫克/天。出院后,她服用泼尼松25毫克/天,甲氨蝶呤10毫克。
我们的病例显示该患者没有患卵巢癌的危险因素。相反,我们的患者年轻、不吸烟,在类风湿关节炎诊断之前没有接受过任何治疗,最后,她育有3个孩子,均足月妊娠且身体健康。该病例突出了对MCTD患者保持高度恶性肿瘤怀疑指数的重要性。然而,对于包括MCTD在内的自身免疫性疾病女性中免疫系统在卵巢癌发生中的作用仍有必要进一步研究。