Blagova O V, Tsaregorodtsev D A, Nedostup A V, Maevskaia I V, Petukhova N V, Troitskaia M P, Shadaniia Ia R
Ter Arkh. 2010;82(6):56-61.
Constrictive polyserositis (pleuritis, pericarditis) is a syndrome within the underlying disease (tuberculosis, periodic disease, rheumatoid arthritis, systemic lupus erythematosus, asbestos, silicosis, uremia, some genetic diseases), a complication due to chest surgery or radiation or drug therapy, is occasionally idiopathic (fibrosing mediastinitis). There are frequently great difficulties in making its nosological diagnosis. The paper describes a patient in whom the onset of disease was exudative pleurisy with the signs of constriction, arthralgias; pleural punctures provided serous exudates with 80% lymphocytes. A year later there was ascitis and shin and foot edemas, which concurrent with hepatomegaly and cholestasis was regarded as cryptogenic liver cirrhosis. The signs of constrictive pericarditis were further revealed. The disease was complicated by the development of pulmonary artery thromboembolism (PATE) (which required the use of warfarin) and hemorrhagic vasculitis. Therapy with metipred in combination with isoniazid yielded a slight effect. The diagnoses of tuberculosis, liver cirrhosis, and autoimmune hepatitis, systemic vasculitis were consecutively rejected; the diagnosis of rheumatoid polyarthritis with systemic manifestations was made, by taking into account persistent arthalgias with the minimum signs of arthritis, noticeably increased C-reactive protein, rheumatoid factor, and cyclic citrullinated peptide antibodies (CCPA); plasmapheresis, therapy with metipred and methotrexate, and subtotal pericardectomy were performed. Constrictive polyserositis concurrent with PATE, hemorrhagic vasculitis (probably, drug-induced one), and hepatic lesion has been first described in a CCPA-positive patient with rheumatoid arthritis in the presence of moderate true arthritis (during steroid therapy).
缩窄性多浆膜炎(胸膜炎、心包炎)是潜在疾病(结核病、周期性疾病、类风湿关节炎、系统性红斑狼疮、石棉沉着病、矽肺、尿毒症、某些遗传性疾病)中的一种综合征,是胸部手术、放疗或药物治疗引起的并发症,偶尔为特发性(纤维性纵隔炎)。其病因诊断常常存在很大困难。本文描述了一名患者,疾病起病为渗出性胸膜炎伴缩窄体征、关节痛;胸腔穿刺抽出的浆液性渗出液中淋巴细胞占80%。一年后出现腹水、小腿和足部水肿,同时伴有肝肿大和胆汁淤积,被认为是隐源性肝硬化。进一步发现了缩窄性心包炎的体征。该疾病并发了肺动脉血栓栓塞(PATE)(需要使用华法林)和出血性血管炎。甲泼尼龙联合异烟肼治疗效果不佳。结核病、肝硬化、自身免疫性肝炎、系统性血管炎的诊断均被相继排除;考虑到持续的关节痛且关节炎体征轻微、C反应蛋白、类风湿因子和环瓜氨酸肽抗体(CCPA)明显升高,做出了伴有全身表现的类风湿多关节炎的诊断;进行了血浆置换、甲泼尼龙和甲氨蝶呤治疗以及心包次全切除术。在一名CCPA阳性的类风湿关节炎患者中,在中度真性关节炎(类固醇治疗期间)的情况下,首次描述了并发PATE、出血性血管炎(可能为药物性)和肝脏病变的缩窄性多浆膜炎。