Wielosz Ewa, Majdan Maria, Zychowska Iwona, Jeleniewicz Radosław
Department of Rheumatology and Connective Tissue Diseases, Medical University of Lublin, Jaczewskiego 8 Street, 20-090, Lublin, Poland.
Rheumatol Int. 2008 Jul;28(9):919-23. doi: 10.1007/s00296-008-0544-5. Epub 2008 Mar 5.
We report the case of coexistence of five autoimmune diseases in a 36-year-old woman, who initially developed psoriasis. Several years later, the patient was diagnosed with a mixed connective tissue disease and primary biliary cirrhosis (PBC). On admission to the Department of Rheumatology and Connective Tissue Diseases, the patient fulfilled classification criteria of an overlap syndrome systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome/systemic sclerosis (SSc)/Sjogren's syndrome (SS) with coexisting PBC and psoriasis. The SLE symptoms included discoid lupus erythematosus, arthritis, pancytopenia, antinuclear antibodies and anticardiolipin antibodies. Moreover, the patient met the criteria of antiphospholipid syndrome diagnosed based on preterm delivery before week 34, and high values of anticardiolipin antibodies were found at repeated determinations. The SSc symptoms included sclerodactyly, pulmonary fibrosis with pulmonary hypertension and esophageal dysfunction. The SS syndrome involved xerostomia, xerophthalmia, the positive Schirmer's test and presence of anti-SS antibodies. The literature reports overlap syndromes in various combinations; however, the coexistence of five autoimmune diseases is extremely rare.
我们报告了一名36岁女性同时患有五种自身免疫性疾病的病例,该患者最初患银屑病。几年后,患者被诊断为混合性结缔组织病和原发性胆汁性肝硬化(PBC)。在风湿和结缔组织病科住院时,该患者符合重叠综合征的分类标准,即系统性红斑狼疮(SLE)合并继发性抗磷脂综合征/系统性硬化症(SSc)/干燥综合征(SS),同时伴有PBC和银屑病。SLE症状包括盘状红斑狼疮、关节炎、全血细胞减少、抗核抗体和抗心磷脂抗体。此外,该患者符合基于34周前早产诊断的抗磷脂综合征标准,且重复检测发现抗心磷脂抗体值较高。SSc症状包括指(趾)硬皮病、伴有肺动脉高压的肺纤维化和食管功能障碍。SS综合征包括口干、眼干、Schirmer试验阳性和抗SS抗体阳性。文献报道了各种组合的重叠综合征;然而,五种自身免疫性疾病同时存在极为罕见。