Maruoka T, Tokuda M, Inoh M, Kurata N, Takahara J
First Department of Internal Medicine, Kagawa Medical School.
Nihon Rinsho Meneki Gakkai Kaishi. 1995 Apr;18(2):228-34. doi: 10.2177/jsci.18.228.
Felty's syndrome is diagnosed when a patient shows both splenomegaly and leukocytopenia of various degree during the course of rheumatoid arthritis (RA). The accompanying immunologic abnormalities (e.g., antinuclear antibody, antiplatelet antibody, and hypocomplementemia) also characterize Felty's syndrome, but some authors may regard these abnormalities as a transitional form into overlap syndrome [RA + systemic lupus erythematosus (SLE)]. Here we reported a female case of Felty's syndrome who showed marked thrombocytopenia and severe hypocomplementemia. Thrombocytopenia had been refractory against several forms of therapies including high-dose methylprednisolone. Simultaneously, she had various autoantibodies (i.e., antiplatelet antibody, positive Coombs' test, antithyroglobulin antibody, antimicrosome antibody and anti-RNP antibody). Although she did not fulfill the ARA diagnostic criteria for SLE, the degree of thrombocytopenia as well as that of hypocomplementemia argued in favor of the overlap of SLE in this patient. Low-dose cyclosporin A (CsA) combined with small dose of prednisolone could increase both platelet count and level of complement. Notably, the titers of several autoantibodies dropped after CsA was started. These findings might suggest that CsA could normalize the underlying immunologic abnormalities in this patient. However, the disease activity of RA could not be decreased without a help of low-dose methotrexate.
费尔蒂综合征的诊断标准是患者在类风湿关节炎(RA)病程中出现脾肿大和不同程度的白细胞减少。伴随的免疫异常(如抗核抗体、抗血小板抗体和补体低下)也是费尔蒂综合征的特征,但一些作者可能将这些异常视为向重叠综合征[RA + 系统性红斑狼疮(SLE)]转变的形式。我们在此报告一例费尔蒂综合征女性患者,该患者表现出明显的血小板减少和严重的补体低下。血小板减少对包括大剂量甲泼尼龙在内的多种治疗方法均无效。同时,她存在多种自身抗体(即抗血小板抗体、库姆斯试验阳性、抗甲状腺球蛋白抗体、抗微粒体抗体和抗RNP抗体)。尽管她不符合美国风湿病学会(ARA)的SLE诊断标准,但血小板减少和补体低下的程度表明该患者存在SLE重叠。小剂量环孢素A(CsA)联合小剂量泼尼松龙可使血小板计数和补体水平升高。值得注意的是,开始使用CsA后,几种自身抗体的滴度下降。这些发现可能提示CsA可使该患者潜在的免疫异常恢复正常。然而,在未使用小剂量甲氨蝶呤的情况下,RA的疾病活动度无法降低。