CAJ | 학술논문

目的总结嗜酸性筋膜炎(EF)病例并复习相关文献,以提高对该病认识.方法对北京协和医院1990年1月至2011年12月收治的16例住院患者的人口学特点、临床表现、系统受累、实验室检查、病理及治疗等进行分析.结果 ①16例患者中男性13例,女性3例；平均发病年龄(47±8)岁；②3例在发病前有明确的劳累或剧烈运动史；③6例存在抗核抗体阳性(6/12);3例类风湿因子(RF)阳性(3/10)；6例出现高γ球蛋白血症(6/7);8例IgG增高(8/13)；10例出现嗜酸性粒细胞增高(10/16)；④仅1例出现血小板减低(1/16),余无系统受累表现.结论本病可能为遗传因素和环境因素共同参与发病的一种自身免疫病.系统受累较少.激素及免疫抑制剂对本病有效.
목적 총결기산성근막염(EF)병례병복습상관문헌,이제고대해병인식.방법 대북경협화의원1990년1월지2011년12월수치적16례주원환자적인구학특점、림상표현、계통수루、실험실검사、병리급치료등진행분석.결과 ①16례환자중남성13례,녀성3례；평균발병년령(47±8)세；②3례재발병전유명학적로루혹극렬운동사；③6례존재항핵항체양성(6/12);3례류풍습인자(RF)양성(3/10)；6례출현고γ구단백혈증(6/7);8례IgG증고(8/13)；10례출현기산성립세포증고(10/16)；④부1례출현혈소판감저(1/16),여무계통수루표현.결론 본병가능위유전인소화배경인소공동삼여발병적일충자신면역병.계통수루교소.격소급면역억제제대본병유효.
Objective We reported 16 eosinophilic fasciitis (EF) patients with eosinophilic fasciitis and performed a systematic review of the literature to improve the disease awareness.Methods The clinical course of 16 patients with eosinophilic fasciitis at the Peking Union Medical College Hospital were described,inclu-ding demographic data,clinical manifest-ations,laboratory tests,pathology and treatment.Results The mean age at diagnosis was (47±8) years,with 13 female and 3 male patients.Three cases had exertion or strenuous sports before the onset of EF.Positive ANA was noted in 6 of 12,positive RF was noted in 3 of 10,hyper-gammaglobulinemia was noted in 6 of 7,elevated IgG was noted in 8 of 13,peripheral blood eosinophilia was noted in 10 of 16,while thrombocytopenia was found in one patient.Conclusion Based on this and other reported cases in the literature,EF may be a kind of autoimmune disease.Genetic influence and environ-mental factors are involved in the development of this disease.Systemic involvement is rare.In general,corticosteroids and immunosuppressive are effective in EF.