CAJ | 학술논문

目的探讨IgG4相关性腹膜后纤维化(RPF)的临床特征、治疗及预后.方法收集2010年12月至2013年9月期间北京协和医院诊断的IgG4相关疾病(IgG4-RD)病例,总结其中RPF病例的临床表现、实验室检查特点和治疗反应.结果共纳入IgG4-RD 107例,其中RPF 22例(21％).年龄(63 ±11)岁,男∶女4.5∶1.最常见表现为腰痛(55％).20例(91％)合并其他脏器损害,包括淋巴结炎(41％)、主动脉炎(36％)、涎腺炎(32％)、泪腺炎(23％)及自身免疫性胰腺炎(18％).10例(46％)患者有过敏史.血清IgG4亚类均显著升高.外周血嗜酸性粒细胞计数升高5例(23％),检测血清总IgE的13例中12例升高.行活体组织检查12例,包括腹膜后组织2例,显示大量淋巴细胞浸润伴淋巴滤泡形成,纤维组织增生,IgG4染色阳性.均使用糖皮质激素治疗,部分联合免疫抑制剂和他莫昔芬治疗,疗效明显.结论部分RPF与IgG4-RD密切相关,对糖皮质激素和免疫抑制剂的治疗反应良好.
목적 탐토IgG4상관성복막후섬유화(RPF)적림상특정、치료급예후.방법 수집2010년12월지2013년9월기간북경협화의원진단적IgG4상관질병(IgG4-RD)병례,총결기중RPF병례적림상표현、실험실검사특점화치료반응.결과 공납입IgG4-RD 107례,기중RPF 22례(21％).년령(63 ±11)세,남∶녀4.5∶1.최상견표현위요통(55％).20례(91％)합병기타장기손해,포괄림파결염(41％)、주동맥염(36％)、연선염(32％)、루선염(23％)급자신면역성이선염(18％).10례(46％)환자유과민사.혈청IgG4아류균현저승고.외주혈기산성립세포계수승고5례(23％),검측혈청총IgE적13례중12례승고.행활체조직검사12례,포괄복막후조직2례,현시대량림파세포침윤반림파려포형성,섬유조직증생,IgG4염색양성.균사용당피질격소치료,부분연합면역억제제화타막석분치료,료효명현.결론 부분RPF여IgG4-RD밀절상관,대당피질격소화면역억제제적치료반응량호.
Objective To explore the clinical characteristics of IgG4-related retroperitoneal fibrosis (RPF) in China.Methods From December 2010 to September 2013,a total of 107 IgG4-related disease (IgG4-RD) patients were reviewed prospectively.Their clinical,laboratory and histopathological features were summarized.And the treatment responses were analyzed.Results Among them,22 RPF cases were identified.The male-to-female ratio and age were 4.5∶1 and 63-± 11 years respectively.The follow-up period was 3-28 months.The most common onset symptom was low back pain (55％).Multiple organ involvements were documented in 20 patients (91％),including lymphadenopathy (n =9),aortitis (n =8),sialadenitis (n =7),dacryoadenitis (n =5) and autoimmune pancreatitis (n =4).Allergic histories were reported by10 subjects (46％).Eosinophilia was present in 6 subjects (27％).Serum IgG4 levels were significantly elevated in all subjects.Serum total IgE level was elevated in 12/13 tested subjects.Tissue biopsies of 12 subjects revealed massive lymphocytic infiltration and obvious tissue fibrosis.Immunohistochemical staining showed a massive infiltration of tgG4-positive plasma cell.Glucocorticoid was administrated in all subjects.Most subjects received combined therapy with immunosuppressive agents or tamoxifen.Conclusion IgG4-related RPF is a systemic autoimmune disease with favorable responses to the treatments of glucocorticoid and immunosuppressive agents.