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一项关于活检证实的继发性淀粉样变性相关类风湿关节炎患者的研究

[A study of rheumatoid arthritis patients associated with biopsy-proven secondary amyloidosis].

作者信息

Nakano M, Yamada T, Ozawa T, Kikuchi M, Satoh T, Nakazono K, Hanyu T, Murasawa A, Arakawa M

出版信息

Ryumachi. 1989 Aug;29(4):251-8.

PMID:2617367
Abstract

Reactive systemic amyloidosis associated with rheumatoid arthritis (RA) was studied clinically in 28 patients (2 men and 26 women). The diagnosis of amyloidosis was established by histological examination of biopsy materials. Upper gastrointestinal tract biopsy was performed in 14 patients, and renal and rectal biopsy in 8 and 4 respectively. The mean age and duration of RA at diagnosis of amyloidosis were 58.6 (range 35-72) years and 15.5 (range 4-44) years respectively. Almost all patients had intractable and progressive courses of RA. Serological activities determined by C-reactive protein (CRP) and erythrocyte sedimentation rates were moderate to high in over 80% of the cases. Renal abnormalities were noticed in 19 cases, and gastrointestinal disorders in 10. Eight patients died from 1 to 54 (mean 15.3) months after the diagnosis of amyloidosis; 5 died of renal failure and 2 of gastrointestinal involvements. Renal impairments progressed frequently and serum creatinine elevated over 1.5 mg/dl in another 8 cases. Five patients progressing to renal failure were treated with hemodialysis. Three died within several weeks after the induction of hemodialysis, although 2 were treated for more than 2 years. Intractable hypotension and pulmonary congestion were frequently observed in these cases. A close relationship was found between serum amyloid A protein (SAA) and CRP concentration, so that the measurement of SAA seemed to be valuable in assessing disease activity. Concerning the treatment of amyloidosis, cyclophosphamide and corticosteroids seemed to be effective in several cases, although it had been unsatisfactory in most cases.

摘要

对28例类风湿关节炎(RA)相关的反应性系统性淀粉样变性患者(2例男性,26例女性)进行了临床研究。淀粉样变性的诊断通过活检材料的组织学检查确定。14例患者进行了上消化道活检,8例和4例分别进行了肾活检和直肠活检。淀粉样变性诊断时RA的平均年龄和病程分别为58.6岁(范围35 - 72岁)和15.5年(范围4 - 44年)。几乎所有患者的RA病程顽固且呈进行性。超过80%的病例中,由C反应蛋白(CRP)和红细胞沉降率测定的血清学活性为中度至高度。19例出现肾脏异常,10例出现胃肠道疾病。8例患者在淀粉样变性诊断后1至54个月(平均15.3个月)死亡;5例死于肾衰竭,2例死于胃肠道受累。另外8例患者肾功能损害频繁进展,血清肌酐升高超过1.5mg/dl。5例进展为肾衰竭的患者接受了血液透析治疗。3例在开始血液透析后数周内死亡,尽管有2例接受了超过2年的治疗。这些病例中经常观察到顽固性低血压和肺充血。发现血清淀粉样蛋白A(SAA)与CRP浓度之间存在密切关系,因此SAA的测定在评估疾病活动方面似乎具有价值。关于淀粉样变性的治疗,环磷酰胺和皮质类固醇在一些病例中似乎有效,尽管在大多数病例中并不理想。

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