Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases
Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases
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DOI：
10.1093/rheumatology/ken342
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发表时间：
2008-11-01
期刊：
影响因子：
5.5
通讯作者：
Koike, T.
中科院分区：
文献类型：
--
作者：
Fukaya, S.;Yasuda, S.;Koike, T.
目标.噬血细胞综合征（HPS）是一种相对罕见的并发症，在自身免疫性疾病。本文分析了系统性自身免疫性疾病患者HPS的临床特点。从1997年至2000年，收集了1014例北海道大学医院收治的全身性自身免疫性疾病患者[350例SLE，136例RA，98例多发性肌炎/皮肌炎（PM/DM），88例SSc，91例血管炎综合征，37例原发性SS，26例成人斯蒂尔病（AOSD）和188例其他疾病]。回顾性分析其临床特点及治疗效果。30例（3.0%）符合HPS标准（两个或多个谱系中的进行性血细胞减少和网状内皮系统中的噬血细胞增多）。基础疾病为SLE（18例）、RA（2例）、PM/DM（2例）、SSc（2例）、血管炎（1例）、SS（2例）和AOSD（3例）。19例患者被诊断为自身免疫相关HPS，8例感染相关，1例药物诱导，1例造血干细胞移植后发生HPS。26例HPS患者单用大剂量皮质类固醇治疗，12例（46%）有效。15例激素抵抗性自身免疫相关HPS患者中有10例接受CsA、环磷酰胺或他克莫司治疗，缓解率为80%。总死亡率为20%。多因素分析显示，有感染史和HPS上CRP>50 mg/l与预后不良有关。HPS在全身性自身免疫住院患者中的患病率不容忽视。免疫抑制剂的管理是有效的情况下，与自身免疫相关的HPS，而预后差的感染相关的HPS。
Objectives. Haemophagocytic syndrome (HPS) is known as a relatively rare complication in autoimmune diseases. Here we analysed the clinical features of HPS in patients with systemic autoimmune diseases.Methods. One thousand and fourteen patients with systemic autoimmune diseases admitted to Hokkaido University Hospital from 1997 to were recruited [350 SLE, 136 RA, 98 polymyositis/dermatomyositis (PM/DM), 88 SSc, 91 vasculitis syndrome, 37 primary SS, 26 adult onset Still's disease (AOSD) and 188 other diseases]. Clinical features and treatment outcomes were retrospectively analysed.Results. Thirty cases (3.0%) fulfilled HPS criteria (progressive cytopenia in two or more lineages and haemophagocytosis in reticuloendothelial systems). Underlying diseases were SLE (18), RA (2), PM/DM (2), SSc (2), vasculitis (1), SS (2) and AOSD (3). Nineteen patients were diagnosed as having autoimmune-associated HPS, eight infection-associated, one drug-induced and one developed HPS after haematopoietic stem cell transplantation. For the treatment of HPS, high-dose corticosteroid monotherapy was given in 26 cases, being effective in 12 (46%). Ten out of 15 patients with corticosteroid-resistant autoimmune-associated HPS were treated with CsA, cyclophosphamide or tacrolimus, leading to the remission in 80%. The overall mortality rate was 20%. Multivariate analysis showed that the presence of infections and CRP level >50 mg/l on HPS related with poor prognosis.Conclusions. The prevalence of HPS among in-hospital patients with systemic autoimmunity is not ignorable. Administration of immunosuppressants was effective in cases with autoimmune-associated HPS, whereas prognosis was poor in infection-associated HPS.