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血友病性关节炎

Haemophilic arthritis.

作者信息

Steven M M, Yogarajah S, Madhok R, Forbes C D, Sturrock R D

出版信息

Q J Med. 1986 Feb;58(226):181-97.

PMID:3714969
Abstract

A detailed clinical and radiological examination of the joints and laboratory studies were carried out on 139 haemophiliacs attending a single centre. The group included more patients with mild and moderate haemophilia (factor levels 6 to 60 per cent) than in previous studies. Haemarthrosis, the most common bleeding manifestation, had affected more than two-thirds of patients including many with mild or moderate disease. Restriction and contracture of the knees and elbows were the most common clinical features and, with the ankles, these joints were most frequently affected both clinically and radiologically. Using a combination of clinical and radiological features, 42 per cent of the patients could be classed as having 'definite' and a further 14 per cent 'possible' haemophilic arthritis. Although haemarthroses were equally prevalent in patients with classical haemophilia and Christmas disease, arthritis was more frequently present in the former. Haemarthrosis and joint disease were exceptional in von Willebrand's disease. The prevalence of arthritis generally related to disease severity as measured by factor level but, in contrast to earlier studies, definite arthritis was seen in some patients with factor levels up to 20 per cent of normal although the number of affected joints was less in these patients with milder disease. Laboratory test abnormalities including circulating immune complexes and hypocomplementaemia were noted in some patients but the abnormalities correlated poorly with clinical features. The present results suggest a recent slight reduction in the prevalence or severity of haemophilic arthritis, possibly attributable to recent improvements in factor replacement treatment. Longer-term studies are required to show whether arthritis is indeed lessening or whether the onset is merely being delayed.

摘要

对在单一中心就诊的139名血友病患者进行了关节的详细临床和放射学检查以及实验室研究。与以往研究相比,该组中轻度和中度血友病(因子水平为6%至60%)患者更多。关节积血是最常见的出血表现,影响了超过三分之二的患者,包括许多轻度或中度疾病患者。膝关节和肘关节的活动受限和挛缩是最常见的临床特征,踝关节也包括在内,这些关节在临床和放射学上最常受累。结合临床和放射学特征,42%的患者可归类为患有“明确的”血友病性关节炎,另有14%为“可能的”血友病性关节炎。虽然关节积血在典型血友病和克里斯马斯病患者中同样普遍,但关节炎在前一种患者中更常见。血管性血友病患者关节积血和关节疾病罕见。关节炎的患病率一般与通过因子水平衡量的疾病严重程度相关,但与早期研究不同的是,在一些因子水平高达正常20%的患者中也发现了明确的关节炎,尽管这些病情较轻的患者受累关节数量较少。一些患者出现了包括循环免疫复合物和低补体血症在内的实验室检查异常,但这些异常与临床特征的相关性较差。目前的结果表明,血友病性关节炎的患病率或严重程度最近略有下降,可能归因于因子替代治疗的近期改善。需要进行长期研究以确定关节炎是否确实在减轻,或者只是发病时间被推迟。

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