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类风湿关节炎相关血管炎

Vasculitis associated with rheumatoid arthritis.

作者信息

Vollertsen R S, Conn D L

机构信息

Division of Rheumatology and Internal Medicine, Mayo Clinic, Rochester, Minnesota.

出版信息

Rheum Dis Clin North Am. 1990 May;16(2):445-61.

PMID:2189161
Abstract

Vasculitis may accompany rheumatoid arthritis. One must distinguish between vascular involvement associated with the pathogenesis of rheumatoid arthritis, isolated digital vasculitis, and the syndrome of clinical rheumatoid vasculitis. The cause of clinical rheumatoid vasculitis is unknown. High titers of rheumatoid factor, cryoglobulins, diminished circulating complement, an increased prevalence of HLA-DR4, and the pathologic findings suggest an immune etiology. However, similar, but perhaps less pronounced, abnormalities occur in uncomplicated rheumatoid arthritis, and these findings are not universal in complicating vasculitis. Classic cutaneous clinical manifestations include ischemic ulcers, digital gangrene, and palpable purpura. Mononeuritis multiplex is another classic presentation of rheumatoid vasculitis. Small digital infarctions may accompany other manifestations in clinical vasculitis or may occur alone as isolated digital arteritis, in which case the prognosis is relatively favorable. Weight loss, pleuritis, pericarditis, ocular inflammation, splenomegaly, hepatomegaly, and Felty's syndrome have also been reported in association with rheumatoid vasculitis. Although renal involvement has been considered unusual in rheumatoid vasculitis, several studies suggest that this may be more common than previously recognized. Ideally, a biopsy or an angiogram confirms the diagnosis of rheumatoid vasculitis, but often the diagnosis rests upon the clinical picture. In general, blind biopsies are not helpful, although one series indicated that a blind rectal biopsy may be an exception to this rule. An elevated erythrocyte sedimentation rate, increased C-reactive protein level, anemia, thrombocytosis, hypoalbuminemia, and a positive rheumatoid factor are common laboratory findings. Leukocytosis, hypergammaglobinemia, leukocytopenia, an elevated creatinine level, and minimal abnormalities of the urinary sediment also occur in patients with rheumatoid vasculitis. However, these abnormalities overlap in patients with uncomplicated rheumatoid arthritis, and their role in distinguishing rheumatoid vasculitis from uncomplicated rheumatoid arthritis is limited. Other immunologic tests have no established clinical role in diagnosing rheumatoid vasculitis. Therapy depends upon the clinical manifestation of rheumatoid vasculitis. Uncomplicated rheumatoid arthritis deserves appropriate therapy, and general attention to nutrition, cessation of tobacco, and control of blood pressure are indicated for all patients. Isolated digital vasculitis generally requires no more than the usual treatment for uncomplicated rheumatoid arthritis. Appropriate dermatologic management is indicated for ischemic ulcers. Most clinical experience in managing more symptomatic rheumatoid vasculitis has focused on glucocorticosteroids, D-penicillamine, and cytotoxic immunosuppressive drugs.(ABSTRACT TRUNCATED AT 400 WORDS)

摘要

血管炎可能伴发类风湿关节炎。必须区分与类风湿关节炎发病机制相关的血管受累、孤立性指端血管炎以及临床类风湿血管炎综合征。临床类风湿血管炎的病因不明。高滴度类风湿因子、冷球蛋白、循环补体降低、HLA - DR4患病率增加以及病理表现提示免疫病因。然而,类似但可能程度较轻的异常也出现在无并发症的类风湿关节炎中,且这些表现并非在并发血管炎时都普遍存在。典型的皮肤临床表现包括缺血性溃疡、指端坏疽和可触及的紫癜。多发性单神经炎是类风湿血管炎的另一种典型表现。小的指端梗死可能伴随临床血管炎的其他表现,也可能单独作为孤立性指端动脉炎出现,在这种情况下预后相对较好。体重减轻、胸膜炎、心包炎、眼部炎症、脾肿大、肝肿大以及费尔蒂综合征也有报道与类风湿血管炎相关。尽管肾受累在类风湿血管炎中曾被认为不常见,但多项研究表明其可能比之前认识到的更为常见。理想情况下,活检或血管造影可确诊类风湿血管炎,但通常诊断基于临床表现。一般来说,盲目活检并无帮助,不过有系列研究表明盲目直肠活检可能是个例外。红细胞沉降率升高、C反应蛋白水平升高、贫血、血小板增多、低白蛋白血症以及类风湿因子阳性是常见的实验室检查结果。白细胞增多、高球蛋白血症、白细胞减少、肌酐水平升高以及尿沉渣轻微异常也出现在类风湿血管炎患者中。然而,这些异常在无并发症的类风湿关节炎患者中也会出现,它们在区分类风湿血管炎与无并发症的类风湿关节炎方面的作用有限。其他免疫学检查在诊断类风湿血管炎方面尚无明确的临床作用。治疗取决于类风湿血管炎的临床表现。无并发症的类风湿关节炎应接受适当治疗,所有患者都需关注营养、戒烟和控制血压。孤立性指端血管炎通常只需采用无并发症类风湿关节炎的常规治疗。对于缺血性溃疡需进行适当的皮肤科处理。在治疗症状更明显的类风湿血管炎方面,大多数临床经验集中于糖皮质激素、青霉胺和细胞毒性免疫抑制药物。

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