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Systemic rheumatoid vasculitis in the era of modern immunosuppressive therapy

机译：现代免疫抑制疗法时代的系统性类风湿血管炎

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Objectives. Systemic rheumatoid vasculitis (SRV) is a rare but potentially serious systemic disease manifestation of rheumatoid arthritis (RA) characterized by the development of necrotizing vasculitis. The incidence of SRV appears to be decreasing possibly reflecting progress in RA treatment. The aims of this study were to review the clinical manifestations of SRV in a stable well-defined population during 2001-10 and to compare with our previous cohort (1988-2000) and also a cohort from 1975 to 1981. Methods. Using Norfolk Vasculitis Register, a prospective register of patients with systemic vasculitis since 1988, all patients with a diagnosis of SRV from 1 January 2001 until 31 December 2010 were identified. SRV was defined according to the Scott and Bacon criteria (1984). Clinical features were obtained by retrospective case note review. Results. Eighteen patients with SRV were identified (10 male), median age at diagnosis was 72 years and average disease duration 15.6 years. The average annual incidence for 2001-10 was 3.9 per million. Oneyear mortality was 12% and 5-year mortality 60%. The clinical manifestations were similar apart from systemic and cutaneous features which were more common in the earlier cohorts. Conclusion. The incidence of SRV has declined significantly in the last 40 years; but the clinical manifestations remain similar. Systemic symptoms, and cutaneous manifestations such as infarcts and nodules, are slightly less common in the recent cohort. Despite modern immunosuppressive therapy the prognosis remains poor.
机译：目标。系统性类风湿性血管炎（SRV）是类风湿性关节炎（RA）罕见但潜在的严重系统性疾病表现，其特征是坏死性血管炎的发展。 SRV的发生率似乎正在下降，这可能反映出RA治疗的进展。这项研究的目的是回顾SRV在2001-10年间稳定的明确人群中的临床表现，并与我们先前的队列（1988-2000年）以及1975年至1981年的队列进行比较。使用自1988年以来对系统性血管炎患者进行前瞻性登记的诺福克血管炎登记册，确定了从2001年1月1日至2010年12月31日诊断为SRV的所有患者。 SRV是根据Scott和Bacon标准（1984）定义的。通过回顾性病例笔记审查获得临床特征。结果。确定了18例SRV患者（10例男性），诊断时的中位年龄为72岁，平均病程为15.6年。 2001-10年的平均年发病率为每百万人3.9。一年死亡率为12％，五年死亡率为60％。除了全身和皮肤特征外，临床表现相似，这在较早的队列中更为常见。结论。在过去40年中，SRV的发生率已大大下降;但临床表现仍相似。在最近的队列研究中，全身症状和皮肤表现，如梗塞和结节，较不常见。尽管有现代免疫抑制疗法，但预后仍然很差。

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