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结节性多动脉炎

Polyarteritis Nodosa

作者信息

Stanton Monica, Tiwari Vivekanand

机构信息

Campbell Un School of OM/ Harnett Health

Dartmouth Hitchcock Medical Center

Abstract

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis first described in 1866 by Adolph Kussmaul and Rudolph Maier. It typically affects medium-sized arterial vessels but may affect small-sized arterial vessels. Unlike other small-sized arterial vessel vasculitides, polyarteritis nodosa is not typically associated with anti-neutrophil cytoplasmic antibodies (ANCA). It is a systemic disease process; though there is a limited form of the disease called cutaneous polyarteritis nodosa (CPAN). Even with the limited form of the disease, there is significant morbidity secondary to digital ulcerations, ischemia, and painful skin nodules. Though rare, patients with CPAN can progress to systemic polyarteritis nodosa (PAN). PAN chiefly affects the skin but can also involve other organs. The lungs are usually spared.

摘要

结节性多动脉炎(PAN)是一种系统性坏死性血管炎,1866年由阿道夫·库斯莫尔和鲁道夫·迈尔首次描述。它通常影响中等大小的动脉血管,但也可能影响小动脉血管。与其他小动脉血管炎不同,结节性多动脉炎通常与抗中性粒细胞胞浆抗体(ANCA)无关。它是一种全身性疾病过程;不过有一种疾病的局限形式称为皮肤结节性多动脉炎(CPAN)。即使是疾病的局限形式,也会因指端溃疡、缺血和疼痛性皮肤结节而导致严重的发病率。虽然罕见,但CPAN患者可进展为系统性结节性多动脉炎(PAN)。PAN主要影响皮肤,但也可累及其他器官。肺部通常不受影响。

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