Microscopic Polyangiitis: Delineation of a Cutaneous-Limited Variant Associated With Antimyeloperoxidase Autoantibody

Microscopic Polyangiitis: Delineation of a Cutaneous-Limited Variant Associated With Antimyeloperoxidase Autoantibody
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显微镜下多血管炎:与抗髓过氧化物酶自身抗体相关的皮肤有限变异的描绘

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发表时间:
1997
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影响因子:
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通讯作者:
E. Bingham
E. Bingham
中科院分区:
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文献类型:
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作者:
A. Irvine;I. Bruce;M. Walsh;E. Bingham

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背景资料:显微镜下多血管炎是一种全身性小血管炎,虽然主要与坏死性和新月体肾小球肾炎和肺毛细血管炎有关,但通常具有皮肤和肌肉骨骼特征。显微镜下多血管炎与抗神经细胞胞质自身抗体密切相关,最常表现为核周免疫染色模式。这种模式通常表现为抗髓过氧化物酶自身抗体的特异性。我们报告一例显微镜下多血管炎,即使在几年后,仍然主要是皮肤。观察:我们描述了一个病人有22年的历史皮肤紫癜性血管炎。病变发生在作物在4至6周的时间间隔,并与体质不适和血清炎症指数升高。抗髓过氧化物酶滴度与该患者的疾病活动性密切相关。经过密切,长期的审查和广泛的调查,没有证据表明坏死性和新月体性肾小球肾炎,肺毛细血管炎,或其他深器官的参与已被检测到。结论:据我们所知,这是第一份长期评估主要是皮肤显微镜下多血管炎的报告,并表明血清学特征性显微镜下多血管炎可能仍然有限,而不会随后进展为特征性全身受累。这一观察有助于了解和表征显微镜下多血管炎的临床病理谱。皮肤科医生1997;133:474-477
Background: Microscopic polyangiitis is a systemic small vessel vasculitis, which, although primarily associated with necrotizing and crescentic glomerulonephritis and pulmonary capillaritis, often has cutaneous and musculoskeletal features. Microscopic polyangiitis is strongly associated with antineutrophil cytoplasmic autoantibodies, most often demonstrating a perinuclear immunostaining pattern. This pattern usually demonstrates specificity for antimyeloperoxidase autoantibodies. We report a case of microscopic polyangiitis, which, even after several years, has remained predominantly cutaneous. Observations: We describe a patient with a 22-year history of cutaneous purpuric vasculitis. The lesions occur in crops at 4- to 6-week intervals and are associated with constitutional upset and elevated serologic inflammatory indexes. The antimyeloperoxidase titers closely correlate with disease activity in this patient. After close, long-term review and extensive investigations, no evidence of necrotizing and crescentic glomerulonephritis, pulmonary capillaritis, or other deep-organ involvement has been detected. Conclusions: To our knowledge, this is the first report of a long-term evaluation of predominantly cutaneous microscopic polyangiitis and demonstrates that serologically characteristic microscopic polyangiitis may remain limited without subsequent progression to characteristic systemic involvement. This observation contributes to the understanding and characterization of the clinicopathologic spectrum of microscopic polyangiitis. Arch Dermatol. 1997;133:474-477
DOI: 10.1056/nejm198806233182504
发表时间: 1988-06-23
影响因子: 158.5
作者:
FALK, RJ;JENNETTE, JC
通讯作者: JENNETTE, JC