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显微镜下多血管炎作为房室传导阻滞病因的病理证据。

Pathologic Evidence of Granulomatosis With Polyangiitis as a Cause of Atrioventricular Block.

作者信息

Watanabe Naoki, Harada Kenji, Kawai Shigeo, Kobayashi Hisaya, Kario Kazuomi

机构信息

Division of Cardiovascular Medicine, Department of Internal Medicine, Jichi Medical University, Shimotsuke, Tochigi, Japan; Department of Cardiology, Tochigi Medical Center Shimotsuga, Shimotsuke, Tochigi, Japan.

Division of Cardiovascular Medicine, Department of Internal Medicine, Jichi Medical University, Shimotsuke, Tochigi, Japan.

出版信息

JACC Case Rep. 2025 Jul 30;30(21):104414. doi: 10.1016/j.jaccas.2025.104414.

Abstract

BACKGROUND

Atrioventricular block (AVB) is a rare manifestation of granulomatosis with polyangiitis (GPA), and the underlying pathologic relationship between GPA and AVB remains poorly understood.

CASE SUMMARY

We present the case of a 71-year-old woman who developed complete AVB and had a history of microscopic polyangiitis. Despite the absence of clinical signs indicative of GPA relapse throughout her lifetime, postmortem examination revealed microgranulomas and multinucleated giant cells within the atrioventricular node.

DISCUSSION

This case provides pathologic evidence that GPA can lead to complete heart block as an initial clinical manifestation.

TAKE-HOME MESSAGES: GPA-associated AVB can improve with immunosuppressive therapy. GPA should be considered in the differential diagnosis of AVB of unknown etiology.

摘要

背景

房室传导阻滞(AVB)是肉芽肿性多血管炎(GPA)的一种罕见表现,GPA与AVB之间潜在的病理关系仍知之甚少。

病例摘要

我们报告一例71岁女性,发生完全性AVB且有显微镜下多血管炎病史。尽管其一生中无提示GPA复发的临床体征,但尸检发现房室结内有微肉芽肿和多核巨细胞。

讨论

该病例提供了病理证据,表明GPA可导致完全性心脏传导阻滞作为初始临床表现。

要点

GPA相关的AVB可通过免疫抑制治疗改善。在病因不明的AVB鉴别诊断中应考虑GPA。

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本文引用的文献

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Cardiac Involvement in Granulomatosis with Polyangiitis.肉芽肿性多血管炎的心脏受累情况
J Rheumatol. 2015 Jul;42(7):1209-12. doi: 10.3899/jrheum.141513. Epub 2015 May 1.
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Involvement of the heart by small and medium vessel vasculitis.中小血管血管炎累及心脏。
Curr Opin Rheumatol. 2009 Jan;21(1):29-34. doi: 10.1097/BOR.0b013e32831cb94d.
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ANCA small-vessel vasculitis.抗中性粒细胞胞浆抗体相关性小血管炎
J Am Soc Nephrol. 1997 Feb;8(2):314-22. doi: 10.1681/ASN.V82314.

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