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一例罕见的显微镜下多血管炎合并短暂性垂体炎病例。

A Rare Case of Microscopic Polyangitis With Associated Transient Hypophysitis.

作者信息

Martinez-Gil Mercedes, Rodriguez Monica, Hughes Jeremy, Kalya Savitha B, Yuen Kevin C J

机构信息

Department of Internal Medicine, Creighton University School of Medicine, Phoenix, AZ 85013, USA.

Barrow Pituitary Center, Barrow Neurological Institute, University of Arizona College of Medicine and Creighton University School of Medicine, Phoenix, AZ 85013, USA.

出版信息

JCEM Case Rep. 2025 Feb 13;3(3):luaf010. doi: 10.1210/jcemcr/luaf010. eCollection 2025 Mar.

Abstract

Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are rare forms of antineutrophil cytoplasmic antibody-associated vasculitides, characterized by systemic inflammation and necrosis of small blood vessels, which can lead to multiorgan damage. GPA is identified by the presence of granulomas and serine proteinase-3 positivity, while MPA is marked by necrotizing vasculitis without granulomas and is associated with myeloperoxidase (MPO) positivity. Central nervous system involvement is more frequent in GPA, affecting 7% to 10% of patients, compared to its occurrence in approximately 1% of MPA cases. In this case report, we present a 41-year-old woman who exhibited symptoms of pituitary mass effect, initially suspected to be a macroadenoma. Further investigation revealed pituitary enlargement due to MPA, confirmed by positive MPO antibodies and lung biopsy findings consistent with MPA. After treatment with prednisone and rituximab, the patient's pituitary gland returned to its normal size, with significant improvement in her symptoms. This case highlights the diagnostic complexities in differentiating MPA from more common causes of pituitary enlargement and underscores the necessity of considering vasculitic origins in similar clinical scenarios. Further research is essential to deepen the understanding of the pathophysiology and to optimize the management of pituitary involvement in MPA.

摘要

肉芽肿性多血管炎(GPA)和显微镜下多血管炎(MPA)是抗中性粒细胞胞浆抗体相关血管炎的罕见形式,其特征为全身炎症和小血管坏死,可导致多器官损害。GPA通过肉芽肿的存在和丝氨酸蛋白酶-3阳性来识别,而MPA的特征是无肉芽肿的坏死性血管炎,并与髓过氧化物酶(MPO)阳性相关。GPA患者中枢神经系统受累更为常见,发生率为7%至10%,而MPA患者的发生率约为1%。在本病例报告中,我们介绍了一名41岁女性,她出现了垂体占位效应的症状,最初怀疑是大腺瘤。进一步检查发现是MPA导致垂体肿大,MPO抗体阳性以及肺活检结果与MPA一致证实了这一点。在用泼尼松和利妥昔单抗治疗后,患者的垂体恢复正常大小,症状明显改善。该病例突出了将MPA与垂体肿大更常见原因相鉴别时的诊断复杂性,并强调在类似临床情况下考虑血管炎病因的必要性。进一步研究对于加深对病理生理学的理解以及优化MPA垂体受累的管理至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b41b/11822851/fe5b416c900d/luaf010f1.jpg

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