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从皮肤科到肾病科:揭开显微镜下多血管炎的面纱。

From Dermatology to Nephrology: Unveiling Granulomatosis With Polyangiitis.

作者信息

Hussain Miranda Shazia, Davila Pacheco Rafael, Zaragoza Ramírez Elizabeth, Arenas Sánchez Kelly Andrea, Eduardo Ordaz Velázquez Delfino

机构信息

Internal Medicine, Centro Medico Nacional 20 de Noviembre, Mexico City, MEX.

Internal Medicine, Hospital Regional Lic. Adolfo Lopez Mateos, Mexico City, MEX.

出版信息

Cureus. 2025 Aug 7;17(8):e89554. doi: 10.7759/cureus.89554. eCollection 2025 Aug.

Abstract

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare form of pauci-immune vasculitis that primarily affects the respiratory tract and kidneys, though it can involve virtually any organ system. As a systemic vasculitis, it targets small- and medium-sized blood vessels and is associated with anti-neutrophil cytoplasmic antibodies (ANCA), particularly those directed against proteinase 3 (PR3). Due to its nonspecific symptoms and variable clinical presentation, GPA requires a high index of suspicion for timely diagnosis. Early treatment is essential, as the disease carries a high risk of morbidity and mortality if left untreated. We present a case of a patient with a one-year history of cutaneous lesions who was ultimately diagnosed with GPA, with an unusual concurrent infection by cytomegalovirus (CMV).

摘要

肉芽肿性多血管炎(GPA),以前称为韦格纳肉芽肿,是一种罕见的寡免疫性血管炎,主要影响呼吸道和肾脏,尽管实际上它可累及任何器官系统。作为一种系统性血管炎,它靶向中小血管,并与抗中性粒细胞胞浆抗体(ANCA)相关,尤其是那些针对蛋白酶3(PR3)的抗体。由于其症状不具特异性且临床表现多样,GPA需要高度怀疑才能及时诊断。早期治疗至关重要,因为如果不治疗,该疾病会带来很高的发病和死亡风险。我们报告一例有一年皮肤病变病史的患者,最终被诊断为GPA,并伴有不寻常的巨细胞病毒(CMV)并发感染。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8fa2/12413906/b8db7a63e6e0/cureus-0017-00000089554-i01.jpg

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