Badia Rohit R, Hendricks Allen R, Perez Carlos L, Sertich Anthony, Ripley Lindsay
Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, USA.
Department of Pathology, University of Texas Southwestern Medical Center, Dallas, USA.
Cureus. 2021 Mar 23;13(3):e14069. doi: 10.7759/cureus.14069.
Microscopic polyangiitis (MPA) is an autoimmune small-vessel vasculitis often positive for perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA), or anti-myeloperoxidase (MPO), that classically affects the lungs, kidneys, and skin. Several atypical presentations of MPA involving other organs have also been reported in the literature. We report a unique case of a patient who presented with rare presentations of MPA: hearing and vision loss, dysphagia, renal dysfunction. Despite the atypical nature of her symptoms, her p-ANCA serology was positive and kidney biopsy was consistent with MPA. Regardless of the bizarre nature of a patient's symptoms, we highlight the importance of considering MPA as a differential diagnosis in the setting of positive p-ANCA serology.
显微镜下多血管炎(MPA)是一种自身免疫性小血管炎,通常核周抗中性粒细胞胞浆抗体(p-ANCA)或抗髓过氧化物酶(MPO)呈阳性,典型地累及肺、肾和皮肤。文献中也报道了MPA累及其他器官的几种非典型表现。我们报告了一例独特的患者,该患者出现了MPA的罕见表现:听力和视力丧失、吞咽困难、肾功能不全。尽管其症状具有非典型性,但其p-ANCA血清学检查呈阳性,肾脏活检结果与MPA相符。无论患者症状多么奇特,我们强调在p-ANCA血清学检查呈阳性的情况下,将MPA作为鉴别诊断的重要性。