Dacheva Rositsa, Kurteva Ekaterina, Boyadzhieva Vladimira, Stoilov Rumen, Kyurkchiev Dobroslav, Stoilov Nikolay
Department of Rheumatology, Clinic of Rheumatology, University Hospital St. Ivan Rilski, Medical University of Sofia, 1612 Sofia, Bulgaria.
Laboratory of Clinical Immunology, Department of Clinical Immunology, University Hospital St. Ivan Rilski, Medical University of Sofia, 1431 Sofia, Bulgaria.
Biomedicines. 2023 Oct 18;11(10):2826. doi: 10.3390/biomedicines11102826.
Takayasu arteritis (TA) is a chronic large-vessel vasculitis characterized by immune-mediated panarteritis, which predominantly affects the aorta and its main branches and is most prevalent in young women. TA is unusually associated with the presence of antiphospholipid antibodies. We present a case report of a 48-year-old Caucasian woman with acute aortic dissection as an initial feature of TA, where detailed clinical, imaging and laboratory studies were performed. Computed tomography angiography (CTA) of the chest and abdomen revealed aortic dissection DeBakey I. Bentall and De Bono surgery was performed. Additional immunological tests revealed positive antineutrophil cytoplasmic antibodies (ANCAs) with the simultaneous presence of pANCA and cANCA antibodies on indirect immunofluorescence, along with anti-MPO+PR3+antibodies positivity in the absence of a clinically relevant disease. Surprisingly, antiphospholipid antibodies (aPLs) were detected. Then, we performed a thorough review of the current literature. The coexistence of aPL antibodies and dual specificity for MPO and PR3 in a patient diagnosed with Takayasu arteritis is unusual and poses a diagnostic challenge. The presented case report outlines a rare case of aortic dissection as a presenting symptom of TA, along with atypical ANCA positivity and positive APL antibodies.
高安动脉炎（TA）是一种慢性大血管血管炎，其特征为免疫介导的全动脉炎，主要累及主动脉及其主要分支，在年轻女性中最为常见。TA异常地与抗磷脂抗体的存在相关。我们报告一例48岁白种女性病例，其以急性主动脉夹层作为TA的首发特征，并进行了详细的临床、影像学和实验室检查。胸部和腹部计算机断层血管造影（CTA）显示为DeBakey I型主动脉夹层。实施了Bentall和De Bono手术。额外的免疫学检查显示抗中性粒细胞胞浆抗体（ANCA）阳性，间接免疫荧光显示同时存在pANCA和cANCA抗体，且在无临床相关疾病的情况下抗髓过氧化物酶（MPO）+蛋白酶3（PR3）+抗体呈阳性。令人惊讶的是，检测到了抗磷脂抗体（aPL）。然后，我们对当前文献进行了全面回顾。在诊断为高安动脉炎的患者中，aPL抗体与对MPO和PR3的双重特异性共存是不寻常的，并且带来了诊断挑战。本病例报告概述了一例罕见的以主动脉夹层作为TA首发症状的病例，伴有非典型ANCA阳性和aPL抗体阳性。
