Makeen Ahmad, Alorainan Nourah A, Baloush Samar A, Khadwardi Raghdah, Awais Muhammad
Department of Medicine, Nephrology Section, King Abdulaziz Medical City, Ministry of National Guard, Jeddah, SAU.
Department of Medicine, King Abdullah International Medical Research Center, Jeddah, SAU.
Cureus. 2025 May 9;17(5):e83781. doi: 10.7759/cureus.83781. eCollection 2025 May.
Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare autoimmune disorder that affects small blood vessels, leading to systemic symptoms and kidney damage due to the production of autoantibodies. A limited number of case reports have explored the potential link between AAV and antiphospholipid syndrome (APS). Catastrophic antiphospholipid syndrome (CAPS), a severe variant of APS, is associated with high mortality and poor prognosis. We present a case of AAV that initially achieved complete remission, only to later develop CAPS. The presence of antiphospholipid antibodies at baseline may increase the risk of CAPS and thrombotic events in AAV patients. A high level of suspicion for this association, along with early detection of these antibodies, could facilitate prompt diagnosis and enable early, appropriate treatment, potentially improving the patient's outcome.
抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）是一种罕见的自身免疫性疾病，会影响小血管，由于自身抗体的产生导致全身症状和肾脏损害。少数病例报告探讨了AAV与抗磷脂综合征（APS）之间的潜在联系。灾难性抗磷脂综合征（CAPS）是APS的一种严重变体，与高死亡率和不良预后相关。我们报告一例最初实现完全缓解但随后发展为CAPS的AAV病例。基线时抗磷脂抗体的存在可能会增加AAV患者发生CAPS和血栓事件的风险。对此关联保持高度怀疑，以及早期检测这些抗体，有助于及时诊断并实现早期、恰当的治疗，可能改善患者的预后。
