Primary antiphospholipid syndrome complicated with moyamoya syndrome: a case report and literature review

Abstract

Objective: To analyze the clinical data of a case of primary antiphospholipid syndrome (PAPS) combined with moyamoya syndrome (MMS), and to explore the clinical characteristics of APS combined with MMS. Methods:The clinical data of a patient with PAPS and MMS were collected and analyzed by searching the relevant literatures. Results: The patient was a 23 year old female with 2 pathological pregnancies. The disease started with cerebral infarction. Cerebrovascular imaging showed severe stenosis or occlusion of bilateral internal carotid arteries with collateral vessels formation, which was consistent with MMS findings. Abdominal CT showed splenomegaly. The symptoms were improved after anticoagulation and immunosuppressive therapy. Through literature review at home and abroad, 7 patients with APS complicated with smoky vascular changes with detailed clinical data were found, all of them were female, with an average age of 26.6 years. Their clinical manifestations were heterogeneous, in which limb weakness was a common symptom, with arteriovenous thrombosis, pathological pregnancy and persistent positive antiphospholipid antibody as the main clinical manifestations. A total of 6 patients were followed up in detail, and the symptoms of the 5 patients were improved after anticoagulation and immunosuppressive therapy. One patient died of cerebral hemorrhage. Conclusions: APS is an autoimmune disease involving multiple systems. The case report of APS with MMS is very rare. The anticoagulant or antiplatelet therapy of APS is contradictory to the risk of bleeding in the collateral vessels of MMS, so dual antiplatelet therapy should be carefully selected.

Keywords

• |antiphospholipid syndrome|moyamoya syndrome|pathological pregnancy|ischemic stroke|splenomegaly