Cerebral Vein Thrombosis in the Antiphospholipid Syndrome: Analysis of a Series of 27 Patients and Review of the Literature.

Alba Jerez-Lienas,Ricard Cervera,Miguel Hie,Julien Haroche,Alexis Mathian,Jenifer Aboab,Fleur Cohen-Aubart,Isabelle Crassard,Zahir Amoura,Denis Wahl

doi:10.3390/brainsci11121641

Abstract

(1) Background: The Antiphospholipid Syndrome (APS) is a systemic autoimmune disorder characterized by arterial and/or venous thrombosis, pregnancy morbidity and raised titers of antiphospholipid antibodies. Cerebral vein thrombosis (CVT) is a rare form of cerebrovascular accident and an uncommon APS manifestation; the information in the literature about this feature consists of case reports and small case series. Our purpose is to describe the particular characteristics of CVT when occurs as part of the APS and compare our series with the patients published in the literature. (2) Methods: We conducted a retrospective observational study collecting data from medical records in three referral centers for APS and CVT, and a systematic review of the literature for CVT cases in APS patients. (3) Results: Twenty-seven APS patients with CVT were identified in our medical records, the majority of them diagnosed as primary APS and with the CVT being the first manifestation of the disease; additional risk factors for thrombosis were identified. The review of the literature yielded 86 cases, with similar characteristics as those of our retrospective series. (4) Conclusions: To our knowledge, our study is the largest CVT series in APS patients published to date, providing a unique point of view in this rare thrombotic manifestation.

Highlights

The antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by arterial and/or venous thromboses, pregnancy morbidity and raised titers of antiphospholipid antibodies [1,2]
We conducted a retrospective study in three main referral hospitals, collecting data from patients with Cerebral vein thrombosis (CVT) associated to APS: Service de Médecine Interne 2 of Hôpital PitiéSalpêtrière, Paris, France (French national referral center for systemic lupus erythematosus (SLE) and APS patients); the Department of Neurology of Hôpital Lariboisière, Paris, France (referral center for rare vascular diseases of the brain and the eye (CERVCO)) and the Department of Autoimmune
The differences found between the two series probably are due to the specialized approach in the three referral hospitals and the fact that many cases from the literature were prior to 2006

Summary

Introduction

The antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by arterial and/or venous thromboses, pregnancy morbidity and raised titers of antiphospholipid antibodies (aPL) [1,2]. It is considered to be the most common acquired thrombophilia Thrombotic events in these patients consist in deep vein thrombosis (32%), stroke (13%), superficial thrombophlebitis (9%), pulmonary embolism (9%) and transient ischemic attack (7%), among other clinical manifestations. Cerebral vein thrombosis (CVT) is the thrombotic occlusion of a cerebral sinus or parenchymal vein, which occurs predominantly in young women (median age, 39 years; female/male ratio, 3/1) [4]. It is a rare disease, as it represents only 0.5–1% of the cerebral vascular accidents [5] and has an estimated incidence of 1.32 per 100.000 person-years [6]

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Results

Discussion

Conclusion