EULAR 2016 | Daily Highlights
NEUROLOGICAL INVOLVEMENT IN PATIENTS WITH ANTIPHOSPHOLIPID SYNDROME: A CASE SERIES WITH A LONG-TERM FOLLOW-UPAbstract: SAT0316
Authors: M. Estévez 1,*, A. Argibay1, A. Pérez1, C. Vázquez-Triñanes1, B. Machado1, R. Lorenzo1, A. Sousa1, I. Villaverde1, M. Freire1, A. Rivera1
Co Authors: 1Thrombosis and Vasculitis Unit, University Hospital of Vigo, Vigo, Spain
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by autoantibody production and thrombosis or pregnancy morbidity. Central nervous system abnormalities are a common feature of APS that have been attributed to both vascular thrombosis as well as direct injury to neuronal tissue by antiphospholipid antibodies. Neurological manifestations in APS are diverse and may be confused with other neurologic syndromes.
The aim of our study was to determine the prevalence and the type of neurological involvement in patients with APS.
A case series of patients with APS diagnosed and followed for a long period in a specific unit of Thrombosis and Vasculitis was conducted. Clinical and laboratory data were collected in a retrospective manner. Patients with neurological manifestations were compared with patients without nervous system involvement. Chi2 and t-student were used, using the statistical package SPSS22.0.
88 patients with APS were included, 63 (71,6%) female, mean age 56,5 ± 20 years [range 11-89], with a mean follow-up of 9,1 years (median 7 years). 23 patients (26,1%) had a secondary APS: 20 SLE, 2 systemic sclerosis and 1 Sjögren's syndrome. 4 (4,5%) were pure obstetric APS, 36 (40,9%) had an arterial thrombosis, 30 (34,1%) had a venous thrombosis and 18 (20,4%) had mixed events (arterial, venous and obstetric). 60 (68,2%) patients had a cardiovascular risk factor (hypertension, dyslipidemia, type 2 DM) and 15 (17%) had atrial fibrillation. 49 patients of 88 with APS (55,7%) had neurological involvement. 26 (53,1%) patients had ischemic stroke, 8 (16,3%) transient ischemic attack, 16 (32,6%) cognitive deficits, 14 (28,6%) epilepsy, 8 (16,3%) headache, 5 (10,2%) psychosis, 7 (14,3%) movement disorders and 3 (6,1%) had sensorineural hearing loss. 28 (31,8%) patients had ?2 neurologic symptoms. 19 patients (38,8%) had white matter lesions on MRI but only 1 patient was diagnosed with multiple sclerosis. In 17 patients (34,7%) neurological involvement was diagnosed before APS (mean 7,2 ± 7,9 years). Only 3 patients had livedo reticularis: 2 SLE y 1 Sneddon syndrome. No significant differences in age, sex, classification, antiphospholipid antibodies, comorbidities and therapy were observed between patients with or without neurological manifestations. Hospitalization (93,9% vs 74,4%, p 0,01) and mortality (36,7% vs 7,7%, p 0,001) was higher in neurological APS.
More than half of patients with APS had neurological involvement. Cerebrovascular ischemic events and cognitive deficits were the most common manifestations. Neurological involvement was associated with higher mortality and hospitalization. Neurological symptoms may occur long before diagnosis of APS. We have not identified risk markers of central nervous system involvement.
1)Arnson Y et al. The antiphospholipid syndrome as a neurological disease. Semin Arthritis Rheum. 2010;40(2):97. 2)Muscal E, Brey RL. Neurologic manifestations of the antiphospholipid syndrome: integrating molecular and clinical lessons. Curr Rheumatol Rep. 2008;10(1):67.
Disclosure of Interest
The Spanish colleagues analyzed the characteristics of patients with APS with the hope to identify risk factors for neurologic involvement. Of 88 patients (63 female) with a mean age of 56.5y and a mean follow-up of 9.1 years 26 had a secondary APS. Arterial and venous events were found in 41% and 34% of the cases, the remaining being mixed thrombotic events. Of note, 2/3 of the patients had a cardiovascular risk factor such as hypertension.
49/88 patients showed neurologic involvement: ischemic stroke, TIA, cognitive deficits, epilepsy, headache, psychosis, movement disorders and sensorineural hearing loss (listed in decreasing prevalence). Remarkably, white matter lesions in MRI were found in 39% of the cases only, and multiple sclerosis was diagnosed in 1 case. Neurologic involvement was associated with higher mortality and hospitalization rate.
In summary, the data show no differences in disease characteristics such as antiphospholipid antibodies between patients with and patients without neurological involvement. The well-known livedo reticularis and Sneddon syndrome are rarely present. As a clinician one has to search for the presence of a broad range of clinical neurological signs, keeping in mind that MRI is no screening tool for the diagnosis of neurological APS.
Prof. Dr. Peter Villiger
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