Long-term outcome of 32 patients with chorea and systemic lupus erythematosus or antiphospholipid antibodies

• Published in: Movement disorders 2011-11, Vol.26 (13), p.2422-2427
• Main Authors: Reiner, Peggy, Galanaud, Damien, Leroux, Gaëlle, Vidailhet, Marie, Haroche, Julien, Huong, Du Le Thi, Francès, Camille, Papo, Thomas, de Gennes, Christian, Musset, Lucile, Wechsler, Bertrand, Amoura, Zahir, Piette, Jean-Charles, Costedoat-Chalumeau, Nathalie
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Antibodies, Antiphospholipid - biosynthesis; antiphospholipid; Antiphospholipid Syndrome - complications; Antiphospholipid Syndrome - immunology; Antiphospholipid Syndrome - physiopathology; Antipsychotic Agents - therapeutic use; Arteries - physiopathology; Biological and medical sciences; Child; chorea; Chorea - drug therapy; Chorea - immunology; Chorea - physiopathology; Computer Science; Female; Follow-Up Studies; Humans; lupus; Lupus Erythematosus, Systemic - immunology; Lupus Erythematosus, Systemic - physiopathology; Male; Medical Imaging; Medical sciences; Middle Aged; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis; Neurology; Retrospective Studies; Risk; Steroids - therapeutic use; thrombosis; Thrombosis - immunology; Thrombosis - physiopathology; Time Factors; Treatment Outcome; Young Adult
• ISSN: 0885-3185; 1531-8257
• DOI: 10.1002/mds.23863

Objective: The aim of this work was to describe chorea during systemic lupus erythematosus or antiphospholipid antibodies and its long‐term outcome. Methods: We retrospectively analyzed clinical features, laboratory findings, imaging characteristics, and outcome in a series of 32 patients. Results: Most patients were women (28 of 32), and mean age at onset of chorea was 20.6 (9–62) years. Chorea was inaugural for 28 patients. Improvement was observed with various treatments. During follow‐up (12.2 ± 11.3 years), severe manifestations of systemic lupus erythematosus were rare. Antiphospholipid antibodies were repeatedly positive for 90% of the patients. Twelve patients developed arterial thrombosis. Prophylactic treatment with antithrombotic therapy might reduce the risk of further thrombosis (8% versus 57%; P = 0.01). Cardiac valvulopathy occurred in 22 patients during follow‐up. Chorea relapsed in 8 cases. Conclusions: Chorea had a good outcome in itself. This long‐term follow‐up shows, for the first time, that these patients have substantial risk for further arterial thrombosis. © 2011 Movement Disorder Society