Patients with unexplained chorea should be tested for antiphospholipid antibodies
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Key takeaways:
- Most patients with chorea and antiphospholipid antibody were women, with chorea onset at an average age of 22.8 years.
- Classification criteria identified fewer than 50% of patients as having antiphospholipid syndrome.
Unexplained chorea, especially among women, should prompt testing for antibodies associated with antiphospholipid syndrome, according to data published in Lupus Science & Medicine.
“Chorea is a movement disorder characterized by irregular, random, involuntary and jerky movements affecting any part of the body,” Shikai Hu, of the department of rheumatology and clinical immunology at the Chinese Academy of Medical Sciences & Peking Union Medical College, in Beijing, and colleagues wrote.
“Current evidence on [antiphospholipid antibody (aPL)]-associated chorea is limited to narrative reviews, case reports and small case series, illustrating highly variable clinical characteristics and outcomes,” they added. “This variability complicates its diagnosis and treatments.”
To assess the current available evidence regarding the link between chorea and antiphospholipid antibody positivity, Hu and colleagues conducted a mixed-methods study of 180 patients. Focusing on patients with confirmed antiphospholipid antibody positivity and documented chorea, they drew 167 cases from literature and 13 from an incident cohort of admissions to the Peking Union Medical College Hospital.
Overall, 81.7% of the patients were women. According to the researchers, chorea was the initial symptom for 87.9%, with onset coming at an average age of 22.8 years. Most patients demonstrated only one episode of chorea (67%), commonly involving bilateral limbs (58.8%) and both upper and lower limbs (87.2%).
Meanwhile, fewer than half of the patients were identified as having antiphospholipid syndrome based on either the 2006 Sydney classification criteria (41.1%) or the 2023 American College of Rheumatology/EULAR classification criteria (43.3%). However, positron emission tomography scans revealed contralateral striatal hypermetabolism in all patients, according to the researchers.
Chorea was ultimately resolved in 74.8% of the patients and partially improved in 20.7%. In addition to anticoagulant and antiplatelet treatments, those who received glucocorticoids and immunosuppressive therapies had a higher rate of complete resolution (61.5%) than those who did not (48.6%).
“This study offers a thorough examination of antiphospholipid antibody-associated chorea, drawing on the most robust evidence from an incident cohort of 180 patients spanning the past 40 years,” Hu and colleagues wrote.
“Despite its rarity, chorea emerges as a noteworthy manifestation of [antiphospholipid syndrome],” they added. “Our findings underscore the crucial significance of promptly recognizing and testing for aPL in cases of unexplained chorea. This awareness is pivotal, as aPL-associated chorea is a treatable condition with a favorable outcome.”
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