Key result
Huntington's disease linked to ~138% higher anti-AT1R auto-antibody titers versus healthy controls.
Why the study?
A role of the immune system in Huntington's disease is increasingly recognized, but the presence of T cell activating auto-antibodies against angiotensin II type 1 receptors in HD compared to controls and MS patients was not investigated.
Are auto-antibodies against angiotensin II type 1 receptors (AT1R) increased in patients with Huntington's disease compared to healthy controls and multiple sclerosis patients?
Cross-Sectional (n=358)
Single-blind
Yes
Are auto-antibodies against angiotensin II type 1 receptors (AT1R) increased in patients with Huntington's disease compared to healthy controls and multiple sclerosis patients?
Absolute Event Rate: 20.5% vs 8.6%
p-value: p=<0.001
Patients with Huntington's disease have increased levels of auto-antibodies against angiotensin II type 1 receptors, suggesting adaptive immune system dysfunction.
Supports immune dysregulation hypothesis in Huntington's disease; leaves open biomarker or pathogenic role pending prospective validation.
BACKGROUND: In the recent years, a role of the immune system in Huntington's disease (HD) is increasingly recognized. Here we investigate the presence of T cell activating auto-antibodies against angiotensin II type 1 receptors (AT1R) in all stages of the disease as compared to healthy controls and patients suffering from multiple sclerosis (MS) as a prototype neurologic autoimmune disease. RESULTS: As compared to controls, MS patients show higher titers of anti-AT1R antibodies, especially in individuals with active disease. In HD, anti-AT1R antibodies are more frequent than in healthy controls or even MS and occur in 37.9% of patients with relevant titers ≥ 20 U/ml. In a correlation analysis with clinical parameters, the presence of AT1R antibodies in the sera of HD individuals inversely correlated with the age of onset and positively with the disease burden score as well as with smoking and infection. CONCLUSIONS: These data suggest a dysfunction of the adaptive immune system in HD which may be triggered by different stimuli including autoimmune responses, infection and possibly also smoking.
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Lee et al. (2014) conducted a cross-sectional in Huntington's disease (n=358). Huntington's disease vs. Healthy controls and multiple sclerosis patients was evaluated on Mean anti-AT1R antibody titer (p=<0.001). Huntington's disease patients exhibited significantly higher mean anti-AT1R auto-antibody titers (20.5 U/ml) compared to healthy controls (8.6 U/ml) and multiple sclerosis patients (11.1 U/ml).
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