
The study investigated the prevalence and clinical features of anti-Sp4 autoantibodies in juvenile-onset idiopathic inflammatory myopathies (IIM). The study found that anti-Sp4 autoantibodies were present in 7% of patients with juvenile myositis and predominantly in those with co-existing anti-TIF1 autoantibodies. Patients with anti-Sp4 autoantibodies represented a phenotypic subset of anti-TIF1 autoantibody-positive myositis characterized by frequent Raynaud's phenomenon and less pronounced muscle involvement. The study also identified novel immunogenetic risk factors for White patients with IIM who had anti-Sp4 autoantibodies.
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