Publications by authors named "I N Targoff"
Objective: Myositis-associated autoantibodies (MAAs) have been associated with overlap myositis, certain disease manifestations such as interstitial lung disease (ILD), and worse prognosis in the idiopathic inflammatory myopathies. MAAs overall remain largely uncharacterized in patients with juvenile-onset myositis. Moreover, it is unknown whether the number of MAAs is associated with disease severity.
View Article and Find Full Text PDFObjective: Autoantibodies recognizing specificity protein 4 (Sp4) were recently discovered in adults with idiopathic inflammatory myopathies (IIM). Anti-Sp4 autoantibodies co-occurred in patients with anti-transcription intermediary factor 1 (anti-TIF1) autoantibody-positive dermatomyositis (DM) and were associated with a reduced risk of cancer. In the present study, the prevalence and clinical features associated with anti-Sp4 autoantibodies in juvenile-onset IIM were investigated.
View Article and Find Full Text PDFArticle Synopsis
- Idiopathic inflammatory myopathies (IIM) are autoimmune diseases causing muscle and skin inflammation, leading to symptoms like chronic weakness and fatigue, with complement-mediated destruction involved in their pathology.
- A study analyzed gene copy number variations in 1644 IIM patients and 3526 healthy controls, finding low GCNs and complement deficiencies significantly increased the risk of IIM.
- Results indicated that complement deficiency is particularly relevant in cases of dermatomyositis and polymyositis, while a specific gene was linked to a high risk of inclusion body myositis (IBM).
Objectives: Four-and-a-half LIM domains 1 (FHL1) is a muscle-specific protein. Autoantibodies against FHL1 were recently discovered in adults with idiopathic inflammatory myopathies (IIMs) and were found to be associated with clinical features and outcomes indicative of increased disease severity. Anti-FHL1 autoantibodies have not been described in children.
View Article and Find Full Text PDFBackground: Environmental exposures have been associated with the juvenile idiopathic inflammatory myopathies (JIIM). We undertook a questionnaire-based study to evaluate patient-reported exposures as possible risk factors for JIIM.
Findings: One-hundred-seven patients with JIIM were enrolled in a myositis natural history protocol and completed environmental questionnaires.