Seminars in arthritis and rheumatism · 2026
Spectrum of clinical phenotypes associated with myositis-specific and myositis-associated antibodies in juvenile idiopathic inflammatory myositis: Our experience from North India.
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Vignesh Pandiarajan, Basu Suprit, Nadig Pallavi L, Sivagnanaganesan Sivanesan, Dod Aditya, Kumrah Rajni, Garg Ravinder, Khalko Phooljems, Sudhakar Murugan, Tyagi Reva, Dhaliwal Manpreet, Sharma Saniya, Pilania Rakesh Kumar, Jindal Ankur Kumar, Suri Deepti, Singh Surjit, Rawat Amit
Аннотация
BACKGROUND: Myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) are increasingly being identified in juvenile inflammatory myositis (JIIM). The current study's objective was to characterize the incidence and clinical-phenotypic profile of MSA/MAA in a cohort of JIIM from North India.
METHODS: We reviewed the medical records of the Pediatric Rheumatology Clinic of a tertiary care referral centre in Chandigarh, North India (1992 to 2024) and analysed the clinical details of children with JIIM. MSA/MAAs were assayed using a 16-antigen kit immunoblot assay (Euroline Autoimmune Inflammatory Myopathies 16 Ag, Euroimmune, Lübeck, Germany).
RESULTS: Of the 173 patients of JIIM in our cohort, assay for MSA/MAA was performed in 113 patients which included juvenile dermatomyositis (n=89), clinically amyopathic dermatomyositis (n=8), overlap myositis (n=13), and juvenile polymyositis (n=3). Autoantibody positivity was seen in 72.5% (82/113; MSA =70, MAA=12) of the children, with anti-NXP2 (n=24) being the most common followed by anti-MDA5 (n=14), anti-TIF-1γ (n=12), anti-Mi 2 (n=9) in that order. Patients with NXP2 positivity were younger at onset [3.15 years; (1.2-11.88 years) p=0.015], had severe muscle weakness at onset (p=0.04) and persistent calcinosis (p=0.004) in follow-up. Patients with anti-TIF1γ antibody were observed to have persistent skin lesions (p <0.001) during follow-up. Incidence of arthritis (p=0.001), inverse Gottron papules (p=0.003), skin ulceration (p=0.065), oral ulcers (p=0.012), and ILD (p<0.001) were high in anti-MDA5-JDM.
CONCLUSION: Anti-NXP2 antibody is the most common MSA noted in our North Indian cohort of JIIM. The findings emphasize the importance of region-specific data in understanding disease variability and guiding management.
METHODS: We reviewed the medical records of the Pediatric Rheumatology Clinic of a tertiary care referral centre in Chandigarh, North India (1992 to 2024) and analysed the clinical details of children with JIIM. MSA/MAAs were assayed using a 16-antigen kit immunoblot assay (Euroline Autoimmune Inflammatory Myopathies 16 Ag, Euroimmune, Lübeck, Germany).
RESULTS: Of the 173 patients of JIIM in our cohort, assay for MSA/MAA was performed in 113 patients which included juvenile dermatomyositis (n=89), clinically amyopathic dermatomyositis (n=8), overlap myositis (n=13), and juvenile polymyositis (n=3). Autoantibody positivity was seen in 72.5% (82/113; MSA =70, MAA=12) of the children, with anti-NXP2 (n=24) being the most common followed by anti-MDA5 (n=14), anti-TIF-1γ (n=12), anti-Mi 2 (n=9) in that order. Patients with NXP2 positivity were younger at onset [3.15 years; (1.2-11.88 years) p=0.015], had severe muscle weakness at onset (p=0.04) and persistent calcinosis (p=0.004) in follow-up. Patients with anti-TIF1γ antibody were observed to have persistent skin lesions (p <0.001) during follow-up. Incidence of arthritis (p=0.001), inverse Gottron papules (p=0.003), skin ulceration (p=0.065), oral ulcers (p=0.012), and ILD (p<0.001) were high in anti-MDA5-JDM.
CONCLUSION: Anti-NXP2 antibody is the most common MSA noted in our North Indian cohort of JIIM. The findings emphasize the importance of region-specific data in understanding disease variability and guiding management.