OBJECTIVE: We aimed to establish the prevalence and clinical associations of anti-HMG-CoA-reductase (anti-HMGCR) in a large UK cohort with juvenile myositis. METHODS: There were 381 patients investigated for anti-HMGCR using ELISA. RESULTS: Anti-HMGCR autoantibodies were detected in 4 patients (1%). These children had no or minimal rash and significant muscle disease. Muscle biopsies were considered distinctive, with widespread variation in fiber size, necrotic fibers, and chronic inflammatory cell infiltrates; all had prolonged elevation of creatine kinase and all ultimately received biologic therapies. CONCLUSION: Anti-HMGCR in UK children with myositis are associated with severe disease that is poorly responsive to standard treatment.
OBJECTIVE: We aimed to establish the prevalence and clinical associations of anti-HMG-CoA-reductase (anti-HMGCR) in a large UK cohort with juvenile myositis. METHODS: There were 381 patients investigated for anti-HMGCR using ELISA. RESULTS: Anti-HMGCR autoantibodies were detected in 4 patients (1%). These children had no or minimal rash and significant muscle disease. Muscle biopsies were considered distinctive, with widespread variation in fiber size, necrotic fibers, and chronic inflammatory cell infiltrates; all had prolonged elevation of creatine kinase and all ultimately received biologic therapies. CONCLUSION: Anti-HMGCR in UK children with myositis are associated with severe disease that is poorly responsive to standard treatment.
Authors: Sarah L Tansley; Stefania Simou; Gavin Shaddick; Zoe E Betteridge; Beverley Almeida; Harsha Gunawardena; Wendy Thomson; Michael W Beresford; Angela Midgley; Francesco Muntoni; Lucy R Wedderburn; Neil J McHugh Journal: J Autoimmun Date: 2017-06-26 Impact factor: 7.094
Authors: E L Binns; E Moraitis; S Maillard; S Tansley; N McHugh; T S Jacques; L R Wedderburn; C Pilkington; S A Yasin; K Nistala Journal: Pediatr Rheumatol Online J Date: 2017-10-31 Impact factor: 3.054
Authors: Claas H Hinze; Prasad T Oommen; Frank Dressler; Andreas Urban; Frank Weller-Heinemann; Fabian Speth; Elke Lainka; Jürgen Brunner; Heike Fesq; Dirk Foell; Wolfgang Müller-Felber; Ulrich Neudorf; Christoph Rietschel; Tobias Schwarz; Ulrike Schara; Johannes-Peter Haas Journal: Pediatr Rheumatol Online J Date: 2018-06-25 Impact factor: 3.054
Authors: Judith Wienke; Claire T Deakin; Lucy R Wedderburn; Femke van Wijk; Annet van Royen-Kerkhof Journal: Front Immunol Date: 2018-12-18 Impact factor: 7.561