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Review

Recent advances in the immunogenetics of idiopathic inflammatory myopathy

Hector Chinoy12, Janine A Lamb3, William ER Ollier3 and Robert G Cooper13*

Author Affiliations

1 Rheumatic Diseases Centre, Manchester Academic Health Science Centre, The University of Manchester, Salford Royal NHS Foundation Trust, Stott Lane, Salford, M6 8HD, UK

2 Musculoskeletal Research Group, School of Translational Medicine, Manchester Academic Health Science Centre, The University of Manchester, Oxford Road, M13 9PT Manchester, UK

3 Centre for IIntegrated Genomic Medical Research, Manchester Academic Health Science Centre, The University of Manchester, Oxford Road, M13 9PT Manchester, UK

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Arthritis Research & Therapy 2011, 13:216  doi:10.1186/ar3327

Published: 26 May 2011

Abstract

This review summarizes the previous and current literature on the immunogenetics of idiopathic inflammatory myopathy (IIM) and updates the research progress that has been made over the past decade. A substantial part of the genetic risk for developing adult- and juvenile-onset IIM lies within the major histocompatibility complex (MHC), and a tight relationship exists between individual human leukocyte antigen alleles and specific serological subtypes, which in turn dictate clinical disease phenotypes. Multiple genetic regions outside of the MHC are increasingly being identified in conferring IIM disease susceptibility. We are still challenged with the task of studying a serologically and clinically heterogeneous disorder that is rarer by orders of magnitude than the likes of rheumatoid arthritis. An ongoing and internationally coordinated IIM genome-wide association study may provide further insights into IIM immunogenetics.