The association of sporadic inclusion body myositis and Sjögren's syndrome in carriers of HLA-DR3 and the 8.1 MHC ancestral haplotype

A. Rojana-udomsart; M. Needham; Y.B. Luo; V. Fabian; S. Walters; P.J. Zilko; F.L. Mastaglia

doi:10.1016/j.clineuro.2011.03.016

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The association of sporadic inclusion body myositis and Sjögren's syndrome in carriers of HLA-DR3 and the 8.1 MHC ancestral haplotype

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The association of sporadic inclusion body myositis and Sjögren's syndrome in carriers of HLA-DR3 and the 8.1 MHC ancestral haplotype

A. Rojana-udomsart, M. Needham, Y.B. Luo, V. Fabian, S. Walters, P.J. Zilko and F.L. Mastaglia

Clinical Neurology and Neurosurgery, Vol.113(7), pp.559-563

2011

DOI: https://doi.org/10.1016/j.clineuro.2011.03.016

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Abstract

Sporadic inclusion body myositis (sIBM) usually occurs as an isolated condition, but it may occur in association with another autoimmune disorder such as Sjögren's syndrome. We reviewed sIBM cases with Sjögren's syndrome (sIBM/SS) from the Perth Inflammatory Myopathies Database to determine whether they are distinguishable from other sIBM cases. Six such cases were identified, representing 12% of all sIBM cases. Muscle biopsies confirmed the presence of an inflammatory myopathy with rimmed vacuoles and the characteristic muscle fibre inclusions of sIBM. Five of the six were females, contrasting with a 2:1 male preponderance in the rest of the sIBM cohort. The mean age-at-onset and the pattern of muscle weakness were similar in the two groups. Four out of five sIBM/SS patients treated with immune therapies had improvement in muscle strength lasting for 6–24 months, whereas only 27% of other sIBM patients improved. All 6 patients with sIBM/SS carried the HLA-DRB1*0301 allele, or its equivalent HLA-DR3 serological specificity, compared with 83% of other sIBM cases and all carried some or all of the major markers of the 8.1 MHC ancestral haplotype which is also associated with Sjögren's syndrome. Patients with sIBM/SS represent a subgroup of sIBM cases who are more likely to be female and carriers of HLA-DR3 and the 8.1 MHC ancestral haplotype, and are more likely to respond to treatment. The association of sIBM and Sjögren's syndrome is likely to be due to a common genetic predisposition linked to the MHC and supports the notion that sIBM has an autoimmune basis.

Details

Title: The association of sporadic inclusion body myositis and Sjögren's syndrome in carriers of HLA-DR3 and the 8.1 MHC ancestral haplotype
Authors/Creators: A. Rojana-udomsart (Author/Creator) - The University of Western Australia
M. Needham (Author/Creator) - Royal North Shore Hospital
Y.B. Luo (Author/Creator) - The University of Western Australia
V. Fabian (Author/Creator) - Royal Perth Hospital
S. Walters (Author/Creator) - The University of Western Australia
P.J. Zilko (Author/Creator) - The University of Western Australia
F.L. Mastaglia (Author/Creator) - The University of Western Australia
Publication Details: Clinical Neurology and Neurosurgery, Vol.113(7), pp.559-563
Publisher: © 2011 Elsevier B.V.
Identifiers: 991005542378707891
Copyright: 2011 Elsevier B.V.
Murdoch Affiliation: Murdoch University
Language: English
Resource Type: Journal article

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Collaboration types: Domestic collaboration; International collaboration
Citation topics: 1 Clinical & Life Sciences; 1.106 Rheumatology; 1.106.1684 Dermatomyositis
Web Of Science research areas: Clinical Neurology; Surgery
ESI research areas: Clinical Medicine