Inclusion‐body myositis and primary Sjögren syndrome: mechanisms for shared etiologies
- 8 February 2020
- journal article
- research article
- Published by Wiley in Muscle & Nerve
- Vol. 61 (5), 570-574
- https://doi.org/10.1002/mus.26830
Abstract
Herein we report a case of sporadic inclusion‐body myositis (sIBM) occurring at an unusually young age in a patient with primary Sjögren syndrome, and use the case to explore possible shared mechanisms for disease susceptibility. Possible factors may include the association of both conditions with the 8.1 ancestral haplotype; the presence of anti‐cN1A antibodies, which, although considered specific for sIBM, are also seen in pSS; and the shared association with T‐cell large granular lymphocyte leukemia (T‐LGLL). Further evaluation of this patient did in fact reveal underlying T‐LGLL and mechanisms by which T cells in sIBM may escape immune regulation and contribute to disease phenotype are explored. Despite myofiber infiltration with CD8‐positive T cells in sIBM, and, although sIBM is traditionally considered treatment‐refractory, we report a significant response to the anti‐CD20 monoclonal antibody, rituximab, and discuss possible mechanisms by which this response may be mediated.Keywords
This publication has 24 references indexed in Scilit:
- Cytoplasmic 5′‐nucleotidase autoantibodies in inclusion body myositis: Isotypes and diagnostic utilityMuscle & Nerve, 2014
- Evaluation of Histologic, Serologic, and Clinical Changes in Response to Abatacept Treatment of Primary Sjögren's Syndrome: A Pilot StudyArthritis Care & Research, 2013
- Inclusion body myositis associated with Sjögren’s syndromeRheumatology International, 2012
- The association of sporadic inclusion body myositis and Sjögren's syndrome in carriers of HLA-DR3 and the 8.1 MHC ancestral haplotypeClinical Neurology and Neurosurgery, 2011
- Autoantibodies against a 43 KDa Muscle Protein in Inclusion Body MyositisPLOS ONE, 2011
- Expanded T cell receptor Vβ–restricted T cells from patients with sporadic inclusion body myositis are proinflammatory and cytotoxic CD28null T cellsArthritis & Rheumatism, 2010
- T Cell Large Granular Lymphocytic Leukemia in Association with Sjögren’s SyndromeActa Haematologica, 2010
- Inclusion body myositis in a patient with long standing rheumatoid arthritis treated with anti-TNFα and rituximabClinical Rheumatology, 2010
- Plasma cells in muscle in inclusion body myositis and polymyositisNeurology, 2005
- Myositis in a patient with large granular leukocyte leukemiaMuscle & Nerve, 2004