1. Inclusion-body myositis and primary Sjögren syndrome: mechanisms for shared etiologies.
- Author
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Limaye VS, Cash K, Smith C, Koszyca B, Patel S, Greenberg SA, and Hissaria P
- Subjects
- Adult, Azathioprine therapeutic use, CD8-Positive T-Lymphocytes pathology, Female, HLA Antigens genetics, Haplotypes, Humans, Hydroxychloroquine therapeutic use, Immunoglobulins, Intravenous therapeutic use, Immunologic Factors therapeutic use, Leukemia, Large Granular Lymphocytic complications, Magnetic Resonance Imaging, Methotrexate therapeutic use, Muscle, Skeletal diagnostic imaging, Muscle, Skeletal pathology, Myositis, Inclusion Body complications, Myositis, Inclusion Body pathology, Myositis, Inclusion Body therapy, Prednisolone therapeutic use, Rituximab therapeutic use, Sjogren's Syndrome complications, Sjogren's Syndrome therapy, 5'-Nucleotidase immunology, Autoantibodies immunology, Leukemia, Large Granular Lymphocytic immunology, Myositis, Inclusion Body immunology, Sjogren's Syndrome immunology
- 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., (© 2020 Wiley Periodicals, Inc.)
- Published
- 2020
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