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Systemic Sclerosis-Associated Myopathy: How to Treat

  • Other CTD: Inflammatory Myopathies (J Milisenda, Section Editor)
  • Published:
Current Treatment Options in Rheumatology Aims and scope Submit manuscript

Abstract

Purpose of Review

Systemic sclerosis (SSc) and myositis are two different entities that may coexist as an overlap syndrome. Immunological biomarkers such as anti-PM/Scl or anti-Ku reinforce the syndrome. This review is focused on the treatment of different and characteristic manifestations of this syndrome.

Recent Findings

Among the different phenotypes of muscle involvement in patients with SSc, the fibrotic pattern and the sporadic inclusion body myositis must be identified early to avoid a futile immunosuppressive treatment. Other forms such as dermatomyositis, non-specific myositis, and immune-mediated necrotizing myopathy need to receive conventional immunosuppressive therapy considering that high dose of glucocorticoids may induce a scleroderma renal crisis in patients with SSc. Physicians must be aware of the existence of a “double trouble” association of hereditary myopathy with an autoimmune phenomenon. Several autoantibodies, mainly anti-PM/Scl and anti-Ku, may help to define specific phenotypes with characteristic clinical manifestations that need a more specific therapy. Vasculopathy is one of the underlying mechanisms that link SSc and myositis. Recent advances in this topic are reviewed.

Summary

Current treatment of SSc-associated myopathy must be tailored to specific organs involved. Identifying the specific clinical, pathological, and immunological phenotypes may help to take the correct therapeutic decisions.

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Funding

Instituto de Salud Carlos III co-financed by the European Regional Development Fund (ERDF) PI18/01609. Grant recipient: Albert Selva-O’Callaghan.

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Authors and Affiliations

  1. Systemic Autoimmune Diseases Unit, Internal Medicine Department, Universitat Autónoma de Barcelona, Vall d’Hebron Hospital, 08035, Barcelona, Spain

    A. Selva-O’Callaghan, A. Guillen-Del-Castillo, A. Gil-Vila & C. Simeón-Aznar

  2. Rheumatology Department, Vall d’Hebron Hospital, Barcelona, Spain

    E. Trallero-Araguás

  3. Muscle Research Unit, Internal Medicine Service, Hospital Clinic de Barcelona (HCB), Universidad de Barcelona and Center for Biomedical Research On Rare Diseases (CIBERER), Barcelona, Spain

    A. Matas-García & J. C. Milisenda

  4. Muscle Disease Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA

    I. Pinal-Fernández

  5. Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    I. Pinal-Fernández

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  1. A. Selva-O’Callaghan
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A. Selva-O’Callaghan, A. Guillen-Del-Castillo, A. Gil-Vila, E. Trallero-Araguás, A. Matas-García, J. C. Milisenda, I. Pinal-Fernández, C. Simeón-Aznar each declare no potential conflicts of interest.

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Selva-O’Callaghan, A., Guillen-Del-Castillo, A., Gil-Vila, A. et al. Systemic Sclerosis-Associated Myopathy: How to Treat. Curr Treat Options in Rheum 9, 151–167 (2023). https://doi.org/10.1007/s40674-023-00206-y

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