Abstract
Systemic sclerosis (SSc) is a heterogeneous multisystem autoimmune disease whereby its main pathological drivers of disability and damage are vascular injury, inflammatory cell infiltration, and fibrosis. These mechanisms result in diffuse and diverse impairments arising from ischemic circulatory dysfunction leading to painful skin ulceration and calcinosis, neurovascular aberrations hindering gastrointestinal (GI) motility, progressive painful, incapacitating or immobilizing effects of inflammatory and fibrotic effects on the lungs, skin, articular and periarticular structures, and muscle. SSc-related impairments impede routine activities of daily living (ADLs) and disrupt three critical life areas: work, family, social/leisure, and also impact on psychological well-being. Physical activity and exercise are globally recommended; however, for connective tissue diseases, this guidance carries greater impact on inflammatory disease manifestations, recovery, and cardiovascular health. Exercise, through myogenic and vascular phenomena, naturally targets key pathogenic drivers by downregulating multiple inflammatory and fibrotic pathways in serum and tissue, while increasing circulation and vascular repair. G-FoRSS, The Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis recognizes the scientific basis of and advocates for education and research of exercise as a systemic and targeted SSc disease-modifying treatment. An overview of biophysiological mechanisms of physical activity and exercise are herein imparted for patients, clinicians, and researchers, and applied to SSc disease mechanisms, manifestations, and impairment. A preliminary guidance on exercise in SSc, a research agenda, and the current state of research and outcome measures are set forth.
Original language | English |
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Article number | 101695 |
Number of pages | 41 |
Journal | Best Practice and Research: Clinical Rheumatology |
Volume | 35 |
Issue number | 3 |
Early online date | 1 Jul 2021 |
DOIs | |
Publication status | Published - Sept 2021 |
Bibliographical note
Funding Information:Swedish Research Council (HA, HP), Swedish Rheumatism Association (HA, HP), Promobilia Foundation (HA) National Institute of Health Research UK (AMR), Pulmonary Fibrosis Trust UK (AMR), Irish Lung Fibrosis Association (ILFA) (AMR), National Institutes of Health : K24 AR060297 (CFB) and L30 HL129466 (MRL), National Heart, Lung, Blood Institute K23HL150237-02 (ERV); Charles and Elizabeth Wetmore Foundation of Greater New Orleans (LAS), and Sarcoidosis Awareness Foundation of Louisiana (SAFOL) (LAS).
Publisher Copyright:
© 2021 Elsevier Ltd
Keywords
- Disability
- Exercise
- Health-related quality of life
- Interstitial lung disease
- Muscle
- Myokine
- Physical activity
- Pulmonary hypertension
- Pulmonary rehabilitation
- Scleroderma
ASJC Scopus subject areas
- Rheumatology