Exercise as a multi-modal disease-modifying medicine in systemic sclerosis: An introduction by The Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS)

Henrik Pettersson; Helene Alexanderson; Janet L Poole; Janos Varga; Malin Regardt; Anne-Marie Russell; Yasser Salam; Kelly Jensen; Jennifer Mansour; Tracy Frech; Carol Feghali-Bostwick; Cecília Varjú; Nancy Baldwin; Matty Heenan; Kim Fligelstone; Monica Holmner; Matthew R Lammi; Mary Beth Scholand; Lee Shapiro; Elizabeth R Volkmann; Lesley Ann Saketkoo

doi:10.1016/j.berh.2021.101695

Exercise as a multi-modal disease-modifying medicine in systemic sclerosis: An introduction by The Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS)

Best Pract Res Clin Rheumatol. 2021 Sep;35(3):101695. doi: 10.1016/j.berh.2021.101695. Epub 2021 Jul 1.

Authors

Henrik Pettersson¹, Helene Alexanderson¹, Janet L Poole², Janos Varga³, Malin Regardt⁴, Anne-Marie Russell⁵, Yasser Salam⁶, Kelly Jensen⁷, Jennifer Mansour⁸, Tracy Frech⁹, Carol Feghali-Bostwick¹⁰, Cecília Varjú¹¹, Nancy Baldwin¹², Matty Heenan¹³, Kim Fligelstone¹⁴, Monica Holmner¹⁵, Matthew R Lammi¹⁶, Mary Beth Scholand¹⁷, Lee Shapiro¹⁸, Elizabeth R Volkmann¹⁹, Lesley Ann Saketkoo²⁰

Affiliations

¹ Women's Health and Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden; Division of Rheumatology, Department of Medicin, Solna, Karolinska Institutet, Stockholm, Sweden.
² Occupational Therapy Graduate Program, University of New Mexico, Albuquerque, NM, USA.
³ Department of Pulmonology, Semmelweis University, Budapest, Hungary.
⁴ Women's Health and Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden; Department of Occupational Therapy, Karolinska Institutet, Stockholm, Sweden.
⁵ University of Exeter, College of Medicine and Health, Exeter, UK; National Institute of Health Research, Senior Nurse Research Leader, London, UK.
⁶ Department of Physical Therapy, University of North Texas Health Science Center, Fort Worth, TX, USA.
⁷ Oregon Health and Science University, Portland, OR, USA; New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; Tulane University School of Medicine, New Orleans, USA.
⁸ New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; Tulane University School of Medicine, New Orleans, USA.
⁹ Vanderbilt University, Division of Rheumatology, Nashville, TN, USA.
¹⁰ Department of Medicine, Medical University of South Carolina, Charleston, SC, USA.
¹¹ Department of Rheumatology and Immunology, University of Pécs Clinical Center, Pecs, Hungary.
¹² Scleroderma Foundation, Chicago, IL, USA.
¹³ Scleroderma Foundation/Pulmonary Hypertension Association, Tucson, AZ, USA.
¹⁴ Scleroderma & Raynaud Society UK (SRUK), London, UK; Royal Free Hospital, London, UK.
¹⁵ The Swedish Rheumatism Association National Association for Systemic Sclerosis, Sweden.
¹⁶ New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; University Medical Center - Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, USA; Louisiana State University School of Medicine, Section of Pulmonary Medicine, New Orleans, USA.
¹⁷ University of Utah, Division of Pulmonary Medicine, Pulmonary Fibrosis Center, Salt Lake City, UT, USA.
¹⁸ Division of Rheumatology, Albany Medical Center, Albany, NY, USA; Steffens Scleroderma Foundation, Albany, NY, USA.
¹⁹ University of California, David Geffen School of Medicine, UCLA Scleroderma Program and UCLA CTD-ILD Program, Division of Rheumatology, Department of Medicine, Los Angeles, CA, USA.
²⁰ New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; Tulane University School of Medicine, New Orleans, USA; University Medical Center - Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, USA; Louisiana State University School of Medicine, Section of Pulmonary Medicine, New Orleans, USA. Electronic address: lsaketk@tulane.edu.

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.

Keywords: Disability; Exercise; Health-related quality of life; Interstitial lung disease; Muscle; Myokine; Physical activity; Pulmonary hypertension; Pulmonary rehabilitation; Scleroderma.

Publication types

Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review

MeSH terms

Activities of Daily Living
Exercise
Fellowships and Scholarships*
Fibrosis
Humans
Scleroderma, Systemic* / therapy

Abstract

Publication types

MeSH terms

Grants and funding