Title

A comprehensive framework for navigating patient care in systemic sclerosis: A global response to the need for improving the practice of diagnostic and preventive strategies in SSc

Authors

Lesley Ann Saketkoo, New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; Tulane University School of Medicine, New Orleans, USA; Louisiana State University School of Medicine, Section of Pulmonary Medicine, New Orleans, USA; University Medical Center - Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, USA
Tracy Frech, Vanderbilt University Medical Center, Nashville, TN, USA
Cecília Varjú, Department of Rheumatology and Immunology, Medical School, University of Pécs, Pécs, Hungary
Robyn Domsic, University of Pittsburgh, Pittsburgh, PA, USA
Jessica Farrell, Albany College of Pharmacy and Health Sciences, Albany, NY, USA; Steffens Scleroderma Foundation, Albany, NY, USA
Jessica K. Gordon, Department of Rheumatology at Hospital for Special Surgery, New York, NY, USA
Carina Mihai, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland; Department of Internal Medicine and Rheumatology, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
Nora Sandorfi, Perelman School of Medicine, Philadelphia, PA, USA
Lee Shapiro, Steffens Scleroderma Foundation, Albany, NY, USA; Division of Rheumatology, Albany Medical Center, Albany, NY, USA
Janet L. Poole, Occupational Therapy Graduate Program, University of New Mexico, Albuquerque, NM, USA
Elizabeth R. Volkmann, 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
Monika Lammi, Ochsner Medical Center, New Orleans, LA, USA
Kendra McAnally, Norton Thoracic Institute, St. Joseph's Hospital and Medical Centre, Phoenix, AZ, USA
Helene Alexanderson, Function Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden; Department of Medicin, Solna, Karolinska Institutet, Stockholm, Sweden
Henrik Pettersson, Function Allied Health Professionals, Medical Unit Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden; Department of Medicin, Solna, Karolinska Institutet, Stockholm, Sweden
Faye Hant, Division of Rheumatology, Medical University of South Caroline, SC, USA
Masataka Kuwana, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan
Ami A. Shah, Department of Medicine, Johns Hopkins University, Baltimore, MD, USA
Vanessa Smith, Department of Internal Medicine, Ghent University, and Department of Rheumatology, Ghent University Hospital, Ghent, Belgium
Vivien Hsu, Rutgers- RWJ Scleroderma Program, New Brunswick, NJ, USA
Otylia Kowal-Bielecka, Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland
Shervin Assassi, Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, USA
Maurizio Cutolo, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Polyclinic San Martino Hospital, Genova, Italy
Cristiane Kayser, Escola Paulista de Medicina, Federal University of São Paulo (UNIFESP) São Paulo, SP, Brazil
Victoria K. Shanmugam, Department of Rheumatology, George Washington University, School of Medicine and Health Sciences, Washington, DC, USA
Madelon C. Vonk, Department of the rheumatic diseases, Radboud University Medical Center, Nijmegen, the Netherlands
Kim Fligelstone, Patient Research Partner, Scleroderma & Raynaud Society UK (SRUK), London, UK; Royal Free Hospital, London, UK
Nancy Baldwin, Patient Research Partner, Scleroderma Foundation, Chicago, IL, USA
Kerri Connolly, Scleroderma Foundation, Danvers, MA, USA
Anneliese Ronnow, Federation of European Scleroderma Associations, Copenhagen, Denmark; Federation of European Scleroderma Associations, Budapest, Hungary; Federation of European Scleroderma Associations, London, UK
Beata Toth, Federation of European Scleroderma Associations, Copenhagen, Denmark; Federation of European Scleroderma Associations, Budapest, Hungary; Federation of European Scleroderma Associations, London, UK
Maureen Suave, Scleroderma Canada, Canada
Sue Farrington, Patient Research Partner, Scleroderma & Raynaud Society UK (SRUK), London, UK; Federation of European Scleroderma Associations, Copenhagen, Denmark; Federation of European Scleroderma Associations, Budapest, Hungary; Federation of European Scleroderma Associations, London, UK
Elana J. Bernstein, Columbia University/New York-Presbyterian Scleroderma Program, Division of Rheumatology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA
Leslie J. Crofford, Vanderbilt University Medical Center, Nashville, TN, USA
László Czirják, Department of Rheumatology and Immunology, Medical School, University of Pécs, Pécs, Hungary
Kelly Jensen, Tulane University School of Medicine, New Orleans, USA; Oregon Health and Science University, Portland, OR, USA
Monique Hinchclif, Yale School of Medicine, Department of Internal Medicine, Section of Rheumatology, Allergy & Immunology, USA
Marie Hudson, Division of heumatology and Department of Medicine, Jewish General Hospital and McGill University, Montreal, QC, Canada
Matthew R Lammi, New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; Louisiana State University School of Medicine, Section of Pulmonary Medicine, New Orleans, USA; University Medical Center - Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, USA
Jennifer Mansour, Tulane University School of Medicine, New Orleans, USA
Nadia D. Morgan, Department of Medicine, Johns Hopkins University, Baltimore, MD, USA
Fabian Mendoza, Rheumatology Division, Department of Medicine, Thomas Jefferson University, Philadelphia, PA, USA
Mandana Nikpour, Jefferson Institute of Molecular Medicine and Scleroderma Center, Thomas Jefferson University, Philadelphia, PA, USA
John Pauling, University of Melbourne, Melbourne at St. Vincent's Hospital Melbourne, Victoria, Australia
Gabriela Riemekasten, Royal National Hospital for Rheumatic Diseases, Bath, UK; University of Lübeck, University Clinic of Schleswig-Holstein, Dept Rheumatology and Clinical Immunology, Lübeck, Germany
Anne-Marie Russell, University of Exeter, College of Medicine and Health, Exeter, UK
Mary Beth Scholand, University of Utah, Division of Pulmonary Medicine, Pulmonary Fibrosis Center, Salt Lake City, UT, USA
Elise Seigart, Department of Rheumatology and Clinical Immunology Charité - Universitätsmedizin Berlin and Berlin Institute of Health, Berlin, Germany
Tatiana Sofia Rodriguez-Reyna, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
Laura Hummers, Department of Medicine, Johns Hopkins University, Baltimore, MD, USA
Ulrich Walker, Dept. of Rheumatology, Basel University Hospital, Basel, Switzerland
Virginia Steen, Division of Rheumatology, Department of Medicine, Georgetown University, Washington, DC, USA

Document Type

Article

Publication Date

9-1-2021

Abstract

Systemic sclerosis (SSc), the most lethal of rheumatologic conditions, is the cause of death in >50% of SSc cases, led by pulmonary fibrosis followed by pulmonary hypertension and then scleroderma renal crisis (SRC). Multiple other preventable and treatable SSc-related vascular, cardiac, gastrointestinal, nutritional and musculoskeletal complications can lead to disability and death. Vascular injury with subsequent inflammation transforming to irreversible fibrosis and permanent damage characterizes SSc. Organ involvement is often present early in the disease course of SSc, but requires careful history-taking and vigilance in screening to detect. Inflammation is potentially reversible provided that treatment intensity quells inflammation and other immune mechanisms. In any SSc phenotype, opportunities for early treatment are prone to be under-utilized, especially in slowly progressive phenotypes that, in contrast to severe progressive ILD, indolently accrue irreversible organ damage resulting in later-stage life-limiting complications such as pulmonary hypertension, cardiac involvement, and malnutrition. A single SSc patient visit often requires much more physician and staff time, organization, vigilance, and direct management for multiple organ systems compared to other rheumatic or pulmonary diseases. Efficiency and efficacy of comprehensive SSc care enlists trending of symptoms and bio-data. Financial sustainability of SSc care benefits from understanding insurance reimbursement and health system allocation policies for complex patients. Sharing care between recognised SSc centers and local cardiology/pulmonary/rheumatology/gastroenterology colleagues may prevent complications and poor outcomes, while providing support to local specialists. As scleroderma specialists, we offer a practical framework with tools to facilitate an optimal, comprehensive and sustainable approach to SSc care. Improved health outcomes in SSc relies upon recogntion, management and, to the extent possible, prevention of SSc and treatment-related complications.

Publication Title

Best practice & research. Clinical rheumatology

ISSN

1532-1770

Volume

35

Issue

3

First Page

101707

Last Page

101707

DOI

10.1016/j.berh.2021.101707

Comments

  • PMCID: PMC8670736 (available on 2022-09-15)

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