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

Lesley Ann Saketkoo*, Tracy Frech, Cecília Varjú, Robyn Domsic, Jessica Farrell, Jessica K. Gordon, Carina Mihai, Nora Sandorfi, Lee Shapiro, Janet Poole, Elizabeth R. Volkmann, Monika Lammi, Kendra McAnally, Helene Alexanderson, Henrik Pettersson, Faye Hant, Masataka Kuwana, Ami A. Shah, Vanessa Smith, Vivien HsuOtylia Kowal-Bielecka, Shervin Assassi, Maurizio Cutolo, Cristiane Kayser, Victoria K. Shanmugam, Madelon C. Vonk, Kim Fligelstone, Nancy Baldwin, Kerri Connolly, Anneliese Ronnow, Beata Toth, Maureen Suave, Sue Farrington, Elana J. Bernstein, Leslie J. Crofford, László Czirják, Kelly Jensen, Monique Hinchclif, Marie Hudson, Matthew R. Lammi, Jennifer Mansour, Nadia D. Morgan, Fabian Mendoza, Mandana Nikpour, John Pauling, Gabriela Riemekasten, Anne Marie Russell, Mary Beth Scholand, Elise Seigart, Tatiana Sofia Rodriguez-Reyna, Laura Hummers, Ulrich Walker, Virginia Steen

*Corresponding author for this work
14 Citations (Scopus)


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.

Original languageEnglish
Article number101707
JournalBest Practice and Research: Clinical Rheumatology
Issue number3
Publication statusPublished - 09.2021

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


Dive into the research topics of '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'. Together they form a unique fingerprint.

Cite this