Russell Edward Pellar1, Janet Elizabeth Pope2. 1. Department of Medicine, University of Western Ontario, London, ON, Canada. 2. Department of Medicine, University of Western Ontario, London, ON, Canada; St Joseph Health Care, London, ON, Canada. Electronic address: janet.pope@sjhc.london.on.ca.
Abstract
OBJECTIVES: Systemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed. METHODS: The updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted. RESULTS: The updated EULAR/EUSTAR guidelines focus specifically on the management of SSc features and include data on newer therapeutic modalities and mention a research agenda. These recommendations are pharmacologic, with few guidelines regarding investigations and non-pharmacologic management. Recommendations from BSR/BHPR are similar to the organ manifestations mentioned in the EULAR/EUSTAR recommendations, and expand on several domains of treatment, including general measures, non-pharmacologic treatment, cardiac involvement, calcinosis, and musculoskeletal features. The guidelines usually agree with one another. Limitations include the lack of guidance for combination or second-line therapy, algorithmic suggestions, the absence of evidence-based recommendations regarding the treatment of specific complications (i.e., gastric antral ectasia and erectile dysfunction). Consensus for when to treat interstitial lung disease in SSc is lacking. There are differences between Europe and North American experts due to access and indications for certain therapies. CONCLUSIONS: Care gaps in SSc have been demonstrated so the EULAR/EUSTAR and BSR/BHP guidelines can promote best practices. Certain complications warrant active investigation to further improve outcomes in SSc and future updates of these recommendations. Care gaps in SSc have been demonstrated so the EULAR/EUSTAR and BSR/BHP guidelines can promote best practices. Certain complications warrant active investigation to further improve outcomes in SSc.
OBJECTIVES:Systemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed. METHODS: The updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted. RESULTS: The updated EULAR/EUSTAR guidelines focus specifically on the management of SSc features and include data on newer therapeutic modalities and mention a research agenda. These recommendations are pharmacologic, with few guidelines regarding investigations and non-pharmacologic management. Recommendations from BSR/BHPR are similar to the organ manifestations mentioned in the EULAR/EUSTAR recommendations, and expand on several domains of treatment, including general measures, non-pharmacologic treatment, cardiac involvement, calcinosis, and musculoskeletal features. The guidelines usually agree with one another. Limitations include the lack of guidance for combination or second-line therapy, algorithmic suggestions, the absence of evidence-based recommendations regarding the treatment of specific complications (i.e., gastric antral ectasia and erectile dysfunction). Consensus for when to treat interstitial lung disease in SSc is lacking. There are differences between Europe and North American experts due to access and indications for certain therapies. CONCLUSIONS: Care gaps in SSc have been demonstrated so the EULAR/EUSTAR and BSR/BHP guidelines can promote best practices. Certain complications warrant active investigation to further improve outcomes in SSc and future updates of these recommendations. Care gaps in SSc have been demonstrated so the EULAR/EUSTAR and BSR/BHP guidelines can promote best practices. Certain complications warrant active investigation to further improve outcomes in SSc.
Keywords:
Best practices; British Society of Rheumatology; European Scleroderma Research Group; Evidence based; Guidelines; ILD; Management; PAH; RP; Recommendations; Scleroderma; Systemic sclerosis; Treatment
Authors: Vanessa Smith; Carlo Alberto Scirè; Rosaria Talarico; Paolo Airo; Tobias Alexander; Yannick Allanore; Cosimo Bruni; Veronica Codullo; Virgil Dalm; Jeska De Vries-Bouwstra; Alessandra Della Rossa; Oliver Distler; Ilaria Galetti; David Launay; Gemma Lepri; Alexis Mathian; Luc Mouthon; Barbara Ruaro; Alberto Sulli; Angela Tincani; Els Vandecasteele; Amber Vanhaecke; Marie Vanthuyne; Frank Van den Hoogen; Ronald Van Vollenhoven; Alexandre E Voskuyl; Elisabetta Zanatta; Stefano Bombardieri; Gerd Burmester; Fonseca João Eurico; Charissa Frank; Eric Hachulla; Frederic Houssiau; Ulf Mueller-Ladner; Matthias Schneider; Jacob M van Laar; Ana Vieira; Maurizio Cutolo; Marta Mosca; Marco Matucci-Cerinic Journal: RMD Open Date: 2018-10-18
Authors: Marina Maria Vieira de Figueiredo Caldas; Francisco Alves Bezerra Neto; Kesley Pablo Morais de Azevedo; Isac Davidson Santiago Fernandes Pimenta; Ana Katherine Da Silveira Gonçalves De Oliveira; Grasiela Piuvezam Journal: Medicine (Baltimore) Date: 2019-09 Impact factor: 1.817