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Evidence-based management of systemic sclerosis: Navigating recommendations and guidelines

Russell Edward Pellar¹, Janet Elizabeth Pope²

¹Department of Medicine, University of Western Ontario, London, ON, Canada.

Seminars in Arthritis and Rheumatism

|January 16, 2017

Insights

Updated guidelines for Systemic sclerosis (SSc) management offer best practices for this rare connective tissue disease. Further research is needed to address care gaps and improve patient outcomes.

Area of Science:

Rheumatology
Internal Medicine
Connective Tissue Diseases

Background:

Systemic sclerosis (SSc) is a rare, heterogeneous connective tissue disease.
There is a need for updated recommendations on managing SSc and its organ complications.

Purpose of the Study:

To compare and contrast updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and British Society of Rheumatology/British Health Professionals in Rheumatology (BSR/BHPR) guidelines for SSc management.

Main Methods:

Comparative analysis of updated EULAR/EUSTAR and BSR/BHPR guidelines for Systemic sclerosis (SSc).

Main Results:

Updated EULAR/EUSTAR guidelines focus on SSc features and newer therapies, with limited non-pharmacologic recommendations.
BSR/BHPR guidelines cover similar organ manifestations and expand on general measures, non-pharmacologic treatment, and specific complications.
Guidelines generally agree, but limitations include lack of guidance on combination/second-line therapy, algorithmic suggestions, and treatment for specific complications like gastric antral ectasia and erectile dysfunction. Consensus on treating interstitial lung disease is lacking, and regional differences in expert recommendations exist.

Conclusions:

The EULAR/EUSTAR and BSR/BHPR guidelines can help address care gaps and promote best practices in Systemic sclerosis (SSc) management.
Further investigation into certain complications is warranted to improve SSc outcomes and inform future guideline updates.

Read full analysis

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.

Keywords:

Best practices British Society of Rheumatology European Scleroderma Research Group Evidence based Guidelines ILD Management PAH RP Recommendations Scleroderma Systemic sclerosis Treatment

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