The contemporary management of systemic sclerosis

ABSTRACT

Introduction: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by vascular dysfunction, fibrosis, inflammation and autoantibodies. The pathophysiology of SSc is not completely understood, and many patients acquire organ or tissue damage despite advances in treatment. Current treatments target affected organs with modest improvements.

Areas covered: This review evaluates several treatment strategies for SSc based on involved organs including skin, pulmonary, cardiac, renal, musculoskeletal, and gastrointestinal. Currently, pulmonary hypertension and interstitial lung disease are the primary causes of increased mortality. We will outline an approach to treatment of SSc based on disease manifestations and current evidence.

Expert commentary: This complex disease is currently treated with therapies developed for similar indications such as for vascular manifestations of SSc using idiopathic pulmonary arterial hypertension treatments. Future directions in this field may include combination and maintenance therapy that is currently used in other autoimmune diseases, and tailoring these treatments according to the patients’ phenotype. This will hopefully increase the efficacy of available treatments and decrease mortality from SSc.

KEYWORDS:

• Autoimmunity
• Connective tissue disease
• Scleroderma
• Systemic sclerosis
• Treatment

Declaration of interest

J Pope has been a consultant for AbbVie, Amgen, BMS, Celtrion, GSK, Janssen, Lilly, Merck, Novartis, Pfizer, Roche, Sandoz, Sanofi, UCB The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper is not funded.