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Diagnosis and Management of Systemic Sclerosis: A Practical Approach

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Abstract

Systemic sclerosis is a devastating multisystem rheumatologic condition that is characterized by autoimmunity, tissue fibrosis, obliterative vasculopathy and inflammation. Clinical presentation and course of the condition vary greatly, which complicates both diagnosis and corresponding treatment. In this regard, recent advances in disease understanding, both clinically and biochemically, have led to newer classification criteria for systemic sclerosis that are more inclusive than ever before. Still, significant disease modifying therapies do not yet exist for most patients. Therefore, organ-based management strategies are employed and research has been directed within this paradigm focusing on either the most debilitating symptoms, such as Raynaud’s phenomenon, digital ulcers and cutaneous sclerosis, or life-threatening organ involvement such as interstitial lung disease and pulmonary arterial hypertension. The current trends in systemic sclerosis diagnosis, evidence-based treatment recommendations and potential future directions in systemic sclerosis treatment are discussed.

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Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, Schulich School of Medicine, Western University, London, ON, Canada

    Jason J. Lee & Janet E. Pope

  2. Division of Rheumatology, St. Joseph’s Health Care, 268 Grosvenor St., London, ON, N6A 4V2, Canada

    Janet E. Pope

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  1. Jason J. Lee
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Correspondence to Janet E. Pope.

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J. J. Lee and J. E. Pope declare no conflict of interest, either financial or non-financial. There was no funding for this review.

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Lee, J.J., Pope, J.E. Diagnosis and Management of Systemic Sclerosis: A Practical Approach. Drugs 76, 203–213 (2016). https://doi.org/10.1007/s40265-015-0491-x

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