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Systemic Sclerosis (Scleroderma) and Raynaud’s Phenomenon

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A Clinician's Pearls and Myths in Rheumatology

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Abstract

Systemic sclerosis (scleroderma; SSc) is a chronic con nective tissue disease characterized by inflammation within, and fibrosis of, the skin, vascular abnormalities, visceral damage, and the production of autoantibodies. SSc is divided generally into limited and diffuse forms, based on the extent of skin involvement. A group of conditions known as “localized” sclero-derma, which includes morphea, linear scleroderma, and en coup de sabre, is discussed in the chapter on Scleroderma Mimickers (Chap. 11). Diffuse SSc has a greater extent of skin involvement and is more likely to be associated with renal crisis, pulmonary fibrosis, and cardiomyopathy. Patients with diffuse SSc have a higher mortality rate. Pulmonary disease remains a major source of morbidity and mortality for patients with SSc. Patients with limited SSc are predisposed to the development of pulmonary arterial hypertension (PAH). Those with diffuse SSc are predisposed to interstitial fibrosis. Autoantibody profiles can predict to a certain extent the types of organ involvement that can be expected in SSc. Raynaud's phenomenon (RP) is characterized by vasospasm of the digital arteries that leads to well-demarcated pallor with cyanosis and rubor. RP is essentially universal among patients with SSc and is the first symptom in a large majority of patients. Primary RP is often mild enough not to require treatment; however, with secondary RP from SSc, there is not only vasospasm but also fixed blood vessel problems with luminal narrowing, so the ischemia can be more severe. Complications of RP in SSc can include digital ulcers often leading to infection and amputation causing pain and functional disability. Diffuse SSc often presents with puffiness of the hands associated with pain, in a way that can mimic rheumatoid arthritis. RP often occurs around the time of development of puffy hands in diffuse SSc.

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Authors and Affiliations

  1. St. Joseph Health Care, London 268 Grosvenor St., London, Ontario, N6A 4V2, Canada

    Janet E. Pope

  2. UCLA Medical School, 1000 Veterans Avenue Rehab Center, Box 951670, Los Angeles, California, 90095-1670, USA

    Philip J. Clements, Daniel E. Furst & Dinesh Khanna

  3. Johns Hopkins Scleroderma Center, Johns Hopkins University, 5501 Hopkins Bayview Circle, Baltimore, MD, 21224, USA

    Laura K. Hummers

  4. University of Texas-Houston Health Science, Center/Rheumatology 6431 Fannin MSB 5.270, Houston, Texas, 77030, USA

    Maureen D. Mayes

  5. University of Pittsburgh Medicine/Rheumatology, 3500 Terrace Street 7S BST, Pittsburgh, Pennsylvania, 15261

    Thomas Medsger

  6. Division of Rheumatology, University of Michigan Medical Center, Ann Arbor, Michigan, USA

    James Seibold

  7. Department of Rheumatology, Georgetown University Medical Center, 3800 Reservoir Road, Washington, D.C., 20007, USA

    Virginia Steen

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  1. Janet E. Pope
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  1. Clinical Rheumatology, Massachusetts General Hospital, 55 Fruit Street / Yawkey 2, Boston, MA, 02114

    John H. Stone (Director) (Director)

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Pope, J.E. et al. (2009). Systemic Sclerosis (Scleroderma) and Raynaud’s Phenomenon. In: Stone, J.H. (eds) A Clinician's Pearls and Myths in Rheumatology. Springer, London. https://doi.org/10.1007/978-1-84800-934-9_10

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