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Scleroderma
See also in: Nail and Distal Digit
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Scleroderma

See also in: Nail and Distal Digit
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Contributors: Jeffrey M. Cohen MD, Vivian Wong MD, PhD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

This summary discusses scleroderma in adults. Scleroderma in children is addressed separately.

Scleroderma, or systemic sclerosis, is an autoimmune connective tissue disease that involves sclerotic changes of the skin and internal organs. While the etiology remains unknown, the disease is characterized by autoantibody production, collagen deposition, and vascular dysfunction. The disease is observed in all ages and is slightly more common in individuals of African descent and 3-4 times more common in women. The age of onset is usually between 30 and 50 years.

Scleroderma can affect the connective tissue of any organ, including the skin, gastrointestinal tract, lungs, kidneys, joints, muscles, heart, and blood vessels. Pulmonary disease is the leading cause of mortality. Additional common clinical features include esophageal fibrosis and dysmotility, arthralgias, and Raynaud phenomenon. Less common manifestations include hypertensive renal crisis, pulmonary hypertension and interstitial lung disease, and cardiomyopathy.

There are three major clinical subsets of scleroderma:

1) Limited cutaneous systemic sclerosis (distal skin sclerosis, Raynaud phenomenon, frequent severe late-stage complications such as pulmonary hypertension and gastrointestinal involvement). CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) refers to a subset of patients with limited scleroderma.

2) Diffuse cutaneous systemic sclerosis (proximal extremity or trunk skin sclerosis, Raynaud phenomenon of shorter duration, high risk of renal crisis, and cardiac and lung fibrosis).

3) Systemic sclerosis sine scleroderma (Raynaud phenomenon and systemic involvement without skin sclerosis).

The subsets are defined by the degree of skin involvement, so the classification does not predict systemic organ disease. The systemic sclerosis overlap syndrome is characterized by features of one of the scleroderma subsets with those of another autoimmune disease.

Patients of African descent tend to have an earlier onset (35-44 years compared with 45-55 years for all others), a more severe course, and increased mortality. This may be due to their being more likely to have diffuse, rather than limited, cutaneous systemic scleroderma. In addition, lung function is often worse. There is a greater prevalence of anti-topoisomerase I antibodies, anti-RNP, and anti-Ro antibodies in these patients, although the frequency of anticentromere antibodies is less compared with individuals of Northern European descent.

There are reports in the literature of drug-induced scleroderma. The majority of reports point to a scleroderma-like disease rather than true systemic scleroderma. See drug-induced sclerodermoid reactions for further details.

Codes

ICD10CM:
M34.9 – Systemic sclerosis, unspecified

SNOMEDCT:
89155008 – Scleroderma

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Last Reviewed: 11/03/2017
Last Updated: 09/14/2017
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Scleroderma
See also in: Nail and Distal Digit
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Scleroderma (General Manifestations) : Fatigue, Creatinine elevated, Insomnia, Joint stiffness, Muscle weakness, Proteinuria, Sclerodactyly, Arthralgia, Dysphagia, Hardened skin
Clinical image of Scleroderma
Bound-down skin over the digits with scars and surrounding erythema over the knuckles.
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