Scleroderma overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] M. Khurram Afzal, MD [2]

Overview

Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen in the skin or other organs. The localized type of the disease, while disabling, tends not to be fatal. Diffuse scleroderma or systemic sclerosis, the generalized type of the disease, can be fatal as a result of heart, kidney, lung or intestinal damage.[1]

Historical Perspective

The word scleroderma comes from greek words; skleros (hard) and derma (skin). Scleroderma was first described by Carlo Curzio in Naples, Italy in 1753. The association between abnormal vasoconstriction and diffuse scleroderma was made in 1865 by Raynaud.

Classification

Pathophysiology

Causes

The cause of scleroderma has not been identified. There is a possibility of an underlying immunologic abnormality. To review risk factors for the development of scleroderma click here.

Differentiating Scleroderma from other Diseases

Epidemiology and Demographics

The majority of cases of scleroderma have been reported from the United States. The prevalence of scleroderma is approximately 24 cases per 100,000 individuals in the United States. Scleroderma commonly affects individuals between 20 to 50 years of age. Choctaw native Americans have a much higher prevalence of scleroderma than the general population. Females are more commonly affected than males. Familial clustering of scleroderma has been reported in United States and Australia.

Risk Factors

Common risk factors in the development of scleroderma include occupational and environmental exposure to certain chemicals, certain genetic variations and infectious agents. Most commonly implicated occupational and environmental risk factors are exposure is to silica, chlorinated and aromatic solvents as well as welding fumes.

Natural History, Complications and Prognosis

If left untreated, patients with scleroderma may progress to develop pulmonary arterial hypertension (PAH), interstitial lung disease and severe gastrointestinal disease. Common complications of scleroderma include pulmonary fibrosis, pulmonary arterial hypertension, interstitial lung disease and scleroderma renal crisis. The 10-year survival rate of patients with scleroderma is approximately 70%-80%.

Diagnosis

History and Symptoms

Physical Examination

Electrocardiogram

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Treatment

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Prevention

References

  1. Klippel J (ed). Systemic sclerosis and related syndromes. Primer on the rheumatic diseases, 11th edition. The Arthritis Society. 1997;269. ISBN 1-91242-316-2.


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