Scleroderma - What is Scleroderma?

Scleroderma is a chronic autoimmune disease characterized by fibrosis (or hardening), vascular alterations, and autoantibodies. 

The cause of Scleroderma is unknown. Scleroderma runs in families, but the genes have not been identified. It affects the small blood vessels known as arterioles, in all organs

There are two major forms of Scleroderma:

Limited systemic sclerosis/scleroderma's cutaneous manifestations mainly affect the hands, arms and face. Previously called CREST syndrome in reference to the following complications: Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasias.

Additionally, pulmonary arterial hypertension may occur in up to one third of patients and is the most serious complication for this form of scleroderma.

Diffuse systemic sclerosis/scleroderma is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart and lungs. This form of scleroderma can be quite disabling.

There are no treatments for scleroderma itself, but individual organ system complications are treated.

Other forms of scleroderma include Systemic sine scleroderma, which lacks skin changes, but has systemic manifestations, and two localized forms which affect the skin, but not the internal organs: morphea, and linear scleroderma.

Prognosis is generally good for limited cutaneous scleroderma patients that escape pulmonary complications.

Prognosis is worse for diffuse cutaneous disease, particularly in older age, and for males. Death occurs most often from pulmonary, heart and kidney complications. In diffuse cutaneous disease, 5-year survival is 70%, 10-year survival 55%.

First, the endothelial cells of the arteriole die off, along with smooth muscle cells, by a process of apoptosis. They are replaced by collagen and other fibrous material.

Inflammatory cells, particularly CD4+ helper T cells, infiltrate the arteriole, and cause further damage. Many of the inflammatory and destructive protein signals have been identified, and they are potential targets for drugs that could interrupt the process.

  • Localized scleroderma
    • Localized morphea
    • Morphea-lichen sclerosus et atrophicus overlap
    • Generalized morphea
    • Atrophoderma of Pasini and Pierini
    • Pansclerotic morphea
    • Morphea profunda
    • Linear scleroderma
    • Systemic scleroderma
      • CREST syndrome
      • Progressive systemic sclerosis

This disease is found among all races worldwide, but women are four times more likely to develop scleroderma than men. In the United States, approximately one person in 1,000 is affected.

Children rarely suffer the systemic type, but localized scleroderma is common. Most adults are diagnosed after their 30th birthday and before age 50.

The disease has high rates among the native American Choctaw tribe and African-American females.

Further Reading


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