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Scleroderma

A chronic disorder marked by hardening and thickening of the skin.

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

About Scleroderma

Derived from the Greek words "sklerosis," meaning hardness, and "derma," meaning skin, scleroderma literally means "hard skin." Although it is often referred to as if it were a single disease, scleroderma is really a symptom of a group of diseases that involve the abnormal growth of connective tissue, which supports the skin and internal organs. It is sometimes used, therefore, as an umbrella term for these disorders. In some forms of scleroderma, hard, tight skin is the extent of this abnormal process. In other forms, however, the problem goes much deeper, affecting blood vessels and internal organs, such as the heart, lungs, and kidneys.

Scleroderma is called both a rheumatic (roo-MA-tik) disease and a connective tissue disease. The term rheumatic disease refers to a group of conditions characterized by inflammation or pain in the muscles, joints, or fibrous tissue. A connective tissue disease is one that affects tissues such as skin, tendons, and cartilage....Read more about Scleroderma NIH - National Institute of Arthritis and Musculoskeletal and Skin Diseases

What works? Research summarized

Evidence reviews

Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies

The review evaluated the effectiveness of the prophylactic use of cyclophosphamide for pulmonary function in patients with scleroderma and interstitial lung disease. It found limited evidence for an improvement in forced vital capacity, but not for diffusing lung capacity for carbon monoxide. The review had no major flaws, but the authors considered the result not to be clinically significant.

Analysis of the validation status of quality of life and functional disability measures in pulmonary arterial hypertension related to systemic sclerosis: results of a systematic literature analysis by the expert panel on outcomes measures in pulmonary arterial hypertension related to systemic sclerosis (EPOSS)

OBJECTIVE: We aimed to assess the current validity status of the Health Assessment Questionnaire-Disability Index (HAQ-DI) and the 36-item Medical Outcomes Study Short Form Health Survey (SF-36).

Atherosclerosis in systemic sclerosis: a systematic review and meta-analysis

OBJECTIVE: Systemic sclerosis (SSc) is characterized by calcification, vasculopathy, and endothelial wall damage, all of which can increase the risk of developing atherosclerosis and cardiovascular disease. The aim of this study was to perform a systematic review and meta-analysis to determine whether the risk of atherosclerosis is increased in SSc patients compared to healthy individuals.

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Summaries for consumers

Bone Marrow or Blood Stem Cell Transplants in Children With Severe Forms of Autoimmune Disorders or Certain Types of Cancer: A Review of the Research for Parents and Caregivers

This summary will cover: Information about each autoimmune disorder and type of cancer Possible benefits of an HSCT What researchers have found about children with a very severe form of one of these diseases who received an HSCT that used their own stem cells What an HSCT is and how it is done Possible risks of an HSCT

Prazosin for Raynaud's phenomenon in progressive systemic sclerosis

Scleroderma is a connective tissue disease causing fibrosis and commonly affects the skin and internal organs such as the GI tract, lungs, kidney and heart. Most people with scleroderma also have raynaud's phenomenon (RP). RP is defined as vasospasm of arteries or arterioles causing pallor and at least one other colour change upon reperfusion such as cyanosis or redness. Primary RP occurs in the absence of causes such as connective tissue disease. Secondary RP occurs in people with underlying diseases that affect blood vessels especially scleroderma and lupus. The RP that occurs in scleroderma is often more severe in that there is not only vasospasm but also a fixed blood vessel deficit with intimal proliferation and therefore narrowing of the blood vessels. Raynaud's phenomenon may also be accompanied by digital ulcers which are possibly secondary to ischemia.

Iloprost and cisaprost for Raynaud's phenomenon in progressive systemic sclerosis

Scleroderma is a connective tissue disease causing fibrosis and commonly affects the skin and internal organs such as the GI tract, lungs, kidney and heart.

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More about Scleroderma

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Other terms to know:
Connective Tissue, Inflammation, Skin

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