Diffuse scleroderma

Common Name(s)

Diffuse scleroderma, Diffuse systemic sclerosis

Diffuse scleroderma is a type of scleroderma characterized as a systemic (internal) chronic connective tissue disease that is visible through the hardening of the skin (please also see: Scleroderma).

Scleroderma can be diffuse or limited. In diffuse scleroderma, skin thickening and hardening occurs more rapidly and involves more skin areas than in limited disease. Skin of the arms, legs, and trunk are more likely to be involved. The tightened skin makes it difficult to bend fingers, hands, and other joints. There is sometimes inflammation of the joints, tendons and muscles. Tight skin on the face can reduce the size of a person’s mouth and increases the importance of good dental care. Skin may gain or lose pigment (color) creating light and dark patches. In addition, people with diffuse scleroderma have a higher risk of developing “sclerosis” or fibrous hardening of the internal organs such as the heart, lungs and kidneys. The amount of organ involvement is highly variable; some individuals have no organ involvement while others may be severely affected. Scleroderma can develop in every age group from infants to the elderly, but its onset is most frequent between the ages of 25 to 55 and affects females more frequently than males.

The diagnostic process may require consultation with rheumatologists (arthritis specialists), and/or dermatologists (skin specialists) and require blood studies and numerous other specialized tests. Specific organ and skin treatments are available. Treatments range from anti-inflammatory medications, immunosuppressive therapy, drug therapy of vascular disease, and anti-fibrotic agents. If you or a loved one has been diagnosed with diffuse scleroderma, talk to your doctor about the most current treatment options. Support groups are also a good source of information and support.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Diffuse scleroderma" for support, advocacy or research.

International Scleroderma Network

Our mission is to provide stellar worldwide research, support, education and awareness for scleroderma and related illnesses.

Last Updated: 18 Apr 2010

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Raynaud's & Scleroderma Ireland

• To improve the lives of patients, their families and carers by providing support, advice and information. • To increase the awareness of Raynaud’s and Scleroderma amongst healthcare professionals, Government and Statutory agencies and the general public. • To work in partnership with appropriate healthcare professionals and agencies to promote and co-ordinate the attainment of the objectives of the strategy. • Maintain a working environment that fosters communication, teamwork and diversity among all members of staff- permanent, temporary and voluntary

Last Updated: 6 May 2014

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General Support Organizations

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Diffuse scleroderma" for support, advocacy or research.

International Scleroderma Network

Our mission is to provide stellar worldwide research, support, education and awareness for scleroderma and related illnesses.

http://www.sclero.org/

Last Updated: 18 Apr 2010

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Raynaud's & Scleroderma Ireland

• To improve the lives of patients, their families and carers by providing support, advice and information. • To increase the awareness of Raynaud’s and Scleroderma amongst healthcare professionals, Government and Statutory agencies and the general public. • To work in partnership with appropriate healthcare professionals and agencies to promote and co-ordinate the attainment of the objectives of the strategy. • Maintain a working environment that fosters communication, teamwork and diversity among all members of staff- permanent, temporary and voluntary

http://www.irishraynauds.com

Last Updated: 6 May 2014

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Diffuse scleroderma" returned 20 free, full-text research articles on human participants. First 3 results:

Patterns and predictors of change in outcome measures in clinical trials in scleroderma: an individual patient meta-analysis of 629 subjects with diffuse cutaneous systemic sclerosis.
 

Author(s): P A Merkel, N P Silliman, P J Clements, C P Denton, D E Furst, M D Mayes, J E Pope, R P Polisson, J B Streisand, J R Seibold,

Journal: Arthritis Rheum.. 2012 Oct;64(10):3420-9.

 

To examine the range and responsiveness to change of clinical outcome measures and study predictors of clinical response in patients with diffuse cutaneous systemic sclerosis (dcSSc) in the context of clinical trials.

Last Updated: 1 Oct 2012

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Skin thickness progression rate: a predictor of mortality and early internal organ involvement in diffuse scleroderma.
 

Author(s): Robyn T Domsic, Tatiana Rodriguez-Reyna, Mary Lucas, Noreen Fertig, Thomas A Medsger

Journal: Ann. Rheum. Dis.. 2011 Jan;70(1):104-9.

 

To examine the association of skin thickness progression rate (STPR) with mortality, and as a predictor of future internal organ involvement in an inception cohort of diffuse cutaneous systemic sclerosis (SSc) patients.

Last Updated: 17 Dec 2010

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Stimulation with type I collagen induces changes in gene expression in peripheral blood mononuclear cells from patients with diffuse cutaneous systemic sclerosis (scleroderma).
 

Author(s): S P Atamas, I G Luzina, J Ingels, J Choi, W K Wong, D E Furst, P J Clements, , A E Postlethwaite

Journal: Clin. Exp. Immunol.. 2010 Sep;161(3):426-35.

 

An autoantigenic role for collagen type I (CI) has been suggested previously in diffuse cutaneous systemic sclerosis (dcSSc). Whether CI is indeed capable of affecting the immune system in dcSSc is not known. Patients with early (3 years or less) or late (>3 years) dcSSc and healthy ...

Last Updated: 5 Nov 2010

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Diffuse scleroderma" returned 0 free, full-text review articles on human participants.

 
 
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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

IVIG Treatment in Systemic Sclerosis
 

Status: Recruiting

Condition Summary: Systemic Sclerosis; Diffuse Scleroderma

 

Last Updated: 28 Jan 2015

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IL1-TRAP, Rilonacept, in Systemic Sclerosis
 

Status: Recruiting

Condition Summary: Scleroderma; Systemic Sclerosis; Diffuse Scleroderma; Diffuse Systemic Sclerosis

 

Last Updated: 26 Jan 2015

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Multi-center, Web Based Observational Study of Pulmonary Hypertension in Scleroderma Patients
 

Status: Recruiting

Condition Summary: Systemic Sclerosis; Scleroderma; Pulmonary Hypertension; Pulmonary Arterial Hypertension

 

Last Updated: 9 Mar 2015

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