Systemic Scleroderma

Common Name(s)

Systemic Scleroderma

Systemic scleroderma is an autoimmune disorder that affects the skin and internal organs. It is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs. The fibrosis is caused by the body's production of too much collagen, which normally strengthens and supports connective tissues. The signs and symptoms of systemic scleroderma usually begin with episodes of Raynaud's phenomenon, which can occur weeks to years before fibrosis. This may be followed by puffy or swollen hands before the skin becomes thickened and hard. Fibrosis can also affect internal organs and can lead to impairment or failure of the affected organs. The most commonly affected organs are the esophagus, heart, lungs, and kidneys.

There are three types of systemic scleroderma, defined by the tissues affected in the disorder.

Limited scleroderma  Diffuse scleroderma  Sine scleroderma -
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Systemic Scleroderma" for support, advocacy or research.

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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 "Systemic Scleroderma" for support, advocacy or research.

Logo
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 "Systemic Scleroderma" returned 114 free, full-text research articles on human participants. First 3 results:

Oesophageal stenosis dilatation through retrograde trans-gastrostomal approach in a patient with systemic scleroderma.
 

Author(s): D Serban, C Brănescu, M Comandaşu, A M Dascălu, D Dumitrescu, H Bumbea, B Braticievici, C Savlovschi

Journal: Chirurgia (Bucur). ;109(3):396-401.

 

The paper presents the surgical solving of an oesophageal stenosis, using a device of pneumatic dilatation with trans-gastrostomal approach, in a patient with multiple disabling handicaps, secondary severe malnutrition and previously diagnosed with scleroderma.

Last Updated: 24 Jun 2014

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Systemic scleroderma associated with ulnar nerve entrapment at the elbow.
 

Author(s): Kaouther Ben Abdelghani, Sarra Esseghir, Leila Souabni, Salwa Belhadj, Leith Zakraoui

Journal: Tunis Med. 2013 Nov;91(11):676-7.

 

Last Updated: 17 Dec 2013

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[Peripheral neuropathy associated with cerebrovascular disease revealing systemic scleroderma].
 

Author(s): Kaouther Ben Abdelghani, Khaoula Ben Abdelghani, Leith Zakraoui, Adel Kheder

Journal: Tunis Med. 2013 Nov;91(11):675-6.

 

Last Updated: 17 Dec 2013

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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 "Systemic Scleroderma" returned 10 free, full-text review articles on human participants. First 3 results:

Pediatric scleroderma: systemic or localized forms.
 

Author(s): Kathryn S Torok

Journal: Pediatr. Clin. North Am.. 2012 Apr;59(2):381-405.

 

Pediatric scleroderma includes 2 major groups of clinical entities, systemic sclerosis (SSc) and localized scleroderma (LS). Although both share a common pathophysiology, their clinical manifestations differ. LS is typically confined to the skin and underlying subcutis, with up to ...

Last Updated: 7 May 2012

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Pathogenesis and therapeutic approaches for improved topical treatment in localized scleroderma and systemic sclerosis.
 

Author(s): I Badea, M Taylor, A Rosenberg, M Foldvari

Journal: Rheumatology (Oxford). 2009 Mar;48(3):213-21.

 

SSc is a chronic progressive disorder of unknown aetiology characterized by excess synthesis and deposition of collagen and other extracellular matrix components in a variety of tissues and organs. Localized scleroderma (LS) differs from SSc in that with LS only skin and occasionally ...

Last Updated: 19 Feb 2009

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Coexistence of systemic sclerosis, scleroderma-like syndromes and neoplastic diseases.
 

Author(s): Mariusz Ciołkiewicz, Izabela Domysławska, Agata Ciołkiewicz, Piotr Adrian Klimiuk, Anna Kuryliszyn-Moskal

Journal: Pol. Arch. Med. Wewn.. 2008 Mar;118(3):119-26.

 

Coexistence of rheumatic and neoplastic diseases may take different forms. Rheumatic paraneoplastic syndromes, including systemic sclerosis, scleroderma-like changes and Raynaud's phenomenon are induced by substances secreted by neoplastic cells and immunological disturbances connected ...

Last Updated: 14 May 2008

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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

IL1-TRAP, Rilonacept, in Systemic Sclerosis
 

Status: Recruiting

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

 

Last Updated: 15 Jul 2014

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Allogeneic Hematopoietic Cell Transplantation After Nonmyeloablative Conditioning for Patients With Severe Systemic Sclerosis
 

Status: Recruiting

Condition Summary: Systemic Scleroderma; Severe Systemic Sclerosis

 

Last Updated: 17 Mar 2014

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Effect of Sildenafil on the Microcirculatory Blood Flow and Endothelial Progenitor Cells in Systemic Sclerosis
 

Status: Recruiting

Condition Summary: Scleroderma, Systemic; Scleroderma, Diffuse; Scleroderma, Limited; Raynaud Phenomenon

 

Last Updated: 3 May 2011

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