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 152 free, full-text research articles on human participants. First 3 results:

Anti-neutrophil cytoplasmic antibody negative crescentic paucimmune glomerulonephritis in a case of scleroderma with systemic lupus erythematosus overlap.
 

Author(s): Rohit Tewari, Sonia Badwal, Arun Kumar, Shankar Subramaniam, V S Nijhawan, V Srinivas

Journal: Saudi J Kidney Dis Transpl. 2016 May;27(3):602-5.

 

Renal Involvement in scleroderma is a known problem and the manifestations are well described. Renal involvement in systemic lupus erythematosus (SLE) is also well known. However, in scleroderma and SLE overlap syndrome, the renal findings may vary being a combination of features ...

Last Updated: 24 May 2016

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[RACAND syndrome: distinct entity or initial presentation of systemic scleroderma].
 

Author(s): Monia Smiti Khanfir, Syrine Bellakhal, Fatma Said, Thouraya Ben Salem, Imed Ben Ghorbel, Mounir Lamloum, Amira Hamzaoui, Mohamed Habib Houman

Journal: Tunis Med. 2015 Jun;93(6):392.

 

Last Updated: 8 Dec 2015

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Digital Ulcers, Systemic Sclerosis Sine Scleroderma and Paraneoplastic Phenomena Responding to Bosentan Therapy.
 

Author(s): Itzhak Sharabi, Amir Tanay, Gisele Zandman-Goddard

Journal: Isr. Med. Assoc. J.. 2015 Feb;17(2):126-7.

 

Last Updated: 30 Jul 2015

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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 13 free, full-text review articles on human participants. First 3 results:

Consensus best practice pathway of the UK Scleroderma Study Group: digital vasculopathy in systemic sclerosis.
 

Author(s): Michael Hughes, Voon H Ong, Marina E Anderson, Frances Hall, Pia Moinzadeh, Bridget Griffiths, Eileen Baildam, Christopher P Denton, Ariane L Herrick

Journal: Rheumatology (Oxford). 2015 Nov;54(11):2015-24.

 

Digital vasculopathy (comprising RP, digital ulceration and critical digital ischaemia) is responsible for much of the pain and disability experienced by patients with SSc. However, there is a limited evidence base to guide clinicians in the management of SSc-related digital vasculopathy. ...

Last Updated: 14 Oct 2015

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Systemic sclerosis (scleroderma) and cancer risk: systematic review and meta-analysis of observational studies.
 

Author(s): Martina Bonifazi, Irene Tramacere, Giovanni Pomponio, Barbara Gabrielli, Enrico V Avvedimento, Carlo La Vecchia, Eva Negri, Armando Gabrielli

Journal: Rheumatology (Oxford). 2013 Jan;52(1):143-54.

 

A higher incidence of cancer in scleroderma patients compared with the general population has been suggested by several observational studies, reporting, however, different estimates. Therefore, we aimed to perform a systematic review and meta-analysis to definitely assess this association.

Last Updated: 14 Dec 2012

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

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

Treatment of Refractory Sever Systemic Scleroderma by Injection of Allogeneic Mesenchymal Stem Cells
 

Status: Recruiting

Condition Summary: SYSTEMIC SCLERODERMA; ALLOGENEIC MESENCHYMAL STEM CELLS; ADULT

 

Last Updated: 15 Apr 2016

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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: 10 Mar 2016

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Oral Ifetroban to Treat Diffuse Cutaneous Systemic Sclerosis (SSc) or SSc-associated Pulmonary Arterial Hypertension
 

Status: Not yet recruiting

Condition Summary: Scleroderma, Diffuse; Scleroderma, Systemic; Scleroderma, Limited; Sclerosis, Progressive Systemic; Skin Diseases; Connective Tissue Diseases; Pathologic Processes; Autoimmune Diseases

 

Last Updated: 29 Jul 2016

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