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

Clinical and serological features of systemic sclerosis in a multicenter African American cohort: Analysis of the genome research in African American scleroderma patients clinical database.
 

Author(s): Nadia D Morgan, Ami A Shah, Maureen D Mayes, Robyn T Domsic, Thomas A Medsger, Virginia D Steen, John Varga, Mary Carns, Paula S Ramos, Richard M Silver, Elena Schiopu, Dinesh Khanna, Vivien Hsu, Jessica K Gordon, Heather Gladue, Lesley A Saketkoo, Lindsey A Criswell, Chris T Derk, Marcin A Trojanowski, Victoria K Shanmugam, Lorinda Chung, Antonia Valenzuela, Reem Jan, Avram Goldberg, Elaine F Remmers, Daniel L Kastner, Fredrick M Wigley, Pravitt Gourh, Francesco Boin

Journal: Medicine (Baltimore). 2017 Dec;96(51):e8980.

 

Racial differences exist in the severity of systemic sclerosis (SSc). To enhance our knowledge about SSc in African Americans, we established a comprehensive clinical database from the largest multicenter cohort of African American SSc patients assembled to date (the Genome Research ...

Last Updated: 31 Dec 1969

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Pharmacokinetic optimitzation of CCG-203971: Novel inhibitors of the Rho/MRTF/SRF transcriptional pathway as potential antifibrotic therapeutics for systemic scleroderma.
 

Author(s): Kim M Hutchings, Erika M Lisabeth, Walajapet Rajeswaran, Michael W Wilson, Roderick J Sorenson, Phillip L Campbell, Jeffrey H Ruth, Asif Amin, Pei-Suen Tsou, Jeffrey R Leipprandt, Samuel R Olson, Bo Wen, Ting Zhao, Duxin Sun, Dinesh Khanna, David A Fox, Richard R Neubig, Scott D Larsen

Journal: Bioorg. Med. Chem. Lett.. 2017 04;27(8):1744-1749.

 

We recently reported the development of a novel inhibitor of Rho-mediated gene transcription (1, CCG-203971) that is efficacious in multiple animal models of acute fibrosis, including scleroderma, when given intraperitoneally. The modest in vivo potency and poor pharmacokinetics (PK) ...

Last Updated: 31 Dec 1969

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Epithelial Fli1 deficiency drives systemic autoimmunity and fibrosis: Possible roles in scleroderma.
 

Author(s): Takehiro Takahashi, Yoshihide Asano, Koji Sugawara, Takashi Yamashita, Kouki Nakamura, Ryosuke Saigusa, Yohei Ichimura, Tetsuo Toyama, Takashi Taniguchi, Kaname Akamata, Shinji Noda, Ayumi Yoshizaki, Daisuke Tsuruta, Maria Trojanowska, Shinichi Sato

Journal: J. Exp. Med.. 2017 Apr;214(4):1129-1151.

 

Systemic sclerosis (SSc), or scleroderma, is a multisystem autoimmune disorder characterized by vasculopathy and fibrosis in the skin and internal organs, most frequently in the esophagus and lungs. Hitherto, studies on SSc pathogenesis centered on immune cells, vascular cells, and ...

Last Updated: 31 Dec 1969

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

Systemic sclerosis sine scleroderma.
 

Author(s): Eugeniusz J Kucharz, Magdalena Kopeć-Mędrek

Journal: Adv Clin Exp Med. 2017 Aug;26(5):875-880.

 

Systemic sclerosis is a rare generalized disease with scleroderma, i.e. skin thickening as one of the most common symptoms. The disease has 2 main subsets, diffuse and limited forms. The subset known as systemic sclerosis sine scleroderma (ssSSc) is a very rare subset characterized ...

Last Updated: 31 Dec 1969

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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: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Scleroderma Treatment With Autologous Transplant (STAT) Study
 

Status: Recruiting

Condition Summary: Systemic Scleroderma

 

Last Updated: 8 Jan 2018

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Evaluation of the Serum Soluble Fractalkine as a Biomarker of Pulmonary Fibrosis in Systemic Sclerosis
 

Status: Not yet recruiting

Condition Summary: Systemic Scleroderma

 

Last Updated: 16 Apr 2018

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