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

Systemic scleroderma-related interstitial pneumonia associated with borderline pulmonary arterial hypertension.
 

Author(s): Keishi Sugino, Takayuki Kabuki, Kazutoshi Shibuya, Sakae Homma

Journal:

 

A 65-year-old woman with a 35-year history of limited cutaneous systemic scleroderma was admitted to our hospital complaining of a 3-month history of progressive dyspnoea on exertion. High-resolution CT images of the chest revealed diffuse reticular opacities and traction bronchiectasis ...

Last Updated: 31 Dec 1969

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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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Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study.
 

Author(s): Sébastien Peytrignet, Christopher P Denton, Mark Lunt, Roger Hesselstrand, Luc Mouthon, Alan Silman, Xiaoyan Pan, Edith Brown, László Czirják, Jörg H W Distler, Oliver Distler, Kim Fligelstone, William J Gregory, Rachel Ochiel, Madelon Vonk, Codrina Ancuta, Voon H Ong, Dominique Farge, Marie Hudson, Marco Matucci-Cerinic, Alexandra Balbir-Gurman, Øyvind Midtvedt, Alison C Jordan, Wendy Stevens, Pia Moinzadeh, Frances C Hall, Christian Agard, Marina E Anderson, Elisabeth Diot, Rajan Madhok, Mohammed Akil, Maya H Buch, Lorinda Chung, Nemanja Damjanov, Harsha Gunawardena, Peter Lanyon, Yasmeen Ahmad, Kuntal Chakravarty, Søren Jacobsen, Alexander J MacGregor, Neil McHugh, Ulf Müller-Ladner, Gabriela Riemekasten, Michael Becker, Janet Roddy, Patricia E Carreira, Anne Laure Fauchais, Eric Hachulla, Jennifer Hamilton, Murat Inanç, John S McLaren, Jacob M van Laar, Sanjay Pathare, Susanna Proudman, Anna Rudin, Joanne Sahhar, Brigitte Coppere, Christine Serratrice, Tom Sheeran, Douglas J Veale, Claire Grange, Georges-Selim Trad, Ariane L Herrick

Journal: Rheumatology (Oxford). 2018 02;57(2):370-381.

 

Our aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features.

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 14 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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Primary myocardial disease in scleroderma-a comprehensive review of the literature to inform the UK Systemic Sclerosis Study Group cardiac working group.
 

Author(s): Lesley-Anne Bissell, Md Yuzaiful Md Yusof, Maya H Buch

Journal: Rheumatology (Oxford). 2017 06;56(6):882-895.

 

Cardiac disease is prevalent in SSc and associated with a poor prognosis. Differentiating primary myocardial disease (SSc-cardiomyopathy) from ischaemic heart disease is difficult and the disease phenotype most at risk is unclear. A comprehensive literature review was performed to ...

Last Updated: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

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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Scleroderma Treatment With Autologous Transplant (STAT) Study
 

Status: Recruiting

Condition Summary: Systemic Scleroderma

 

Last Updated: 27 Jul 2018

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