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

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: 24 Feb 2017

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Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS).
 

Author(s): Ariane L Herrick, Xiaoyan Pan, Sébastien Peytrignet, Mark Lunt, Roger Hesselstrand, Luc Mouthon, Alan Silman, Edith Brown, László Czirják, Jörg H W Distler, Oliver Distler, Kim Fligelstone, William J Gregory, Rachel Ochiel, Madelon Vonk, Codrina Ancuţa, Voon H Ong, Dominique Farge, Marie Hudson, Marco Matucci-Cerinic, Alexandra Balbir-Gurman, Øyvind Midtvedt, Alison C Jordan, Paresh Jobanputra, 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 İnanç, John S McLaren, Jacob M van Laar, Sanjay Pathare, Susannah Proudman, Anna Rudin, Joanne Sahhar, Brigitte Coppere, Christine Serratrice, Tom Sheeran, Douglas J Veale, Claire Grange, Georges-Selim Trad, Christopher P Denton

Journal: Ann. Rheum. Dis.. 2017 Jul;76(7):1207-1218.

 

The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. We aimed to use an observational approach to compare effectiveness of currently used treatment approaches.

Last Updated: 11 Feb 2017

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Orofacial Manifestations and Temporomandibular Disorders of Systemic Scleroderma: An Observational Study.
 

Author(s): Vito Crincoli, Laura Fatone, Margherita Fanelli, Rossana Patricia Rotolo, Angela Chialà, Gianfranco Favia, Giovanni Lapadula

Journal:

 

Scleroderma is a disorder involving oral and facial tissues, with skin hardening, thin lips, deep wrinkles, xerostomia, tongue rigidity, and microstomia. The aim of this study was to investigate the prevalence of oral manifestations and temporomandibular disorders (TMD) in Systemic ...

Last Updated: 26 Jul 2016

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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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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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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: 18 Jan 2017

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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: 7 Mar 2017

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