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  3. Diffuse cutaneous systemic sclerosis
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Diffuse cutaneous systemic sclerosis


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Other Names:
Diffuse cutaneous systemic sclerosis; DcSSc; Progressive cutaneous systemic scleroderma; Diffuse cutaneous systemic sclerosis; DcSSc; Progressive cutaneous systemic scleroderma; Progressive cutaneous systemic sclerosis; Diffuse cutaneous systemic scleroderma See More
Categories:
Heart Diseases; Kidney and Urinary Diseases; Lung Diseases; Heart Diseases; Kidney and Urinary Diseases; Lung Diseases; Skin Diseases See More
This disease is grouped under:
Systemic scleroderma

Summary Summary


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Diffuse cutaneous systemic sclerosis (dcSSc) is a subtype of systemic scleroderma (systemic sclerosis) characterized by skin hardening (fibrosis) and problems in many organs of the body. The disease can occur at any age but mainly affects people between 40 and 50 years of age. Symptoms include Raynaud’s phenomenon; skin fibrosis beginning on the fingers and face that rapidly becomes generalized; "spider veins" (telangiectasias) on the thorax, face, lips, tongue, and fingers; gastroesophageal reflux; and difficulty eating (dysphagia) along with weight loss, vomiting, diarrhea or constipation. Dry mouth and dental involvement can occur. Joint pain (arthralgias), muscular pain, weakness, cramps, and destruction of the tips of the fingers or toes (acroosteolysis) are frequent. Severe problems involving the lung (fibrosis or pulmonary hypertension) and kidney problems may also occur.[1][2] The exact cause of the condition is unknown. There is currently no cure. Treatment depends of the symptoms, but may include medication and surgery.[1]
Last updated: 1/30/2017

Symptoms Symptoms


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This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 25 |
Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Autoimmunity
Autoimmune disease
Autoimmune disorder
[ more ]
0002960
Dyspnea
Trouble breathing
0002094
Gastroesophageal reflux
Acid reflux
Acid reflux disease
Heartburn
[ more ]
0002020
Narrow foramen obturatorium 0100958
Oliguria 0100520
Pulmonary infiltrates
Lung infiltrates
0002113
30%-79% of people have these symptoms
Arthralgia
Joint pain
0002829
Arthritis
Joint inflammation
0001369
Carious teeth
Dental cavities
Tooth cavities
Tooth decay
[ more ]
0000670
Dyspareunia 0030016
Dysphagia
Poor swallowing
Swallowing difficulties
Swallowing difficulty
[ more ]
0002015
Flexion contracture
Flexed joint that cannot be straightened
0001371
Malabsorption
Intestinal malabsorption
0002024
Muscle weakness
Muscular weakness
0001324
Osteolysis
Breakdown of bone
0002797
Pulmonary fibrosis 0002206
Skin ulcer
Open skin sore
0200042
Telangiectasia of the skin 0100585
Xerostomia
Dry mouth
Dry mouth syndrome
Reduced salivation
[ more ]
0000217
5%-29% of people have these symptoms
Abnormal bowel sounds 0030142
Congestive heart failure
Cardiac failure
Cardiac failures
Heart failure
[ more ]
0001635
Hypertensive crisis 0100735
Nausea and vomiting 0002017
Pulmonary arterial hypertension
Increased blood pressure in blood vessels of lungs
0002092
Renal insufficiency
Renal failure
Renal failure in adulthood
[ more ]
0000083
Showing of 25 |
Do you have more information about symptoms of this disease? We want to hear from you.
Last updated: 2/1/2021
Do you have updated information on this disease? We want to hear from you.

Related Diseases Related Diseases


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Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnoses include Sharp syndrome, systemic lupus erythematosus, antiphospholipid syndrome, polyarteritis nodosa, polymyositis, and rheumatoid arthritis (see these terms).
Visit the Orphanet disease page for more information.

Research Research


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Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Diffuse cutaneous systemic sclerosis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations


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Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

  • International Scleroderma Network (ISN)
    7455 France Ave So #266
    Edina, MN 55435-4702
    Toll-free: 1-800-564-7099
    Telephone: +1-952-831-3091
    E-mail: isn@sclero.org
    Website: https://www.sclero.org/index.html
  • Scleroderma & Raynaud's UK (SRUK)
    18-20 Bride Lane
    London, EC4Y 8EE United Kingdom
    Toll-free: 0800 311 2756 (Helpline)
    Telephone: 020 7000 1925 (Office)
    E-mail: info@sruk.co.uk
    Website: https://www.sruk.co.uk
  • Scleroderma Society of Ontario
    41 King William Street, Suite 202
    Hamilton, ON, L8R 1A2 Canada
    Toll-free: 1-888-776-7776 (Helpline)
    Telephone: +1-905-544-0343
    E-mail: info@sclerodermaontario.ca
    Website: https://www.sclerodermaontario.ca/
Do you know of an organization? We want to hear from you.

Learn More Learn More


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These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Diffuse cutaneous systemic sclerosis. Click on the link to view a sample search on this topic.

News & Events News & Events


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News

  • Rare Disease Day at NIH 2021
    March 1, 2021

GARD Answers GARD Answers


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Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References


  1. Varga J. Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults. UpToDate. June 03, 2016; http://www.uptodate.com/contents/overview-of-the-clinical-manifestations-of-systemic-sclerosis-scleroderma-in-adults.
  2. Diffuse cutaneous systemic sclerosis. Orphanet. July 2010. http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=18905.
Do you know of a review article? We want to hear from you.
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