National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Limited systemic sclerosis


Other Names:
Systemic sclerosis sine scleroderma; Progressive systemic sclerosis sine scleroderma; Scleroderma, sine
Categories:
This disease is grouped under:
Limited systemic sclerosis (also known as sine scleroderma) is a type of systemic scleroderma that is characterized by Raynaud's phenomenon and the buildup of scar tissue (fibrosis) on one or more internal organs but not the skin.[1][2][3] While the exact cause of limited systemic sclerosis is unknown, it is believed to originate from an autoimmune reaction which leads to the overproduction of collagen (a tough protein which normally strengthens and supports connective tissues throughout the body).[2]

When fibrosis affects internal organs, it can lead to impairment or failure of the affected organs. The most commonly affected organs are the esophagus, heart, lungs, and kidneys. Internal organ involvement may be signaled by heartburn, difficulty swallowing (dysphagia), high blood pressure (hypertension), kidney problems, shortness of breath, diarrhea, or impairment of the muscle contractions that move food through the digestive tract (intestinal pseudo-obstruction).[1][2] While there is no treatment that controls or stops the underlying problem (the overproduction of collagen), many of the symptoms of limited systemic sclerosis can be managed. Many people require a team of specialists to address their symptoms.[4]
Last updated: 3/28/2017

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 Limited 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.
  • The Scleroderma Clinical Trials Consortium is an international organization of scleroderma clinical researchers. The consortium Web site contains a listing of active scleroderma trials, past copies of the Scleroderma Care and Research journal, and a tool for finding your nearest member institution. 

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

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.

Where to Start

  • Genetics Home Reference (GHR) contains information on Limited systemic sclerosis. This website is maintained by the National Library of Medicine.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) support research into the causes, treatment, and prevention of arthritis and musculoskeletal and skin diseases, the training of basic and clinical scientists to carry out this research, and the dissemination of information on research progress in these diseases. Click on the link to view information on this topic.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • 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 Limited systemic sclerosis. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

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. Submit a new question

  • I have systemic sclerosis with lung problems. I am taking oxygen. Is there any other treatment for this? See answer


  1. Systemic scleroderma. Genetics Home Reference (GHR). 2015; http://ghr.nlm.nih.gov/condition/systemic-scleroderma.
  2. Hachulla E. Limited systemic sclerosis. Orphanet. July 2010; http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=18907.
  3. Kucharz EJ, Kopec-Medrek M.. Systemic sclerosis sine scleroderma.. Adv Clin Exp Med. 2017 Aug; 26(5):875-880. http://www.advances.umed.wroc.pl/pdf/2017/26/5/875.pdf.
  4. Handout on Health: Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). August 2016; http://www.niams.nih.gov/Health_Info/Scleroderma/default.asp.