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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. 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).
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). 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.
Source: GARD Last updated on 05-01-20
Systemic sclerosis affects not only the skin, but can also affect the tissues beneath, including the blood vessels and major organs. When the lungs are involved, systemic sclerosis often causes interstitial lung disease and/or pulmonary arterial hypertension. Additional lung complications and conditions can also occur in association with systemic sclerosis.
Interstitial lung disease refers to scarring in the lungs which can make the lungs stiff and make it hard to breathe. Pulmonary arterial hypertension is high blood pressure in the blood vessels in the lungs. This high blood pressure can put strain on the heart.
Options for pulmonary arterial hypertension treatment aims to reduce the strain on your heart. Treatment options include medications, such as endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostacyclins, anticoagulants , calcium channel blockers,diuretics , and inhaled oxygen. Some people with pulmonary arterial hypertension undergo surgery, such as lung transplantation.
You can read this an much more at the following link to the American Lung Association: http://www.lung.org/lung-disease/pulmonary-arterial- hypertension/symptoms-diagnosis.html
Options for interstitial lung disease treatment aims to make breathing more productive, reduce inflammation, and suppress overactive immune systems. Treatment options include medications, such as Mycophenolate (CellCept®) or Cyclophosphamide (Cytoxan®). For more information on these treatments visit the National Jewish Health Web site at http://www.nationaljewish.org/healthinfo/medications/interstitial-lung- disease. Additional treatment options include oxygen therapy, pulmonary rehabilitation programs (e.g., breathing exercises), and lung transplantation.
The following Web pages and articles gives further information on treatment:
Institute of Arthritis and Musculoskeletal and Skin Disorders)
http://www.srfcure.org/for-patients/current-treatments (Scleroderma Reserach Foundation)
http://www.mayoclinic.org/diseases- conditions/scleroderma/basics/treatment/con-20021378 (MayoClinic)
If you have not done so already, you may find it helpful to contact the following organization for further information and support:
Scleroderma Research Foundation
220 Montgomery Street, Suite 1411
San Francisco, CA 94104
Toll-free: 800-441-CURE (2873)
Research is underway to improve treatment options for people with systemic sclerosis. In particular, The Scleroderma Clinical Trials Consortium (SCTC) is a charitable non-profit organization dedicated to finding better treatment for scleroderma. Member institutions of the SCTC conduct clinical treatment trials of new (and sometimes old) medications that appear promising for the treatment of scleroderma. CLICK HERE to read about currently- enrolling clinical trials. You can also view a listing of institutions in the United States involved in clinical research of scleroderma.
Last updated on 05-01-20
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