|What is Scleroderma?
Types of Scleroderma
Scleroderma is a rare, chronic rheumatic disease. The combined forms of scleroderma, including localized, systemic, and related conditions, affect an estimated 300,000 Americans, primarily females who are 30 to 50 years old at onset. The overall occurrence is 1 per 1,000 (.1% of the 310 million U.S. population) and the ratio of women to men is about four to one.
For the subtype of systemic sclerosis, which is the most serious form of the disease, there are about 75,000 current cases in the United States.
About 20 to 24 new cases per million population are diagnosed each year in the U.S. Worldwide, there may be as many as 2.5 million persons with scleroderma.
Scleroderma is both a rheumatic disease and a connective tissue disease. The term rheumatic disease refers to a group of conditions characterized by inflammation and/or pain in the muscles, joints, or fibrous tissue. A connective tissue disease is one that affects tissues such as skin, tendons, and cartilage. NIAMS.
What Is Scleroderma? What Causes Scleroderma? Scleroderma refers to a group of rare chronic autoimmune diseases in which the skin and connective tissues tighten and harden; it is a progressive disease. Scleroderma occurs when the body produces and accumulates too much collagen in tissues. Experts do not know what triggers this. Medical News Today. 01/18/10.
Scleroderma clinical features and treatment. Systemic scleroderma may involve all connective tissue containing organs; however, some represent the main target of the disease. These include the skin, gastrointestinal tract, heart, lungs, and kidneys. Dr. Izharul Hasan, Cardiologist. ArticlesBase. 07/2010.
The systemic forms can affect any part of the body (skin, blood vessels, and internal organs.) The systemic forms are also referred to as "systemic sclerosis" plus other terms such as diffuse, limited, CREST, and overlap.
The localized forms are morphea and linear. They affect only the skin (and sometimes the underlying tissues) but do not affect the internal organs, or reduce one's life expectancy in any way. When any form of scleroderma (either localized or systemic) occurs in children, it is also called Childhood Scleroderma or Juvenile Scleroderma.
Two Main Types: Localized and Systemic
|Localized (Morphea, Linear, En Coup)
Systemic Sclerosis (Limited/CREST, Diffuse, Overlap)
Brochures About Scleroderma. Free brochures in PDF format, to print yourself. Or, order printed copies. Includes valuable systemic scleroderma symptom checklist! ISN.
We strongly recommend the ISN's Voices of Scleroderma book series for top quality medical and support information. Each volume includes articles by world scleroderma experts along with 100 patients, caregiver and survivor stories for all types of scleroderma, who share how they are coping with all types of scleroderma symptoms. Centerpiece medical articles are by the late Dr. Joe Korn, Prof. Carol Black, and Dr. Marco Matucci-Cerinic. (Also see Voices of Scleroderma Book Series and Order ISN Books)
Diagnosis of Scleroderma is usually achieved through your medical history and a thorough physical exam. Blood tests are performed to look for specific antibodies. A skin biopsy may be performed as well as organ specific tests to check for systemic involvment. ISN.
Preliminary analysis of the Very Early Diagnosis of Systemic Sclerosis (SSc) (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers (PuFi) as a pivotal sign for suspicion of systemic sclerosis. PuFi is an important sign raising suspicion for underlying very early SSc in patients with RP. Ann Rheum Dis, 08/12/2013.
Systemic sclerosis without antinuclear antibodies (ANA) or Raynaud's phenomenon (RP): a multicentre study in the prospective EULAR Scleroderma Trials and Research (EUSTAR) database. Prospective studies are needed to elucidate the clinical presentation, evolution and outcome of such patients. PubMed, Rheumatology, 2013 Mar;52(3):560-7. (Also see Antibodies in Systemic Scleroderma, Raynaud's)
Difficult Diagnosis. Proper diagnosis of scleroderma is often long and difficult. Often the diagnostic uncertainty and frustration takes a huge psychological toll on these patients, who describe their journey to diagnosis as being by far the most difficult part of their illness. ISN.
Causes of Scleroderma. The cause of scleroderma is unknown. Some cases of scleroderma have been linked to chemical exposures. Genetics, fetal cells, gluten sensitivity, and viruses might also be factors in the development of scleroderma, and it may be due to a combination of factors. ISN.
Scleroderma Experts are few and far between, because scleroderma is such a rare disease. Use our own ISN Guide to Scleroderma Experts, plus our compilation of other listings, to find the best expert for you! ISN.
Scleroderma is not classified as contagious, which means that you cannot get scleroderma by shaking hands, hugging, kissing, sexual contact, contact with blood or bodily fluids, sharing eating utensils, or by airborne contact from coughing or sneezing. And it is not cancerous.
Systemic Scleroderma Symptoms. Systemic sclerosis (scleroderma) can affect every part of the body, including the skin, blood vessels, and all of the internal organs. There are also dozens of associated conditions and related autoimmune diseases. ISN.
Scleroderma Treatments and Clinical Trials. At present, there are no proven treatments or cure for any forms of scleroderma. However, there are effective therapies for many of the symptoms. Most of the symptoms listed in this website are those of systemic scleroderma. ISN.
History of Scleroderma. Great resource for school reports, includes information about Paul Klee, the abstract painter who is perhaps the most famous person to have had scleroderma. ISN.
Late-onset Systemic Sclerosis (SSc). These findings suggest that older patients with SSc are at greater risk for pulmonary hypertension, renal impairment, cardiac disease, and muscle weakness. Rebecca L. Manno. The Journal of Rheumatology. June 17, 2011.
Overview of Scleroderma MayoClinic.
Voices of Scleroderma Volume 1 Book offers medical information about scleroderma by leading experts. Includes scleroderma symptom checklist, plus 100 personal stories of people living with scleroderma and related illnesses. ISN.
What is Scleroderma? "Imagine for me if you would that in certain areas of your body the skin has begun to thicken and harden. Then on top of this, you have a painful sensitivity to cold, frequent heartburn, stiff joints and various other internal problems." Amy (Daughter of a Systemic Sclerosis patient).
Scleroderma Patient and Caregiver Stories. We feature the world's largest collection of scleroderma patient and caregiver stories, in many languages. The stories from this site also formed the basis for the ISN's Voices of Scleroderma book series. ISN.
(1) Audio pronunciations for this page are from Mirriam-Webster Online Dictionary.
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