| What
is Scleroderma? |
| This page
was written by Shelley
Ensz, and has not yet been medically edited. See Disclaimer. |
|
| What is Scleroderma? |
 Scleroderma is
a rare, chronic autoimmune disease which afflicts an estimated
150,000 to 500,000 Americans, primarily females who are 30
to 50 years old at onset. The overall occurrence is 30 people
per 100,000 and the ratio of women to men is about four to
one. |
| 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. |
| Scleroderma: A Rare but Devastating Disease. The skin thickens and tightens, becoming deformed and unyielding. Then, the skin around the body's joints allows for less and less bending. Hands, elbows and knees all become stiff. "Scleroderma is not less frequent than multiple sclerosis, but MS gets 10 times more funding." HealthDay. 07/27/07. |
| Scleroderma in a Population-based Sample: Evidence Suggests Increasing Incidence. In this large population-based sample, there was evidence of increasing incidence for generalized scleroderma over the interval 1994 to 2003. Part of the observed trend in incidence may reflect an increasing recognition of the disease in the population by physicians. S. Bernatsky. 1193/425. ACR 2007. (Also see: Difficult Diagnosis) |
| Rheumatic Diseases: The Unnoticed Elephant in the Room. The group of conditions known as rheumatic diseases, or musculoskeletal (MSK) disorders, is indeed an elephant, given the magnitude of their impact on the population. Ediitorial. J of Rheumatology. January 2008. |
| Brochures About Scleroderma |
| These brochures are in PDF format.
The files will open automatically in Adobe Reader, which is
a free program which is already installed on most computers.
If you click on the file link and it doesn't open, download
Adobe Reader here. |
 What
in the world is Scleroderma? (PDF Brochure) Scleroderma
(sklare-oh-derma) means "hard skin." This complex
disease involves tightening and thickening of the skin, blood
vessel damage, inflammation and immune system changes. Brochure
includes a systemic scleroderma symptom checklist! International
Scleroderma Network. |
| (Español/Spanish)
¿Que es Escleroderma? (PDF) Escleroderma o Esclerodermia
significa ‘piel dura.’ La forma sistemica de esta complicada
enfermedad involucra la rigidez y el endurecimiento de la
piel, daño a los vasos sanguineos, inflamacion y cambios
en el sistema inmunologico. International Scleroderma
Network. |
 Japanese:
What is Scleroderma? Brochure. This PDF brochure was
translated by Yoko, medically edited by Dr.
Shinichi Sato, and desktop published by Susanne
Piché. Posted 10-03-05. |
| Books About Scleroderma |
 We
strongly recommend the ISN's
Voices of Scleroderma book series for top quality medical
and support information. It receives rave reviews from patients,
caregivers and doctors alike! Articles by scleroderma experts
are combined with true stories from patients and caregivers
sharing their journey in coping with all types of scleroderma
symptoms.
|
| Types
of Scleroderma |
| There are two main types of scleroderma,
localized and systemic. |
| 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. |
See Types
of Scleroderma for further explanation. |
| Difficult
Diagnosis |
| Proper diagnosis of scleroderma
is often long and difficult, since it is a rare disease which
few doctors are well-versed in, and in the early stages it
may resemble many other connective tissue diseases, such as
systemic lupus erythematosus, polymyositis, and rheumatoid
arthritis, just to name a few. |
| Often the diagnostic uncertainty
and frustration takes a huge toll on the psychological well-being
of these patients, who describe their journey to diagnosis
as being by far the most difficult part of their illness.(4) See Difficult
Diagnosis. |
| Effects of Systemic
Scleroderma |
| The systemic forms of scleroderma
cause fibrosis (scar tissue) to be formed in the skin and/or
internal organs. The fibrosis eventually causes the involved
skin or organs to harden, which is why scleroderma is popularly
known as the "disease that turns people into stone." |
| Exercise Performance In Systemic Sclerosis: Relationship With Disease Manifestations. Exercise performance is impaired in most SSc patients, at least in those admitted in a referral clinic. The impairment of exercise performance is correlated to lung, heart and skin involvement. L. Ruocco AB0525 EULAR 2007 (Also see: Cardiac, Pulmonary, and Skin Involvement) |
| Causes
of Scleroderma |
| The cause of scleroderma is unknown.
Some cases of scleroderma have been linked to chemical exposures.
Genetics, fetal cells, and viruses might also be factors in
the development of scleroderma. See Causes
of Scleroderma. |
| There's
no cure, but scleroderma can be managed. We don't know what causes scleroderma.
Researchers
suspect that there is a genetic link. The theory is that while a specific
genetic trait predisposes the individual, scleroderma will only occur after
exposure to some environmental agent, such as a particular virus. Post-Bulletin.
Mayo Clinic. 06/04/07. (Also see: Causes of Scleroderma: Genetics, and Infections) |
| Scleroderma is Not
Contagious or Cancerous |
| 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. |
| Scleroderma
Symptoms and Treatments |
| 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. |
| Systemic Scleroderma Symptom
Checklist in our free PDF from the ISN's "What
is Scleroderma?" brochures. |
| Systemic
Scleroderma Symptoms include: |
|
|
| Scleroderma
Treatments and Clinical Trials |
| Treatment of complications associated with systemic sclerosis. Systemic sclerosis is characterized by various circulatory, dermatological, gastrointestinal, musculoskeletal, pulmonary, and renal complications. Although there is no cure for systemic sclerosis, management of its associated complications can help improve patients' quality of life. (PubMed) Am J Health Syst Pharm. 2008 Feb 15; 65(4):315-21. |
| Mechanisms
and consequences of fibrosis in systemic sclerosis. Overall,
this is an exciting time for new therapies in SSc and advances
are being made in synchrony with an improved understanding
of the molecular and biochemical basis of the disease. PubMed.
Nat Clin Pract Rheumatol. 2006 Mar;2(3):134-44. (Also see: Skin
Fibrosis) |
| Scleroderma
Clinical Trials |
| There are some experimental therapies
which show promise for treatment of scleroderma, including
Stem Cell Transfusion (BMT). See Clinical
Trials. |
| Information about
Scleroderma |
| Scleroderma
(Systemic Sclerosis). American College of Rheumatology. |
| Overview
of Scleroderma MayoClinic. |
| Living With Scleroderma. Understanding Your Disease. This Scleroderma Educational Program was developed by Dr. Jennifer Haythornthwaite with help from Dr. Wigley of the Johns Hopkins Scleroderma Center to help patients learn about Scleroderma and understand more about the physiological and psychological effects of the disease. John Hopkins Medicine, Scleroderma Center. |
| 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. |
| Scleroderma Webcast #1 with Dr. Art Theodore, BUMC |
 Scleroderma Webcast #1 Replay and Scleroderma Webcast Transcript (PDF). Dr. Art Theodore is a nationally recognized expert with special interests in systemic sclerosis and interstitial lung disease from Boston University Medical Campus. 10-30-07. PatientPower. |
| Scleroderma Webcast #2 with Dr. Art Theodore, BUMC |
| Scleroderma Webcast #2 Replay. Dr. Art Theodore is a nationally recognized expert with special interests in systemic sclerosis and interstitial lung disease from Boston University Medical Campus. 04-25-08. PatientPower. (Also see: Pulmonary Involvement) |
| 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). |
| What
is Scleroderma? Arthritis Foundation (U.S.). |
| What
is Scleroderma? MedicineNet. |
| What
is Scleroderma? "Scleroderma can be difficult to
diagnose. There may be slowly evolving skin changes or malfunction
of an organ. First symptoms may include..." Scleroderma
Society of Canada. |
| Scleroderma Patient
Stories |
| We feature the world's largest
collection of scleroderma patient and caregiver stories, in
many languages. The stories from this site form the basis for
the ISN's Voices
of Scleroderma book series. |
| Systemic Scleroderma: |
| CREST
Stories |
| Diffuse
Stories |
| Limited
SD Stories |
| Overlap
Patient Stories |
| |
| Localized Scleroderma: |
| Linear
Stories |
| Morphea
Stories |
| Morphea
Stories (Italiano) |
| |
| Others |
| English
Stories by Type (Main listing) |
| English
Stories Alphabetically by First Name |
| Stories
by Date Posted |
| Stories
in Other Languages |
| Difficult
Diagnosis and Undiagnosed |
| Eosinophilic
Fasciitis |
| Interstitial
Cystitis |
| Sjögren's
Syndrome |
| References |
| (1) Audio
pronunciations for this page are from Mirriam-Webster
Online Dictionary. |
