| Types
of Scleroderma: Overview |
| This page was written
by Shelley Ensz and
has not yet been medically edited. See disclaimer. |
|
| Two Main Types |
| There are many methods used to
categorize the various forms of scleroderma, which has brought
about a confusing abundance of terms. The following categories
are often found in patient literature, but use and interpretation
of these categories varies quite a bit. |
There are two main types of
scleroderma: Localized and Systemic. |
| Classification criteria of scleroderma. Those patients with systemic scleroderma involving the trunk are classified as "Cutaneous diffuse systemic scleroderma"; the association of Raynaud's phenomenon, capillaroscopic abnormalities and specific autoantibodies defines "limited systemic scleroderma"; among the latter patients, those with distal skin involvement are classified "Cutaneous limited systemic sclerosis". The old term "CREST" tends to be abandoned due to its lack of specificity. PubMed. Presse Med. 2006 Dec;35(12 Pt 2):1916-22. |
| Classification
in Systemic Sclerosis. Accurate classification of systemic
sclerosis (SSc) has been an evolving issue in both pediatric
and adult rheumatology literature. The need for classification
criteria has been long recognized as a necessity for scientific
inquiry, and prognosis is dependent on disease severity and
target organ involvement. J Rheumatol 2006 May;33:840
Editorial. (Also see: Juvenile
Scleroderma) |
| Systemic
sclerosis - diagnosis and classification. Early diagnosis
and classification may be difficult if disease expression
is oligosymptomatic (undifferentiated), presenting with only
Raynaud's phenomenon or limited scleroderma. PubMed. Z
Rheumatol. 2006 Jun 28. (Also see: Difficult
Diagnosis) |
| Studies
of scleroderma at The Alfred Hospital, Melbourne. All
types have a high incidence of autoantibodies, but these
are generally not related to the severity of the disease
and do not occur in relatives or spouses. PubMed. Intern
Med J. 2006 Aug;36(8):513-8. (Also see: Antibodies) |
| 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. |
| Books About Scleroderma |
|
But
what is scleroderma really like?! To answer that, we
strongly recommend the ISN's
Voices of Scleroderma book series for top quality medical
and support information.
This book series receives rave reviews from patients,
caregivers and doctors alike! Articles by highly esteemed
scleroderma experts are combined with true stories from patients
and caregivers explaining their illness and sharing their
ideas for coping with all types of scleroderma symptoms. |
|
| Juvenile Scleroderma |
| When
scleroderma of any type (either localized or systemic) afflicts
children, it is called Juvenile
Scleroderma. The localized forms of scleroderma (such as Linear and Morphea) are
most common in children. |
| Systemic Sclerosis and Localized Scleroderma in Childhood. Juvenile scleroderma syndromes, including the systemic and the localized varieties, represent the third most frequent chronic rheumatic conditions in pediatric rheumatology practice. (ScienceDirect) Rheumatic Disease Clinics of North America Vol 34, Issue 1, Feb 2008, Pp 239-255. (Also see: Juvenile Scleroderma and Localized) |
| Unilateral
generalized morphea (UGM) is a rare variant of localized
scleroderma. As the onset of UGM usually occurs in pediatric
patients, pediatricians should be cognizant of the presentation
of this uncommon condition. PubMed. Eur J Med Res. 2006
Apr 28;11(4):152-6. (Also see: Localized Scleroderma: Morphea and Linear) |
| Juvenile
localized scleroderma: clinical and epidemiological features
in 750 children. An international study. The insidious
onset of the disease, the delay in diagnosis, the recognition
of mixed subtype and the better definition of the other subtypes
should influence our efforts in educating trainees and practitioners
and help in developing a comprehensive classification system
for this syndrome. PubMed. Rheumatology (Oxford). 2005
Dec 20. |
| Clinical
Characteristics of Juvenile Systemic Sclerosis in Japanese. Our
study suggests that Japanese patients with juvenile SSc have
more severe skin sclerosis than adults with SSc, although
the frequency of internal organ involvement and the mortality
rate is lower than with adult SSc. J Rheumatol 2005 September;32:1850.
(Also see: Skin
Fibrosis) |
| Juvenile
Systemic Sclerosis: A Follow-up Study of Eight Patients. Our
results suggest that in Juvenile Systemic Sclerosis (jSSc),
Raynaud's phenomenon is more severe, whereas internal organ
manifestations and the frequency of autoantibodies are far
less pronounced than in adult-onset SSc. Also, the survival
rate and final outcome of patients with jSSc appear to be
better than those in patients with adult-onset SSc. PubMed.
Ann N Y Acad Sci. 2005 Jun;1051:229-34. |
| A
case of mandibuloacral dysplasia presenting with features
of scleroderma. In Mandibuloacral dysplasia should be
considered in the differential diagnosis of juvenile scleroderma
in the presence of atypical features such as negative serological
studies, absence of Raynaud's phenomenon, sparse hair and
micrognathia. PubMed. Int J Clin Pract. 2004 Jun;58(6):635-8.
(Also see: Diseases
Similar to Scleroderma) |
| Localized |
| There are two types of Localized
Scleroderma: Linear and Morphea. Localized
scleroderma affects the skin. It may also affect the underlying
muscles and bones, but it does not affect internal organs. |
| In general, Localized Scleroderma
is relatively mild, and may be related to Systemic Scleroderma
only in terms of similar superficial symptoms, such as the
appearance of skin biopsy under the microscope. |
| Collagen Degradation Products And Inflammatory Activity In Systemic (SSc) And Localized Scleroderma (LSc). In patients with SSc our data have shown the most intensive collagen degradation and simultanously an active inflammation which reflects the pathological processes in the skin and visceral organs, compared with psoriasis vulgaris patients and healthy inviduals. In LSc group collagen degradation was similar to that in control groups but a certain inflammatory activity was observed. R. Becvar THU0242 EULAR 2007. (Also see: Systemic Scleroderma and Skin Involvement) |
| Localized
scleroderma in childhood is not just a skin disease. Extracutaneous
manifestations of juvenile localized scleroderma developed
in almost one-fourth of the children in this study. These
extracutaneous manifestations often were unrelated to the
site of the skin lesions and sometimes were associated with
multiple organ involvement. The risk of developing SSc was
very low. PubMed. Arthritis Rheum. 2005 Sep 2;52(9):2873-2881. |
| Localized
scleroderma is an autoimmune disorder. Many previous
studies conclude that localized scleroderma involves autoimmune
abnormalities and is one of the organ-specific autoimmune
disorders targeting mainly skin, although the types of autoimmune
abnormality are different from systemic sclerosis. PubMed.
Rheumatology (Oxford). 2004 Nov 23. (Also see: Morphea, Linear,
and Dr. Shinichi
Sato) |
| Muscle
Cramps Associated with Localized Scleroderma Skin Lesions:
Focal Dystonia, Neuromyotonia, or Nerve Entrapment? Based
on the temporal and spatial correlation of skin lesions and
muscle cramps in our patients, we propose that localized
scleroderma may precipitate muscle cramps, possibly caused
by local nerve injury. J Rheumatol 2006 December;33:2549.
Letter. |
| Chronic
venous insufficiency (CVI) - a potential trigger for localized
scleroderma. It may be that CVI is a potential trigger
factor for LS, which may only develop if a certain amount
of trigger factors are present and resolves if one or more
of the contributing factors (such as CVI) can be treated. PubMed.
J Eur Acad Dermatol Venereol. 2006 Jan;20(1):96-9. |
| Antinucleosome
antibody is a major autoantibody in localized scleroderma. Although
antinucleosome antibody was not specific to localized scleroderma,
its high prevalence in localized scleroderma indicates that
antinucleosome antibody is a major autoantibody in this disease. PubMed.
Br J Dermatol. 2004 Dec;151(6):1182-8. (Also see: Antibodies) |
| Novel
Autoantibody to Cu/Zn Superoxide Dismutase in Patients with
Localized Scleroderma. IgG or IgM anti-Cu/Zn SOD antibody
was detected in the serum of 89% of localized scleroderma
patients, especially 100% of patients with generalized morphea. Minoru
Hasegawa. 1687/510. ACR 2004. (Also see: Generalized
Morphea and Antibodies) |
| Localized
Scleroderma There is a wide spectrum of localized scleroderma,
with types ranging from mild to disabling. eMedicine Dermatology. |
| Nodular Systemic Scleroderma |
| Nodular
Scleroderma: Case Report and Literature Review. Nodular
Systemic Sclerosis is a rare variant that presents with lesions
that clinically resemble keloids. Most patients had symptoms
of systemic sclerosis. J Rheumatol. Volume 30: No. 11
November 2003;30:2500-2. (Also see: Diseases
Similar to Scleroderma) |
| Systemic Sclerosis
(Systemic Scleroderma) |
| There are several types of Systemic
Scleroderma: CREST, Limited and Diffuse. Systemic
scleroderma is also known as systemic sclerosis (SSc). It may
also be referred to as Progressive Systemic Sclerosis (PSSc),
or Familial Progressive Systemic Sclerosis (FPSSc). |
| Systemic scleroderma may affect
the skin, blood vessels, and/or internal organs. When it
affects the skin, it can cause the skin —most commonly on
the hands and/or face —to harden. With the blood vessels,
it can cause Raynaud's.
When it affects the internal organs, it may cause disability
or even death. (Also see Systemic
Scleroderma Symptoms) |
| The Cutoff Points of Antinuclear Antibody (ANA) with High Negative and Positive Predictive Values. Analysis of 5655 Cases. The cutoff point of ANA titer 160 is appropriate to exclude SLE (Lupus), MCTD (Mixed Connective Tissue Disease), SSc (Systemic Scleroderma) in most of the clinical setting. ANA 640 or higher deserves further investigation such as disease specific autoantibodies even without characteristic clinical findings, especially after proper exams for chronic liver and thyroid diseases and RA (Rheumatoid Arthritis). Hisanori Shimizu. 1515/129. ACR 2007. (Also see: Antibodies, Lupus, MCTD, and RA) |
| Risk Factors For Mortality In Patients With Systemic Sclerosis (SSc) And Interstitial Lung Disease (ILD). Mortality in patients with SSc and ILD is increased in those with an early and severe impairment of pulmonary function, concomitant cardiac involvement and elevated ESR (Sed rate), but not in Scl-70 positive patients. Beatriz E. Joven. 6/6. ACR 2007. (Also see: Pulmonary Fibrosis) |
| Scleroderma patients nailfold videocapillaroscopic patterns are associated with disease subset and disease severity. Nailfold videocapillaroscopy, a simple, non-invasive and non-expensive investigation, is useful in staging scleroderma patients and also provides prognostic information. Rheumatology 2007 46(10):1566-1569. (Also see: Nailfold Capillaroscopy) |
| Collagen Degradation Products And Inflammatory Activity In Systemic (SSc) And Localized Scleroderma (LSc). In patients with SSc our data have shown the most intensive collagen degradation and simultanously an active inflammation which reflects the pathological processes in the skin and visceral organs, compared with psoriasis vulgaris patients and healthy inviduals. In LSc group collagen degradation was similar to that in control groups but a certain inflammatory activity was observed. R. Becvar THU0242 EULAR 2007. (Also see: Localized Scleroderma and Skin Involvement) |
| Update on pathophysiology of scleroderma with special reference to immunoinflammatory events. Scleroderma or systemic sclerosis (SSc) is a complex disease in which the vasculopathy and the activation of the immune system with production of inflammatory mediators lead to dysregulated fibroblast activation. The resulting excessive deposition of collagens and other extracellular matrix proteins ends in fibrosis and organ dysfunction. The cause is unknown, but environmental factors are thought to play a role by triggering abnormal responses in genetically susceptible hosts. PubMed. Ann Med. 2007;39(1):42-53. (Also see: Causes of Scleroderma: Genetics, and Environmental) |
| Clinical risk assessment of organ manifestations in systemic sclerosis - a report from the EULAR Scleroderma Trials And Research (EUSTAR) group data base. Diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subsets are associated with particular organ manifestations, but in this analysis the clinical distinction appeared superseded by an antibody based classification in predicting some scleroderma complications. PubMed. Ann Rheum Dis. 2007 Feb 1. (Also see: Antibodies) |
| Clinical
and Immunological Features in Patients with Systemic Sclerosis. There
are differences in clinical and immunological findings between
diffuse scleroderma (dSSc) and limited scleroderma (lSSc):
severe capillary damage, arthralgia, muscle weakness, tendon
friction rubs, joint contractures, esophageal, pulmonary,
cardiac and renal involvement are more common in patients
with dSSc. S. Arsik. AB0194 EULAR 2005. (Also see: Diffuse
Scleroderma, and Limited
Scleroderma) |
| Classification
of systemic sclerosis. There is no convincing evidence
of any advantage for distinguishing the limited, intermediate
and diffuse forms of SSc rather than only the limited and
diffuse forms. PubMed. Rheumatology (Oxford). 2005 May
3. |
| A
proposal of criteria for the classification of systemic sclerosis. Criteria
for the classification of systemic sclerosis have been proposed.
Preliminary testing has defined the sensitivity and specificity
of these criteria as high as 99% and 100%, respectively. PubMed.
Med Sci Monit. 2004 Oct 26;10(11):CR615-621. |
| Identification
of masqueraders of autoimmune disease in the office. There
are several rheumatologic and autoimmune disorders that can
masquerade as allergic disease. These conditions include
rheumatoid and juvenile arthritis, Sjogren's syndrome, systemic
lupus erythematosus, Behcet's and antiphospholipid syndromes,
systemic sclerosis, vasculitis, sarcoidosis, chronic fatigue
syndrome, and fibromyalgia. PubMed. Allergy Asthma Proc.
2003 Nov-Dec;24(6):421-9. (Also see: Rheumatoid
Arthritis, Sjogren's
Syndrome, Lupus, Behcet's, Antiphospholipid, Vasculitis, Sarcoiditis, Fibromyalgia
and CFS) |
| Systemic Sclerosis:
Prognosis and Mortality |
| Years ago, only the worst cases
of systemic scleroderma were diagnosed, thus leading to the
widespread belief that systemic scleroderma was always progressive
and fatal. Now that the full range of types and severity of
scleroderma are being recognized and diagnosed, there is increasing
awareness that many people have a milder type of illness with
a much rosier longterm prognosis. |
| Cardiac Involvement in Systemic Sclerosis: The Strongest Predictive Factor of Prognosis in Patients with Scleroderma. The results disclosed that most frequent cardiac manifestation at the initial evaluation of scleroderma is subclinical arrhythmia. More importantly, cardiac involvement at early stage of the disease is the strongest predictive factor for death. Sumiaki Tanaka. 13/13. ACR 2007. (Also see: Cardiac Involvement) |
| Skin Thickness Progression Rate (STPR) in Systemic Sclerosis with Diffuse Cutaneous Involvement: A Predictor of Outcome. Rapid STPR at first evaluation in early dcSSc patients is a predictor of both internal organ involvement at one year after onset of skin thickening and 5 year mortality. Assessment of individual risk in dcSSc patients and planning of clinical trials involving these patients should include evaluation of STPR. (Also see: Diffuse SSc, and Skin Fibrosis). |
| Risk Factors For Mortality In Patients With Systemic Sclerosis (SSc) And Interstitial Lung Disease (ILD). Mortality in patients with SSc and ILD is increased in those with an early and severe impairment of pulmonary function, concomitant cardiac involvement and elevated ESR (Sed rate), but not in Scl-70 positive patients. Beatriz E. Joven. 6/6. ACR 2007. (Also see: Pulmonary Fibrosis) |
| Scleroderma patients nailfold videocapillaroscopic patterns are associated with disease subset and disease severity. Nailfold videocapillaroscopy, a simple, non-invasive and non-expensive investigation, is useful in staging scleroderma patients and also provides prognostic information. Rheumatology 2007 46(10):1566-1569. (Also see: Nailfold Capillaroscopy) |
| Systemic Sclerosis Patients Have Activating Antibodies Targeting Both Endothelin Receptor Type A And Angiotensin Ii Type 1 Receptor Predicting Worse Prognosis. Anti-AT1R and anti-ETAR antibodies are a biomarker for severe disease and worse prognosis and could explain pathogenic features found in systemic sclerosis. The detection of these antibodies could identify SSc patients that might benefit from a receptor blockade or from a specific modulation of the antibody-receptor interaction. G. Riemekasten OP0162 EULAR 2007. (Also see: Causes of Scleroderma: Endothelin and Antibodies) |
| Mortality And Histological Characteristics Of SSc - A Retrospective Study Of 12 Autopsy Patients. SSc is a progressive multifocal process characterized by histological (vascular and interstitial) changes co-existing in different stages of their progression. In the course of the disease new foci develop, which increase in size and number, may become confluent, ultimately leading to diffuse, systemic interstitial sclerosis. Á. Apáthy THU0235 EULAR 2007. |
| Impairment
of the antifibrotic effect of hepatocyte growth factor (HGF) in lung fibroblasts
from African Americans: Possible role in systemic sclerosis. Reduced levels of HGF as well
as a deficiency in c-Met receptor function appear to be present in African
American patients with SSc. These findings may explain in part the greater
disease severity and worse prognosis observed in African Americans with SSc. Arthritis and Rheumatism. Volume 56, Issue 7, Pages 2432 - 2442.
(Also see: Pulmonary Fibrosis) |
| Scleroderma Outlook
Improves as Survival Increases. Professor Virginia Steen, M.D., studied
2,000 patients with scleroderma (also known as systemic sclerosis) treated
between 1972 and 2001 at the University of Pittsburgh and found that 10-year
survival steadily improved over those years by 12 percent-- from 54 percent
to 66 percent. Georgetown University Medical Center. 07/10/07. |
| Changes
in causes of death in systemic sclerosis, 1972- 2002. Survival
of scleroderma has changed since the treatment of renal crisis
became possible. The change in pattern of scleroderma- related
mortality over the past 30 years implicates the lung (both
pulmonary hypertension and pulmonary fibrosis) as the primary
causes of scleroderma related deaths today. PubMed. Ann
Rheum Dis. 2007 Feb 28. (Also see: Pulmonary
Involvement and Renal
Involvement) |
| Prognostic markers for systemic sclerosis. The prognosis of systemic sclerosis depends chiefly on the extent of the skin lesions, which correlates with the severity of the cardiovascular, pulmonary, and renal manifestations. PubMed. Joint Bone Spine. 2006 Oct;73(5):490-4. |
| Morbidity and mortality of patients diagnosed with systemic sclerosis after the age of 75: a nested case-control study. We conclude that a diagnosis of SSc at an older age appears to be a poor prognostic indicator related to both disease severity and comorbidities. A higher clinical suspicion will lead to an earlier diagnosis and a potential decrease in morbidity and mortality. PubMed. Clin Rheumatol. 2006 Nov;25(6):831-4. |
| Outcome of patients with scleroderma admitted to intensive care unit. A report of nine cases. The outcome of scleroderma patients admitted to the ICU was extremely poor. Infectious complication was the most common cause of death in our patients. PubMed. Clin Exp Rheumatol. 2006 Jul-Aug;24(4):380-6. (Also see: Pulmonary Fibrosis) |
| Gender
Differences in Systemic Sclerosis Clinical Expression and
Survival. Male systemic sclerosis patients present more
renal failure, conduction disturbances and inflammatory myopathy,
and less anti-centromere antibodies than female patients. B.
Joven. FRI0361 EULAR 2006. (Also see: Causes
of Scleroderma: Hormones and Chromosomes) |
| Predictive
markers for development of severe organ involvement in patients
with systemic sclerosis (SSc). Prognosis of SSc is associated
with the extent of skin involvement and the presence of lung,
heart, kidney, and/or digestive tract damage. Tto avoid irreversible
tissue injury, early detection of visceral involvement is
crucial for prompt initiation of therapy. PubMed. Ann
N Y Acad Sci. 2005 Jun;1051:455-64. |
| Predictors
of Severe Internal Organ Involvement in Early Systemic Sclerosis. Rapidly
progressive SSc may be predictable on clinical and lab grounds
at the time of disease onset that allow to identify patients
who require more careful follow-up and aggressive treatment. N.
G. Guseva. FRI0100 EULAR 2005. |
| Mortality
in systemic sclerosis: An International meta-analysis of
individual patient data. Systemic sclerosis confers a
high mortality risk, but there is considerable heterogeneity
across settings. Internal organ involvement and anti-topoisomerase
I antibodies are important determinants of mortality. PubMed.
Am J Med. 2005 Jan;118(1):2-10. |
| Changes
in Causes of Death in Systemic Sclerosis Over the Past 30
Years. Throughout the past 30 years, the frequency of
deaths from RC (renal crisis) has dramatically decreased
and at the same time the frequency of PF (pulmonary fibrosis)
increased. However, only 10% of SSc patients surviving RC
have died of PF, which may be because patients with the highest
frequency of RC have a low frequency of PF. Over the past
10 years, SSc patients have had improved survival, longer
disease duration at the time they die and are less likely
to die from scleroderma related complications. Pulmonary
hypertension and pulmonary fibrosis now account for 50% of
SSc related deaths and 25% of all causes of death in SSc
patients. Virginia Steen. 1052/432. ACR 2004. (Also see: Renal
Involvement, Pulmonary
Fibrosis, and Pulmonary
Hypertension.) |
| The
Outcome of Systemic Sclerosis Patients who Present in the
First Year of Their Illness. Systemic sclerosis (SSc)
has a variable onset and course. Patients who have diffuse
skin thickening very early in their disease have the highest
risk for severe organ involvement, but those patients with
diffuse scleroderma who do not develop severe organ involvement
within the first 3 years of disease have an excellent long
term survival. Virginia Steen. 1051/431. ACR 2004. (Also
see Diffuse
Scleroderma) |
| CREST Syndrome |
| CREST
Syndrome, by itself, does not have any skin tightening
at all. CREST stands for Calcinosis, Raynaud's, Esophagus,
Sclerodactyly, and Telangiectasia. CREST may occur alone,
or in combination with any other form of Scleroderma (or
even other autoimmune diseases) such as CREST with Limited
Scleroderma, or CREST with Lupus. See CREST
Syndrome. |
| Limited Scleroderma |
| Limited
Scleroderma is when skin involvement is limited to the
hands (although the face and neck may also be involved.) See Limited
Scleroderma. |
| Diffuse Scleroderma |
| Diffuse
Scleroderma is when skin tightening also occurs above
the wrists (or elbows, see below). There are several subcategories
of Diffuse, such as Scleroderma sans Scleroderma where there
is internal organ fibrosis, but no skin tightening; and Familial
Progressive Systemic Sclerosis, a rare form which runs in
families. See Diffuse
Scleroderma. |
| Overlap Syndrome |
| Overlap
Syndrome. If a scleroderma patient also has any other
autoimmune disease (such as lupus, rheumatoid arthritis,
etc.) it is referred to as overlap;
as in "Diffuse scleroderma in overlap with lupus." Scleroderma
symptoms can also be a part of mixed connective tissue disease
(MCTD), or undifferentiated connective tissue disease (UCTD). See Overlap
Syndrome. |
| See Also |
| Juvenile
Scleroderma by ISN. |
| Scleroderma
Patient and Caregiver Stories by ISN. |
