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Systemic Sclerosis (Scleroderma)

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Types of Systemic Scleroderma
Classification: Limited and Diffuse
CREST (old name for Limited)
Limited Systemic Sclerosis
Diffuse Systemic Sclerosis


Overview of Systemic Scleroderma
Presented by Amanda Thorpe

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. (Also see: What is Scleroderma?, Types of Scleroderma and Systemic Symptoms)

Types of Systemic Sclerosis (Systemic Scleroderma)

Classification Criteria for Systemic Sclerosis. The ACR/EULAR classification criteria for systemic sclerosis (SSc) performed better than the 1980 ACR criteria and should allow for more patients to be classified correctly as having the disease. Arthritis & Rheumatism, 3 October 2013.
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 Types of Scleroderma, and Systemic Scleroderma Symptoms)
Psoriasis and concomitant fibrosing disorders: Lichen sclerosus, morphea, and systemic sclerosis. In this population, a predisposition toward autoimmunity is seen as 38.5% of patients had a personal history of a third concomitant autoimmune disease, in addition to psoriasis and fibrosing disorder, whereas 42.3% reported a history of a first-degree relative with an autoimmune disease. Journal of the American Academy of Dermatology Volume 67, Issue 5 , Pages 1079-1083, November 2012. (Also see: Psoriasis, Lichen Sclerosus, Morphea, and Multiple Autoimmune Syndrome)

Classification of Systemic Sclerosis: Limited and Diffuse

The degree of skin involvement identifies distinct lung disease outcomes and survival in systemic sclerosis (SSc). These data suggest that the current classification subdividing SSc into limited and diffuse cutaneous subtypes misclassifies an intermediate group of patients exhibiting unique autoantibody profile, disease course and clinical outcomes. PubMed, Ann Rheum Dis, 2013 Apr 20. (Also see: Classification of Systemic Sclerosis)
Late-onset systemic sclerosis (SSc) —a systematic survey of the EULAR scleroderma trials and research group database. Late-onset SSc shows a distinct clinical presentation and outcome. Patients with late-onset SSc suffer more frequently from the limited subtype and pulmonary hypertension, but fewer patients have digital ulcers. Pulmonary Hypertension (PH) may in part be determined by underlying cardiovascular disease. Tomas Hugle. Rheumatology (2010) doi: 10.1093.
Diagnosis and Classification of Systemic Sclerosis (SSc). Patients with limited SSc (lSSc) must have Raynaud's phenomenon and SSc specific nail fold capillary changes and/or SSc specific autoantibodies. Some lSSc patients who have no cutaneous involvement but common SSc nail fold capillaroscopy abnormalities, specific antinuclear antibodies, and visceral involvement are sometimes called SSc sine scleroderma. Eric Hachulla. Clinical Reviews in Allergy and Immunology. February 09, 2010. (Also see: Diagnosis)

CREST Syndrome (the former name for Limited Systemic Scleroderma)

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. ISN.
Overview: What is CREST?
"Pure" CREST
"Plus" CREST
CREST Antibodies
CREST Overlaps
CREST Patient Stories
Also See

Limited Systemic Scleroderma (lSSc)

Limited Scleroderma is when skin involvement is limited to the hands (although the face and neck may also be involved.) ISN.
What is Limited Scleroderma?
Diagnosis of Limited Scleroderma
Research for Limited Scleroderma
Online Support for Limited Scleroderma
Patient and Caregiver Stories

Diffuse Systemic Scleroderma (dSSc)

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. ISN.
What is Diffuse Scleroderma?
Causes of Scleroderma
Sine: Diffuse Without Skin Involvement
Diffuse Patient Stories

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. ISN.
Overview of Polyautoimmunity
Scleroderma in Overlap
Connective Tissue Disease (CTD)
Mixed Connective Tissue Disease (MCTD)
Multiple Autoimmune Syndrome
Shared Autoimmunity
Undifferentiated Connective Tissue Disease (UCTD)
Overlap Patient and Caregiver Stories

Also See

Juvenile Scleroderma by ISN.
Scleroderma Patient and Caregiver Stories by ISN.
Go to Localized: Linear Scleroderma

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