| What is Scleroderma? (MAIN MENU) |
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| Types of Scleroderma: Overview |
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Systemic Sclerosis (Scleroderma) |
| This page was written by Shelley Ensz and has not yet been medically edited. See disclaimer. |
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| Overview |
| 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. |
| Types of 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 Types of Scleroderma, and Systemic Scleroderma Symptoms) |
| Classification of Systemic Sclerosis: Limited and Diffuse |
| Systemic sclerosis. Systemic sclerosis and its subtypes differ significantly from other diseases in rheumatology and clinical immunology, as the aberrant activation of the immune system does not result in an inflammation-driven destruction but in a progressive matrix synthesis, especially of the skin. (PubMed) Muller-Ladner U. Internist(Berl). 2008 Mar;49(3):278-285. (Also see: Prof. Ulf Müller-Ladner, MD: ISN Medical Advisory Board) |
| 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) |
| 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) |
| 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) |
| 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) |
| 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. |
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| 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. |
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| 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. |
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| 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. |
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| See Also |
| Juvenile Scleroderma by ISN. |
| Scleroderma Patient and Caregiver Stories by ISN. |
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