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)
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)
Face Validity Of Potential Systemic Sclerosis (SSc) Classification Criteria. Raynaud's phenomenon, digital ulcers, pulmonary fibrosis, telangiectasia, abnormal nailfold capillaries, calcinosis, dysphagia, sclerodactyly, anticentromere and SCL-70 antibodies have face validity. S. R. Johnson. EULAR 2011 OP0290. Ann Rheum Dis 2011;70(Suppl3):167.
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.
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.
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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