- Type I - Diffuse skin involvement proximal to elbows/knees; includes trunk.
- Type II - Intermediate skin involvement proximal to the metacarpal phalangeal/metatarsal phalangeal joints, distal to the elbows/knees; trunk not involved.
- Type III - Digital sclerodactyly only (meets American College of Rheumatology minor criteria but excludes those without skin involvement).
- Type IV - Scleroderma sine scleroderma (capillary pattern or pitting scars and visceral involvement; no anticentromere antibodies; no telangiectasia).
- Type V - Undifferentiated connective-tissue disease with 2 of 3 of the following scleroderma features: sclerodactyly, pitting scars, or scleroderma capillary pattern; or one of these features along with one of the following: Raynaud phenomenon, pulmonary fibrosis, or visceral involvement (esophagus, heart, kidney); but do not meet the criteria for groups III and IV; no anticentromere antibodies; no telangiectasia.
- Type VI - CREST; no skin involvement, or sclerodactyly only, telangiectasia is required with one or more other acronyms; or anticentromere antibodies are required with any 2 or more acronyms.
References:
* "CREST Syndrome" eMedicine (link)
* Maricq HR, Valter I. A working classification of scleroderma spectrum disorders: a proposal and the results of testing on a sample of patients. Clin Exp Rheumatol. Jan-Feb 2004;22(3 Suppl 33):S5-13.
Tags: Anticentromere Antibodies - CREST - CT Disease - Pulmonary Fibrosis - Raynaud's Phenomenon - Sclerodactyly - Scleroderma - Scleroderma Spectrum Disorders - Telangiectasia
Posted by Medicalchemy
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