A type of systemic sclerosis characterized by skin fibrosis of the fingers (sclerodactyly) and, in some cases, of the face and neck or the skin distal to the elbows and/or knees. It does not affect the upper arms, upper legs, or trunk.
Previously known as CREST syndrome, which stands for the presence of calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. This term is rarely used now as CREST features are not limited to this type of systemic sclerosis.
Presence of anticentromere antibody implies better prognosis and longer survival.
Treatment is targeted to symptoms, or to organs involved, and to influencing the fibrosis of the skin and other connective tissues.
Renal crisis will be encountered by a small number of patients only; angiotensin-converting enzyme inhibitors reduce the likelihood of renal failure, dialysis, and death from this complication.
Systemic sclerosis (a type of scleroderma) is an autoimmune connective tissue disease characterized by the production of autoantibodies (e.g., antinuclear antibody, anticentromere antibody, and antitopoisomerase I antibody) and the overproduction of collagen, which causes fibrosis of the skin and organs as well as vasculopathy with Raynaud phenomenon and obliteration of blood vessels.
There are two main types of systemic sclerosis: limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc). The two types can be differentiated by the extent of skin involvement. lcSSc is characterized by skin fibrosis of the fingers (sclerodactyly) and, in some cases, of the face and neck or the skin distal to the elbows and/or knees. It does not affect the upper arms, upper legs, or trunk. In contrast, dcSSc also affects the trunk and the skin distal and proximal to the elbows and/or knees. The presence of anticentromere antibodies is uncommon in patients with dcSSc; however, antitopoisomerase I and anti-RNA polymerase III antibodies are more common in dcSSc compared with lcSSc. Systemic sclerosis sine scleroderma is a third, less common type of systemic sclerosis where there is no skin involvement but other features of systemic sclerosis are present. This monograph focuses on the evaluation and management of lcSSc.
CREST syndrome is defined by the presence of calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. Previously, the term was used to label patients with lcSSc; however, as CREST syndrome can occur in both lcSSc and dcSSc, the use of the term is now considered inaccurate, and it is not commonly used.
History and exam
Key diagnostic factors
- Raynaud phenomenon (RP)
- dilated nailbed capillaries
- bright shiny skin of hands, feet
- symmetrical swelling, tight fingers
- prayer sign
- claw hand deformities
- ulcers on fingers
- flexion contracture of hands
- carpal tunnel syndrome
- large well-demarcated telangiectasia
Other diagnostic factors
- skin pigmentation changes
- family history of connective tissue disease
- silica dust exposure
- family history of scleroderma
1st investigations to order
- complete blood count
- serum creatinine
- serum antinuclear antibody
- serum extractable nuclear antigens
- serum erythrocyte sedimentation rate (ESR)
- pulmonary function tests (PFTs)
Investigations to consider
- peripheral blood smear
- anticentromere antibody
- esophageal manometry
no renal crisis
- Generalized morphea
- Eosinophilic fasciitis (Shulman syndrome)
- Nephrogenic systemic fibrosis
- Update of EULAR recommendations for the treatment of systemic sclerosis
Carpal tunnel syndrome
Raynaud's phenomenonMore Patient leaflets
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