A type of systemic sclerosis characterised 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's phenomenon, oesophageal dysmotility, sclerodactyly, and telangiectasia. This term is rarely used now as CREST features are not limited to this type of systemic sclerosis.
Presence of anti-centromere antibody implies better prognosis and longer survival.
Treatment is targeted to symptoms, or to organs involved, and to influence 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 auto-immune connective tissue disease characterised by the production of auto-antibodies (e.g., antinuclear antibody, anti-centromere antibody, and anti-topoisomerase I antibody) and the overproduction of collagen, which causes fibrosis of the skin and organs as well as vasculopathy with Raynaud's 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 characterised 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 anti-centromere antibodies is uncommon in patients with dcSSc; however, anti-topoisomerase 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's phenomenon, oesophageal 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
- presence of risk factors
- Raynaud's 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
- full 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
- anti-centromere antibody
- oesophageal manometry
no renal crisis
Janet E. Pope, MD, MPH, FRCPC
Professor of Medicine
Division of Rheumatology
Department of Medicine
Schulich School of Medicine and Dentistry
University of Western Ontario
Division of Rheumatology
St. Joseph's Health Care
JEP has consulted for and/or performed research trials with AbbVie, Actelion, Amgen, BMS, Lilly, Merck, Novartis, Pfizer, Roche, Sandoz, Sanofi, and United Chemicals Belgium. JEP is an author of several references cited in this topic.
Virginia Steen, MD
Professor of Medicine
VS has received research funding, consulting fees, and honorarium from Actelion and Gilead, which could result in competing interests. VS is an author of a number of references cited in this topic.
Frank Wollheim, MD
Department of Rheumatology
Lund University Hospital
FW is an author of a reference cited in this topic.
Gabriela Riemekasten, PD, Dr. med.
Medizinische Klinik mit Schwerpunkt
Rheumatologie und Klinische Immunologie
GR is a consultant for Encysive, Actelion, Bayer, GSK, Roche, and other companies, and received lecture fees from all of these companies involved in systemic scleroderma. GR is an author of a reference cited in this topic.
- Generalised morphoea
- Eosinophilic fasciitis (Shulman's syndrome)
- Nephrogenic systemic fibrosis
- BSR and BHPR guideline for the treatment of systemic sclerosis
- Update of EULAR recommendations for the treatment of systemic sclerosis
HeartburnMore Patient leaflets
- Log in or subscribe to access all of BMJ Best Practice
Use of this content is subject to our disclaimer