Scleroderma, also known as systemic sclerosis, is an autoimmune connective tissue disorder characterised by multisystem fibrosis and soft tissue calcification. As such, it affects many separate organ systems, which are discussed separately:
The remainder of this article is a general discussion of scleroderma.
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Epidemiology
Although presentation in childhood is indeed recognised 1, scleroderma is considered a disease of middle age (30-50 years of age) 3,4. There is a strong female predilection (F:M 3:1), even more pronounced during reproductive years (F:M 10:1) 2.
Associations
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other connective tissue disorders
Clinical presentation
Clinical manifestation depends on which systems are predominantly involved. Approximately 60% (range 24-97%) develop an arthropathy that mostly affects the fingers, wrists, and ankles 3,5,6. Oesophageal involvement is common, occurring in approximately 80% of patients 5. Although 90% of patients have pulmonary involvement histologically, only a minority are symptomatic 4,5.
Pathology
The disease is characterised by widespread deposition of collagen and other extracellular matrix proteins. This is believed to occur as a result of an abnormal immune response. Small vessels are involved early in the disease, accounting for the involvement of organs with a dense capillary network. This results in perivascular fibrosis and gradual luminal stenosis 5.
Markers
rheumatoid factor (RF): positive in ~40% (range 30-50%) 3,6
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antinuclear antibodies (ANA): positive in ~65% (range 35-96%) 3
anti-Scl-70 antibodies: positive in ~50% (range 30-70%), particularly in diffuse disease 3
anti-centromere antibodies: positive in ~30% (range 20-40%), particularly in limited disease 3
anti-topoisomerase I autoantibodies 3
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anti-cyclic citrullinated peptide (anti-CCP) autoantibodies 6
positive in ~5% (range 2.6-8.3%)
may identify patients with scleroderma-RA overlap syndrome, who in turn are more likely to develop joint erosions
Radiographic features
These are best discussed under manifestations of individual systems. Please refer to respective subsections.
Treatment and prognosis
No cure for scleroderma exists. Treatment aims to reduce systemic involvement and target particular symptomatic organ systems. For targeted therapy, please refer to the articles that pertain to scleroderma and the particular body system.
Systemic medication aimed at reducing the deposition of fibrous tissue and at reducing inflammation has been tried with limited success 5.
Overall prognosis is variable, depending on the organ(s) involved and the severity. For patients with limited cutaneous involvement, 10-year survival is approximately 75%. However, patients with diffuse involvement have a 10-year survival closer to 55% 5.
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