Scleroderma

Scleroderma, also known as systemic sclerosis, is a multi-system autoimmune connective tissue disorder. As such, it affects many separate organ systems, which are discussed separately:

The remainder of this article is a general discussion of scleroderma.

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), which is even more pronounced during reproductive years (F:M 10:1) 2.

Clinical manifestation depends on which systems are predominantly involved. It should be noted that the majority of patients 70-97% develop an arthropathy that mostly affects the fingers, wrists and ankles 3,5. 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.

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, and results in perivascular fibrosis and gradual luminal stenosis 5.

Markers

Serological markers include 3:

  • elevated erythrocyte sedimentation rate (ESR)
  • rheumatoid factor (RF): positive in 30-40%
  • antinuclear antibodies (ANA): 35-96%
  • anti SCL-70: 30-70% (particularly in diffuse disease)
  • anti-centromere antibodies: 20-40% (particularly in limited disease)
  • antitopoisomerase I
Associations

These are best discussed under manifestations of individual systems. Please refer to respective subsections.

No cure for scleroderma exists. Treatment aims to reduce systemic involvement and target particular symptomatic organ systems. For targetted 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, the 10-year survival is approximately 75%. However, patients with diffuse involvement have a 10-year survival closer to 55% 5.

Share article

Article Information

rID: 2028
Synonyms or Alternate Spellings:
  • Progressive systemic sclerosis (PSS)
  • Systemic sclerosis
  • Progressive systemic sclerosis

Support Radiopaedia and see fewer ads

Cases and Figures

  • Drag
    Case 1
    Drag here to reorder.
  • Drag
    Case 2
    Drag here to reorder.
  • Drag
    Csse 3: with small bowel involvement
    Drag here to reorder.
  • Drag
    Scleroderma
    Case 4
    Drag here to reorder.
  • Drag
    Case 5
    Drag here to reorder.
  • Drag
    Case 6
    Drag here to reorder.
  • Drag
     Case 7
    Drag here to reorder.
  • Drag
    Case 8
    Drag here to reorder.
  • Updating… Please wait.
    Loadinganimation

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.