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Signs and Symptoms

The signs and symptoms that someone experiences may range from mild to debilitating and in some cases can be life-threatening. They depend upon the type and severity of the PSS and will vary both from person to person and over time. Many of the symptoms may also be seen with other disorders and some are nonspecific. Nonspecific symptoms may include:

  • Joint pain
  • Difficulty swallowing
  • Fatigue
  • Dry mouth and mucus membranes
  • Loss of appetite

Localised sclerosis (morphea and linear scleroderma) 
People with localised sclerosis may have one or many patches or lines of thickened, hardened skin. The skin may be reddened or white with purplish borders. In some cases, large areas of the skin may have patterns of hypo- and hyper-pigmentation that give a salt-and-pepper effect.

Systemic sclerosis (LSS and PSS) 
People with limited SS commonly have one or more of the group of symptoms referred to as "CREST." These include:

  • Calcinosis: Small calcium deposits that develop under the skin, primarily on the fingers, knees and elbows. They can become infected and can cause painful, open sores when they break through the skin.
  • Raynaud's phenomenon: Blood vessels in the hands and feet contract in response to cold or emotional stress with this condition. When this happens, blood flow decreases and the affected fingers and toes turn white, then blue. When blood flow returns to normal, they turn red. The recurrent or prolonged interruption in blood flow can damage tissues, leading to sores and, in some severe cases, to gangrene and the need for amputation. Mild to moderate Raynaud's phenomenon is relatively common and only a small percentage of people who have Raynaud's also have PSS. On the other hand, up to 90 per cent of people with limited cutaneous PSS will have Raynaud's.
  • Esophageal dysfunction: Many people with PSS have difficulty swallowing due to muscle dysfunction in their oesophagus and may have acid reflux and chronic heartburn.
  • Sclerodactyly: This refers to the development of tight, thick skin on the fingers that makes them difficult to bend. There may be hair loss and the loss of sweat glands. The skin may be shiny and discoloured.
  • Telangiectasia: Associated with tiny red spots on the face and hands. It is caused by the swelling of small blood vessels.

The presence of a centromere pattern as seen on an ANA test by immunofluorescence is strongly associated with CREST. About 60-80 per cent of people with CREST will have this pattern. (See the section on Tests) Tests that are specific for centromere antibodies, available from certain reference laboratories, also will be positive in many of those with CREST.

When PSS occurs on the face, it may make the mouth opening narrower, making teeth cleaning and care more difficult.

Those with PSS may have skin and organ involvement. Organs that may be affected include:

  • Lungs: Fibrosis can stiffen lung tissue, making it harder to breath and leading to pulmonary hypertension. Lung dysfunction is the leading cause of death in systemic PSS.
  • Kidney: PSS can cause acute renal crisis that can be life-threatening. It can also cause hypertension and chronic renal dysfunction.
  • Heart: Less common, and more likely to occur after several years, heart involvement can lead to heart palpitations and congestive heart failure.

Complications associated with systemic sclerosis that may also be associated with other disorders include:

  • Carpal tunnel syndrome
  • Joint contractures
  • Muscle weakness
  • Constipation, diarrhoea, and malnutrition
  • Erectile dysfunction

Last Review Date: January 13, 2020