Other Complications and Their Treatments

Joint Complications

Scleroderma is caused by multiple factors including immune system alteration, vascular dysfunction, and overproduction of collagen. Any of these can contribute to stiffness and pain in the joints. Like rheumatoid arthritis, scleroderma is described as an autoimmune rheumatic disease. However, it also includes its own unique symptoms and challenges.

Scleroderma can affect tendons, joints, nerves, and other structures that affect mobility.

People with systemic scleroderma are at high risk for changes in muscle, blood vessels, and other tissues, including the development of fibrotic tissue that can affect movement. Scarring and thickening of the skin can cause painful contractures of the fingers, wrists, or other joints, causing them to become “stuck” in a flexed position.

Those with Raynaud’s phenomenon may also experience the pain and discomfort of flares and ulcers. In addition, scleroderma can affect tendons, joints, nerves, and other structures that affect mobility.

Heart Complications

Among the many body parts affected by scleroderma, the heart is one of the most important. Some effects are direct, such as heart failure, cardiac fibrosis, coronary artery disease, and heart rhythm abnormalities. Other effects are indirect, usually caused by the involvement of other organs. Indirect effects can include renal (kidney) crisis and pulmonary arterial hypertension (PAH).

Symptoms of heart involvement in scleroderma are varied, depending on the location within the heart and the presence of any underlying conditions.

Symptoms of heart complications

Symptoms of heart involvement in scleroderma are varied, depending on the location within the heart and the presence of any underlying conditions. In addition, some cardiac symptoms may not be recognized if they are thought to be caused by pulmonary, musculoskeletal, or esophageal involvement.

Watch for these signs and symptoms:

  • Shortness of breath, which can be a sign of right or left heart failure
  • Night-time breathing difficulty
  • Swollen feet
  • Liver enlargement
  • Chest pain

Kidney Complications

Although not common, kidney problems can occur in certain forms of scleroderma. Scleroderma affects the kidneys in about 15% of the patient population. Kidney involvement in scleroderma tends to occur in the first five years after diagnosis and most often in patients with diffuse skin involvement (skin thickness that reaches the upper arms and trunk). Awareness of symptoms and regular at-home screening can help identify problems before they become severe.

In those with systemic scleroderma, the kidneys can be damaged due to a sudden and severe increase in blood pressure called scleroderma renal crisis (SRC).

In people with scleroderma, compromised kidney function can affect how well these patients can respond to aging and other causes of kidney damage, such as diabetes. In addition, kidney function can also affect the efficacy and possible toxicity of medications.

Because the kidneys remove wastes from the blood, any malfunction may cause these wastes to build in the blood and damage the body. In those with systemic scleroderma, the kidneys can be damaged due to a sudden and severe increase in blood pressure called scleroderma renal crisis (SRC).

People most at risk for this type of kidney crisis include those with:

  • Early diffuse systemic sclerosis with rapid progression (66% develop SRC within 1 year of diagnosis)
  • Presence of tendon friction rubs
  • A history of use of high-dose corticosteroid therapy (for example, >15 mg/day of prednisone)
  • Presence of RNA autoantibodies (anti-RNA polymerase III)
  • New anemia, which may be an early clue to the onset of SRC
  • Cardiac insufficiency, pericardial effusion, or other cardiac events may precede SRC 

Symptoms

Sudden onset of high blood pressure (hypertension) of more than 150/85mmHg is the key symptom of renal crisis. It causes almost immediate decrease in kidney function. Symptoms to watch for are:

  • Headache
  • Visual disturbances
  • Seizures
  • Fever
  • General malaise
  • Changes in blood or urine chemistry
  • Pulmonary edema (fluid in the lungs)