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Almost all complications of systemic scleroderma involve fibrotic changes in one or more organs and can occur in adults as well as children. Complications may include:

Gastrointestinal Complications

Gastrointestinal symptoms are often one of the earliest symptoms experienced by patients systemic scleroderma. Approximately 90% of patients with systemic scleroderma experience some gastrointestinal difficulty. These include:

• Difficulty swallowing
• Heartburn - this is one of the most common symptoms. If left untreated it can lead to Barrett's Esophagus, a precancerous condition.
• Perforations of the bowel due to scarring
• Constipation or diarrhea
• Fecal incontinence

Pulmonary (Lung) Complications

There is almost always a reduction in lung function in both limited and diffuse systemic scleroderma. The most common symptom is shortness of breath after exertion. A non-productive cough may also develop. There is rarely chest pain. Other lung problems may include:

• Pulmonary fibrosis (scarring) - more common in diffuse scleroderma
• Pulmonary hypertension - more common in limited scleroderma
• Pleural effusion - increased amounts of fluid within the pleural cavity usually due to inflammation
• Pleuritis - inflammation of the membranes surrounding the lungs

Many patients stabilize or improve after the initial decline of lung function but every patient with systemic scleroderma must be monitored carefully on a regular basis to evaluate presence of signs of progression of lung disease.

Renal (Kidney) Complications

Approximately 10-45% of patients with systemic scleroderma develop a condition called scleroderma renal crisis within 4 years of the onset of the disease. Scleroderma renal crisis is characterized by:

• Systemic hypertension (high blood pressure)
• Hyperreninemia - elevation of rennin levels in the blood
• Uremia - increased levels of urea in the blood
• Microangiopathic hemolytic anemia
• Kidney failure

Prior to the discovery of a group of drugs called angiotensin-converting enzyme (ACE) inhibitors, scleroderma renal crisis was almost uniformly fatal. Prompt diagnosis and early, aggressive therapy with ACE inhibitors has improved the survival rate significantly in recent years.

Cardiac Complications

Cardiac complications of systemic scleroderma are not common but if they occur, it is usually a late complication. Early cardiac involvement carries a poor prognosis for systemic scleroderma. Some of the cardiac complications may include:

• Fibrosis (scarring) of the heart muscles
• Arrhythmias - heart rhythm and conduction disturbances
• Congestive heart failure
• Hypotension - low blood pressure

Other Complications

Other possible complications of systemic scleroderma include:

• Reduced mouth opening and dental problems
• Risk of premature birth or low birth weight babies
• Osteoporosis
• Biliary (liver) cirrhosis
• Neuropathy (if the fibrosis impinges on a nerve). This often occurs as trigeminal neuralgia when there is facial involvement.
• Hypothyroidism (if there is fibrosis around the thyroid gland)
• Impotence
• Stomach cancer
• Lung cancer