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lindagray

Scleroderma Symptoms 4

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Cardiac (Heart)

Most systemic scleroderma patients have limited heart problems that may be detectable but are not clinically significant.   Even with diffuse scleroderma, serious heart complications are uncommon and occur in only 10% to 15% of patients, usually within the first few years after the disease begins.  The complication rate with limited scleroderma is even lower.  When more severe heart problems develop, they can be difficult to manage and may be associated with poor prognosis.  The most direct effect on the heart is scarring, which increases the risk of heart rhythm problems.  Also, a condition called pericarditis (inflammation of the membrane around the heart) can occur.

Renal (Kidney)


Kidney involvement is common in scleroderma, although there may be no obvious clinical problems. Kidney problems tend to be more serious and more common in the diffuse form of the disease, especially with RNA Polymerase III antibodies, with life-threatening scleroderma renal crisis occurring in 10% to 20% of diffuse scleroderma patients.  Scleroderma renal crisis is much less common in limited scleroderma although it can occur, often early in the disease.  Approximately 80% of all major kidney problems occur within the first 4 to 5 years of the disease. For unknown reasons, serious kidney problems are more common in men and with patients who had an older age of disease onset.  Note that treatment with high dose corticosteroids can increase the chances of developing major kidney problems and should generally be avoided in patients with early diffuse scleroderma.

Since systemic scleroderma patients tend to have relatively low blood pressure compared to the general population, any sudden increase in blood pressure is of concern with scleroderma patients.  For this reason, frequent monitoring of blood pressure is important, especially for diffuse scleroderma patients for the first few years of the disease.

Sexual Dysfunction

Sexual dysfunction is very common in patients with systemic scleroderma.  A recent study (Schouffoer et al. 2009) found that women with systemic sclerosis reported significantly impaired sexual functioning and more sexual distress than healthy controls, often leading to marital distress and depressive symptoms.  Major problems were increased vaginal dryness, skin tightness and decreased lubrication resulting in painful intercourse, heartburn and reflux during intercourse, and reduced frequency and intensity of orgasms.

Men with systemic scleroderma are much more likely to have problems with erectile dysfunction (ED) than men with rheumatoid arthritis (Hong et al. 2004).  Onset of ED averaged about three years after disease onset.  In the Hong study, about 81% of men reported problems with ED compared to 48% with rheumatoid arthritis.

Other Symptoms

While scleroderma does not appear to cause major central nervous system dysfunction, recent studies have shown that more than 50% of all scleroderma patients develop moderate to major depression (Thombs et al. 2007). Patients also frequently have difficulty with altered self-image because scleroderma can be disfiguring in some cases.  The incidence of depression is somewhat higher than would be expected in a population of patients with a severe, chronic disease.  However, in almost all cases the depression is responsive to treatment with medications commonly used to treat depression.

It is very common for patients with both limited and diffuse forms of scleroderma to have severe, sometimes debilitating fatigue.  It is not clear what the specific mechanism of action is for this fatigue, but anemia can often develop with scleroderma, which may contribute to the severity of this symptom.

A significant number of scleroderma patients also suffer from Sjögren’s syndrome (also called Sicca syndrome). The primary symptoms are dry mouth and eyes. This can result in dental complications and the need to use lubricating eye drops to prevent eye problems.

Hypothyroidism (reduced function of the thyroid) is very common in systemic scleroderma because of either fibrosis of the thyroid or thyroid autoimmune disorder. Hypothyroidism causes many bodily functions to slow down. Some of the more common symptoms include: hoarse voice, slowed speech, eye and face puffiness, weight gain, cold intolerance, dry skin,  carpal tunnel syndrome, and coarse, dry, sparse hair.

Sleep disturbance is also common with scleroderma patients (Frech et al. 2011).  There is a variety of reasons for this, including muscle and other pain (e.g., digital ulceration), difficulty breathing, and reflux symptoms.

Other symptoms that have been linked to scleroderma include: severe chronic chilling even in the absence of hypothyroidism, trigeminal neuralgia (sudden painful spasms in the lower portion of the face radiating to the neck), osteoporosis, increased occurrence of vertigo (dizziness), and liver damage.  However, in some cases, the linkage may not necessarily be a direct result from the underlying disease process in scleroderma.  For example, while an increased risk of osteoporosis is often listed as a potential complication of scleroderma, other co-factors such as early menopause, usage of corticosteroids, or malabsorption may in fact be the causal agent, rather than the disease process itself.

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