scleroderma: do you know the signs?
the five symptoms most commonly reported by systemic scleroderma patients are fatigue, raynaud’s phenomenon, hand stiffness, joint pain and difficulty sleeping
4 minute read
for many patients, the first sign of scleroderma is a temporary decrease in the size of the blood vessels in the fingers and toes known as raynaud’s phenomenon. getty
scleroderma is a progressive autoimmune disease that results in the overproduction of collagen, which hardens the skin. although collagen typically has a softening effect, when it is allowed to accumulate it makes the skin thicken and scar. according to scleroderma canada, the rare condition can also produce vascular lesions in small blood vessels that contribute to this thickening while also threatening some of the body’s major organs. there are two main forms of the disease: localized scleroderma (which primarily affects the skin and has a better long-term prognosis) and systemic scleroderma (which affects the skin, underlying tissues and major organs and involves more potential complications). there is no cure for the chronic disease but there are treatments to slow its progression and improve quality of life for patients.
symptoms of scleroderma
for many patients, the first sign of scleroderma is a temporary decrease in the size of the blood vessels in the fingers and toes known as raynaud’s phenomenon. according to the scleroderma association of b.c., this symptom, which is triggered by cold weather or emotional stress, can result in bluish skin, a whitening of the toes or fingers and numbness. this phenomenon is present in 95 per cent of people with scleroderma and can appear up to 10 years before other symptoms, making it a key clue in diagnosing the disease. while raynaud’s phenomenon is most common in the hands and feet, it can also affect the nose, tongue and ears and may be related to digital ulcers (collagen-induced sores that form on the fingertips).
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other symptoms of the skin include microstomia (a hardening of the skin that makes it difficult to open the mouth) and contractures (a hardening of the skin on the fingers that forces them into a flexed or bent posture).
systemic scleroderma can involve a number of other symptoms that are unrelated to the skin itself, including:
- digestive problems — esophageal involvement can result in heartburn, regurgitation and trouble swallowing. small intestine involvement can result in obstruction of the intestines and affect the ability to absorb food. patients can also experience gastroesophageal reflux, which is acid regurgitation in the esophagus that produces a burning sensation following a meal. in rare, untreated cases, this can cause cancer and other complications. other digestive symptoms can include bloating, diarrhea, constipation and fecal incontinence.
- lungs, heart and blood vessels — patients can experience shortness of breath, dizziness and scarring of tissues. blood pressure can increase and cause pulmonary hypertension and, in some cases, an irregular heartbeat or even heart failure.
- kidneys — around five per cent of systemic scleroderma patients can experience renal crisis, which may require temporary or permanent dialysis.
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diagnosing scleroderma
scleroderma can be difficult to pin down because of the wide range of symptoms produced by the disease. doctors typically rely on blood tests (which can detect elevated levels of antibodies) and imaging and organ function tests when making a diagnosis.
treating scleroderma
there is no currently no way to prevent the overproduction of collagen, according to the mayo clinic, so treatment typically involves a combination of drugs (to manage symptoms) and therapy (to improve strength and mobility). medications are often prescribed to:
- dilate blood vessels — this can help treat raynaud’s phenomenon
- suppress the immune system — to slow skin thickening and lung damage
- decrease digestive symptoms — antacids relieve heartburn; antibiotics and other medications can help with bloating, constipation and diarrhea
- prevent infections — vaccines are vital to protect scleroderma-damaged lungs
- control pain — over the counter and stronger medications may be used to manage pain
preventing scleroderma
scleroderma is difficult to guard against because doctors are still unsure of its cause. they do know that the disease is not contagious or infectious and that it affects women and men of all races and ethnic groups at any stage of life. research into the disease has determined the average onset to be age 40, with localized scleroderma more common in children and systemic scleroderma more prevalent in adults. people with a family history of rheumatic disease may face a slightly elevated risk.
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prevalence of scleroderma
scleroderma is five-to-six times more common in women than men and affects around 2.7 million people around the world, or one in 100 people. both main forms of the disease are associated with a slight decrease in life expectancy, with the systemic version usually leading to less favourable outcomes. more than 80 per cent of patients are women between the ages of 30 and 50.
support for scleroderma
scleroderma canada is a great place to learn more about the disease, including updates on the latest news and research. the site also features links to local scleroderma societies that may offer patient support in your area.
dave yasvinski is a writer with healthing.ca
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