Scleroderma

Jun 24, 2019

By Trevor Shewfelt, Pharmacist at the Dauphin Clinic Pharmacy

Scleroderma is a relatively rare group diseases that involve the hardening and tightening of the skin and connective tissues. There are about 30,000 people in Canada with scleroderma. Scleroderma affects women more often than men by about 4 to 1. Symptoms usually start between the ages of 30 and 50. There is no cure for scleroderma, but there are treatments.

There are many different types of scleroderma. In about 2/3 of people, scleroderma affects only the skin. In about 1/3 of people, scleroderma also affects internal structures. This can include blood vessels, internal organs and the digestive tract (systemic scleroderma). Signs and symptoms vary, depending on which type of scleroderma you have. Nearly everyone who has scleroderma experiences a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines, or cover wide areas of the trunk and limbs. The number, location and size of the patches vary by type of scleroderma. Skin can appear shiny because it's so tight, and movement of the affected area may be restricted.

Scleroderma can affect the fingers or toes. One of the earliest signs of systemic scleroderma is Raynaud's disease, which causes the small blood vessels in your fingers and toes to contract in response to cold temperatures or emotional distress. When this happens, your fingers or toes may turn blue or feel painful or numb. Raynaud's disease also occurs in people who don't have scleroderma.

Scleroderma can affect the digestive system. Scleroderma can cause a variety of digestive symptoms, depending on which part of the digestive tract is affected. If the esophagus is affected, you might have heartburn or difficulty swallowing. If the intestines are affected, you might have cramps, bloating, diarrhea or constipation. Some people who have scleroderma may also have problems absorbing nutrients if their intestinal muscles aren't properly moving food through the intestines.

Scleroderma can affect the heart, lungs or kidneys. Scleroderma can affect the function of the heart, lungs or kidneys to varying degrees. These problems, if left untreated, can become life-threatening.

Scleroderma is caused by an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin.

We don't know what causes the abnormal collagen production to begin, but the body's immune system appears to play a role. That is why we call it an autoimmune disease. In 15 to 20 percent of cases, a person who has scleroderma also has symptoms of another autoimmune disease, such as rheumatoid arthritis, lupus or Sjogren's syndrome. Most likely, scleroderma is caused by a combination of factors, including immune system problems, genetics and environmental triggers.

Scleroderma complications range from mild to severe and can affect your:

Fingertips. The variety of Raynaud's disease that occurs with systemic scleroderma can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores. In some cases, the tissue on the fingertips may die and require amputation.

Lungs. Scarring of lung tissue can result in reduced lung function, which can impact your ability to breathe and tolerance for exercise. You may also develop high blood pressure in the arteries to your lungs.

Kidneys. When scleroderma affects your kidneys, you can develop elevated blood pressure and an increased level of protein in your urine. More-serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.

Heart. Scarring of heart tissue increases your risk of abnormal heartbeats and congestive heart failure, and can cause inflammation of the membranous sac surrounding your heart. Scleroderma can also raise the pressure on the right side of your heart and cause it to wear out.

Teeth. Severe tightening of facial skin can cause your mouth to become smaller and narrower, which may make it hard to brush your teeth or to even have them professionally cleaned. People who have scleroderma often don't produce normal amounts of saliva, so the risk of dental decay increases even more.

Digestive system. Digestive problems associated with scleroderma can lead to heartburn and difficulty swallowing. It can also cause bouts of cramps, bloating, constipation or diarrhea.

Sexual function. Men who have scleroderma may experience erectile dysfunction. Scleroderma may also affect the sexual function of women by decreasing sexual lubrication and constricting the vaginal opening.

As always if you have any questions or concerns about these or other products, ask your pharmacist.

The information in this article is intended as a helpful guide only. It is not intended to be used as a substitute for professional advice. If you have any questions about your medications and what is right for you see your doctor, pharmacist or other health care professional.

Scleroderma is a chronic disorder characterized by excessive accumulation and sclerosis (hardening and thickening) of the connective tissues. In its systemic form, there is also vascular dysfunction and dysregulation of the immune system. %u25A0 Scleroderma affects about 30,000 persons in Canada, with about 1/3 of cases being progressive systemic sclerosis (noticeable onset occurring most often in adults aged 30 to 55 years) and the remaining 2/3 being localized cutaneous forms (the linear type occurring most often in children and adolescents and the more common morphea type in women aged 20 to 40 years). PSS involves both skin and organs, and some persons with PSS also exhibit CREST syndrome. %u25A0 Similar to most autoimmune disorders, females are affected more often than males (4:1 in PSS). Other autoimmune disorders (including Sjögren's syndrome, lupus erythematosus, and rheumatoid arthritis) may exist concurrently with systemic scleroderma, resulting in overlap syndromes. %u25A0 Environmental triggers (such as vinyl chloride, epoxy resins, paint solvents, and pesticides) or drugs (e.g., bleomycin) contribute to onset of systemic scleroderma in a minority of cases. %u25A0 Currently there is no definitive treatment that controls the underlying problem - the overproduction of collagen - common to all forms of scleroderma. Corticosteroids and immunosuppressive drugs benefit some persons. %u25A0 In localized cutaneous scleroderma, only the skin is involved, and onset is usually gradual. The morphea type is typified by firm, hardened, and discoloured (often purple-brown) oval patches of skin, usually with hair loss in affected areas. Sometimes there is also muscle and joint pain. While disfiguring, morphea tends to be otherwise relatively benign. By contrast, the linear type tends to have more severe sequelae. It manifests as long, narrow areas of thickened and discoloured skin, most often affecting the lower limbs and torso, and, less frequently, the hands, arms, and face. In severe cases, contracture deformities occur, which limit movement of affected areas.

Systemic scleroderma is potentially life threatening, and sometimes rapidly progressive. In PSS, the disease can affect virtually any organ (in addition to skin and blood vessels) and may progress to affect many organ systems. The skin is typically affected first, although sometimes joint involvement may be the initial sign. Furthermore, Raynaud's phenomenon6 sometimes precedes other manifestations of PSS, including skin tightening. As fibrosis of organs progresses, signs of organ failure (e.g., pulmonary, cardiac, and/or renal) begin to appear. %u25A0 Occasionally, scleroderma manifests in the internal organs without any cutaneous manifestations ("systemic scleroderma sine scleroderma"). %u25A0 Cutaneous manifestations of PSS include pitting edema early in the disease process, followed by rigidity and tightness of the skin. Eventually, the skin becomes hard, smooth, and atrophic, often with telangiectasias7 and areas of hypopigmentation. Painful tightening and swelling of the skin occurs with resultant reduction in range of motion. The face becomes expressionless and resembles a mask, and the eyes may appear narrowed and the nose shrunken or pinched. In other cases, widened palpebral fissures may be associated with an inability to close the eyelids, exacerbating dryness of the eyes. %u25A0 Fibrosis of the fingers results in stiffness and atrophy of the skin over the digits, and the tightening of the skin's connective tissue may cause the fingers to become fixed in a distorted position ("sclerodactyly" or claw-like deformity). Ischemia and ulceration of the fingertips may result from vascular compromise. %u25A0 Joint and muscle pain are experienced by most persons with diffuse systemic scleroderma. As well, severe skin dryness and itching are common, because the excess collagen in the dermis overwhelms the sweat and sebaceous glands. %u25A0 In CREST syndrome, esophageal dysmotility results from fibrosis and atrophy of smooth muscles. Patients/clients may exhibit hoarseness and wheezing, and as the disease progresses, gastroesophageal reflux disease (GERD) can occur, sometimes resulting in stricture formation. %u25A0 Malabsorption of nutrients, weight loss, and diarrhea or constipation may result from smooth muscle disruption in the intestine. %u25A0 Trigeminal neuralgia8 may occur. %u25A0 Dry eyes are common, particularly in PSS patients/clients who also have Sjögren's syndrome. %u25A0 Erectile dysfunction, painful intercourse, or pregnancy complications may occur in persons with systemic scleroderma. %u25A0 In many patients/clients, systemic disease stabilizes after a time. However, continued progression can lead to death, with restrictive lung disease (and associated pulmonary hypertension) being the leading cause. Severe renal disease develops in up to 15% of persons with PSS, leading to acute renal failure ("scleroderma renal crisis") as well as chronic kidney disease. Hypertension needs to be aggressively managed. %u25A0 Most patients/clients with localized scleroderma have a good prognosis and normal lifespan. %u25A0 Depression occurs in some patients/clients when they live with a severe, chronic disease.

 


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