Scleroderma statistics. Systemic Scleroderma 2022-12-27

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Scleroderma, also known as systemic sclerosis, is a chronic autoimmune disease that affects the connective tissue in the body. It causes hardening and thickening of the skin, as well as damage to internal organs and blood vessels. Scleroderma is a rare disease, but it can be serious and life-threatening, depending on the type and severity of the condition.

According to the Scleroderma Foundation, scleroderma affects about 300,000 people in the United States, with women being three times more likely to develop the disease than men. It is most commonly diagnosed in people between the ages of 30 and 50, although it can occur at any age.

There are two main types of scleroderma: localized and systemic. Localized scleroderma affects only the skin and is usually less severe than systemic scleroderma, which can affect the internal organs, blood vessels, and other parts of the body. Systemic scleroderma is divided into two subtypes: limited and diffuse. Limited scleroderma affects only certain areas of the body, such as the hands and face, while diffuse scleroderma affects a larger area and can be more severe.

The exact cause of scleroderma is unknown, but it is thought to be triggered by a combination of genetic and environmental factors. Some research suggests that scleroderma may be related to other autoimmune diseases, such as rheumatoid arthritis and lupus.

There is no cure for scleroderma, but treatments are available to help manage the symptoms and prevent complications. These may include medications to control inflammation, lower blood pressure, and improve blood flow; physical therapy to improve mobility; and skin care products to help maintain healthy skin. In severe cases, surgery may be necessary to repair damaged organs or blood vessels.

Despite advances in treatment, scleroderma can still be a serious and life-threatening disease. The mortality rate for people with scleroderma is about three times higher than the general population, with most deaths occurring within the first five years after diagnosis. However, with proper treatment and management, people with scleroderma can live long and active lives.

In conclusion, scleroderma is a rare autoimmune disease that affects the connective tissue in the body, causing hardening and thickening of the skin and damage to internal organs and blood vessels. It is most commonly diagnosed in women between the ages of 30 and 50, and there are two main types: localized and systemic. While there is no cure for scleroderma, treatments are available to help manage the symptoms and prevent complications. The mortality rate for people with scleroderma is higher than the general population, but with proper treatment and management, people with scleroderma can live long and active lives.

Diagnoses & Tests

scleroderma statistics

Retrieved 15 July 2017. A timely diagnosis is important to ensure appropriate management of the disease and associated complications. In a small number of cases, scleroderma is caused by specific environmental triggers like extensive exposure to silca dust or polyvinyl chloride. There are six other scleroderma-related autoantibodies that are far less common. Laboratory tests on your skin samples can show signs of fibrosis, or tissue scarring. Adverse events were rare and, in almost all cases, mild and transitory.

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Let's Talk About Scleroderma Mortality Statistics (or Not)

scleroderma statistics

While the typical explanation for the therapeutic benefits seen with TPE focuses on temporary reduction of circulating antibodies or other pathogenic factors, we propose instead an explanation based on abnormal blood rheology as a novel disease pathogenesis model for SSc. PDF from the original on 2014-03-06. My mother who did not have any known AI diseases was in her 20's when they discovered she had MVP and it was never a problem - she was 80 when she died. As soon as it becomes final I will be giving copies to all of my providers. The symptoms present, their severity, the rate of progression of the disease, the response to treatment, and overall survival vary widely depending on the affected individual. Your doctor can go over the test results with you and prescribe any treatments you need. Some affected people have muscle pain, breathing problems, and trouble swallowing.

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Systemic Scleroderma

scleroderma statistics

Varga J, Overview of pulmonary complications of systemic sclerosis scleroderma , UpToDate. In severe cases of SRC, dialysis and even renal transplantation might be required if renal function does not improve after the crisis. The combination of esophageal dismotility and LES incompetence can lead to heartburn due to acid reflux a condition known as gastroesophageal reflux disease; GERD , choking on food, and difficulty swallowing dysphagia. Gastrointestinal bleeding also occurs in some patients due to dilation of veins in the stomach gastric venous ectasias. It is my own personal belief to think about and speak about the subject of mortality.

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Research Articles

scleroderma statistics

King TE, Approach to the adult with interstitial lung disease: Clinical evaluation, UpToDate. People with anti-RNA polymerase III antibodies are at higher risk for rapidly progressive skin thickening and kidney problems like scleroderma renal crisis. About three per 100,000 people per year develop the systemic form. Current Opinion in Rheumatology. Environmental exposures likely have a role in the development of scleroderma. Symptoms associated with skin fibrosis include tight and dry skin, itching pruritus , fluid accumulation edema and increased or decreased skin pigmentation. As described above, immunosuppressants can be used in individuals with myocarditis.

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WSF

scleroderma statistics

Nineteen of these were case studies; the rest ranged from small observational studies to prospective randomized clinical trials. African American individuals tend to have a lower age of onset, higher rates of the diffuse cutaneous subtype, and overall more severe disease. Pulmonary function testing often includes a computed tomography CT or CAT scan, which is a series of images taken of your lungs from different angles. The symptoms are variable depending on the affected individual and can overlap with those seen in systemic scleroderma. Archives of Dermatological Research. Heart The treatment of systemic scleroderma-associated cardiac disease will depend on the specific cardiac manifestation that is present.

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10 Facts About Scleroderma

scleroderma statistics

You can eat and drink with the catheter in place. Systemic scleroderma, Genetic and Rare Diseases Information Center. Following a link to an external page is at your own risk. Back then, the statistics were much grimmer than they are today, because of a number of advances in treatment, e. Example of targeted therapies being investigated include rilonacept and tocilizumab, which respectively target IL-1 and IL-6, two inflammatory mediators thought to be important in systemic scleroderma. I worry that the mortality stats would be devastating to someone just newly and possibly poorly diagnosed. To put this in perspective, depending on which study you look at, the prevalence of systemic lupus erythematosus SLE is three to six times as high, close to 1000 per million adults in the US.

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Scleroderma

scleroderma statistics

Following a link to an external page is at at your own risk. I on the other hand knew I had been sick for 11 years by then, so I saw that as encouraging. This cannot be measured with a blood pressure cuff. Polskie Archiwum Medycyny Wewnetrznej. It is unusual for morphea to spread elsewhere in the body, and it is rare for morphea to invade past the skin and cause internal organ damage. These test results show what autoantibodies you have in your blood.

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Prevalence and Incidence of Systemic Scleroderma in the US

scleroderma statistics

You will feel a little pinch and burning sensation when they give you the injections. My husband and I had a similar experience to you and your wife when I was finally, accurately diagnosed. Therefore, additional studies of TPE effects in SSc appear to be highly desirable. Your doctor will use the tool to press down on your thick layer of skin and remove the samples to send to the lab. This can also lead to severe pain and ulcers.

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