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What is the life expectancy of someone with systemic sclerosis?

What is the life expectancy of someone with systemic sclerosis?

Patients with limited cutaneous systemic sclerosis have a 10-year survival estimate of approximately 90%. However, development of interstitial lung disease (ILD) or pulmonary artery hypertension (PAH) dramatically increases the risk of death.

What is juvenile systemic sclerosis?

Juvenile systemic sclerosis (jSSc) is a multisystem connective tissue disease characterized by skin induration and widespread fibrosis of internal organs. The incidence has been reported at 0.27–0.50 per million children per year in the UK and Finland (1, 2), with a prevalence of 3 per million (3–5).

Is systemic scleroderma life-threatening?

Not only can it affect the skin, but it also can affect many internal organs, hindering digestive and respiratory functions, and causing kidney failure. Systemic scleroderma can sometimes become serious and life-threatening.

Can you live a full life with systemic sclerosis?

Many people have a good scleroderma prognosis – they do not die of the disease and live a full and productive life. However, some people do die from scleroderma, for example those with severe lung, heart or kidney involvement.

Is systemic sclerosis a terminal illness?

Prognosis of Systemic Sclerosis Sometimes systemic sclerosis worsens rapidly and becomes fatal (mainly with diffuse systemic sclerosis). At other times, it affects only the skin for decades before affecting internal organs, although some damage to internal organs (such as the esophagus) is almost inevitable.

Is systemic sclerosis curable?

Treatment of Systemic Sclerosis There is no cure for systemic sclerosis. No drug can stop the progression of systemic sclerosis. However, drugs can relieve some symptoms and reduce organ damage. Nonsteroidal anti-inflammatory drugs (NSAIDs) help relieve joint pain but may cause gastrointestinal problems.

Is systemic sclerosis the same as scleroderma?

The word “scleroderma” means hard skin in Greek, and the condition is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs. The condition is also called systemic sclerosis because the fibrosis can affect organs other than the skin.

At what age is scleroderma usually diagnosed?

Scleroderma (sklair-oh-DUR-muh) is a group of rare diseases that involve the hardening and tightening of the skin and connective tissues. Scleroderma affects women more often than men and most commonly occurs between the ages of 30 and 50.

Can systemic sclerosis be cured?

People often have antibodies in the blood characteristic of an autoimmune disorder. There is no cure for systemic sclerosis, but symptoms and organ dysfunction can be treated.

What foods should be avoided with scleroderma?

Avoid foods that may aggra- vate symptoms such as citrus fruits, tomato products, greasy fried foods, coffee, garlic, onions, peppermint, gas-producing foods (such as raw peppers, beans, broccoli or raw onions), spicy foods, carbonated beverages and alcohol.

Is systemic sclerosis a disability?

The SSA recognizes systemic sclerosis as an impairment under Section 14.00 Immune System Disorders. To be eligible for disability benefits, you must be able to provide medical documentation that you meet the definition as listed in its Blue Book.

Can systemic sclerosis cause back pain?

Non-disease-specific symptoms in systemic sclerosis (SSc), like low back pain, are frequently reported in the early stages of the disease and may be associated with higher pain chronification and psychological issues, according to a study in Arthritis Research & Therapy.

What kind of muscle pain does systemic sclerosis cause?

Sometimes inflammation of the muscles ( myositis ), with its accompanying muscle pain and weakness, develops. Systemic sclerosis can damage large areas of skin or only the fingers (sclerodactyly). Sometimes systemic sclerosis tends to stay restricted to the skin of the hands. Other times, the disorder progresses.

What are the side effects of generalized systemic sclerosis?

In generalized systemic sclerosis with diffuse skin involvement, patients have Raynaud phenomenon and gastrointestinal (GI) complications. This type typically evolves rapidly. Interstitial lung disease and scleroderma renal crisis are the major complications.

How is the diagnosis of systemic sclerosis made?

Systemic Sclerosis is diagnosed based on the presence of various symptoms above and physical examination findings. The diagnosis can be confirmed by the presence of certain autoantibodies in the blood as well as radiographic studies. Particularly, the ANA, or the antinuclear antibody test, is positive, but not always.

What is the difference between localized scleroderma and systemic sclerosis?

Localized scleroderma (also known as morphea or linear scleroderma) Two/thirds of those diagnosed with scleroderma have the localized form, which affects the skin and not the internal organs as detailed above. Localized scleroderma does NOT evolve into systemic scleroderma.