What are the first signs of scleroderma?

often starts as Raynaud’s (a circulation problem where fingers and toes turn white in the cold) other typical symptoms include thickening of the skin over the hands, feet and face, red spots on the skin, hard lumps under the skin, heartburn and problems swallowing (dysphagia)

What is the life expectancy of someone with systemic scleroderma?

Patients diagnosed with advanced systemic disease have a prognosis of anywhere from three to 15 years or more depending on the severity of the complications involving the lungs or another internal organ.

How does scleroderma affect the face?

Tight, hardened skin. In limited scleroderma, skin changes typically affect only the lower arms and legs, including fingers and toes, and sometimes the face and neck. Skin can look shiny from being pulled taut over underlying bone. It may become difficult to bend your fingers or to open your mouth.

What is the main goal of treatment of systemic sclerosis?

The aims of treatment are: To relieve symptoms. To prevent the condition from progressing, as much as possible. To detect and treat complications early.

Where does scleroderma usually start?

Morphea usually appears between the ages of 20 and 50, but is often seen in young children. Linear scleroderma is a form of localized scleroderma which frequently starts as a streak or line of hardened, waxy skin on an arm or leg or on the forehead.

What are the stages of scleroderma?

Cutaneous involvement has 3 phases: (1) edematous, (2) indurative, and (3) atrophic. Skin becomes thickened and tight. Note the images below.

Can you live a long life 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.

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.

What does skin thickening in scleroderma look like?

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.

Can systemic sclerosis be treated?

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.

Can systemic sclerosis be controlled?

There is no cure for systemic sclerosis. Survival is determined by the disease subset and internal organ manifestations. Interstitial lung disease and pulmonary artery hypertension account for almost two-thirds of deaths related to systemic sclerosis.

What are the symptoms of systemic sclerosis ( SSc )?

Gastrointestinal Tract: Any part of the gastrointestinal (GI) tract can be involved in SSc, and the symptoms vary depending on the involvement. Over 90% of patients have GI manifestations of some sort. The most common problem is reflux, which can feel like heartburn or lead to chronic cough.

What kind of skin tightening does systemic sclerosis cause?

Limited systemic sclerosis affects just the skin or mainly only certain parts of the skin and is also called CREST syndrome. People who have this type develop skin tightening (scleroderma) over the face and beyond the elbows and knees and may also have gastroesophageal reflux disease.

What are the three cardinal features of scleroderma?

Systemic sclerosis (scleroderma) has three cardinal features: excessive collagen production, vascular damage, and inflammation/autoimmunity. The pathogenesis of systemic sclerosis is obscure, but there are several contributing factors, including genetic, environmental, and autoimmune influences.

What’s the difference between localized and systemic sclerosis?

When scleroderma only affects the skin, it is considered “localized.” However, if it affects the skin and internal organs, it is viewed as “systemic,” also called Systemic Sclerosis (SSc). SSc affects approximately 100,000 people, or about one third of patients with scleroderma in the United States; this article will focus primarily on SSc.