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 the survival rate for scleroderma?

Systemic sclerosis (scleroderma) runs a variable course. A review of 11 studies, totalling over 2000 patients, revealed 5-year cumulative survival rates ranging from 34 to 73 per cent, with a mean of 68 per cent [I]. Organ failure is generally regarded as the major cause of death in this disease.

What is the 10-year survival rate for patients with diffuse cutaneous systemic sclerosis scleroderma?

The 10-year survival rates were 81% for patients with limited cutaneous systemic sclerosis (SSc) and 70% for patients with diffuse cutaneous SSc (P = . 006).

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).

How does progressive systemic sclerosis affect the skin?

In progressive systemic sclerosis, the skin is affected in a number of other ways, including calcifications, telangiectasias, finger swelling, and sclerodactyly (tightening of the skin of the digits). Raynaud’s phenomenon occurs in as many as 90% of patients with progressive systemic sclerosis.

Is there a cure for systemic sclerosis ( SSc )?

The understanding of the pathogenesis of SSc has improved in recent years, though many questions remain.

Is it possible to have systemic sclerosis without skin?

It is possible to have Systemic Sclerosis without skin thickening (sine scleroderma), which is when a patient has the internal organ manifestations without the skin findings, but this is very rare.

What’s the difference between scleroderma and systemic sclerosis?

Introduction. Scleroderma is a group of conditions affecting approximately 300,000 people in the United States. 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).