What is systemic sclerosis (SSc)?

What is systemic sclerosis (SSc)?

Systemic sclerosis (SSc), also known as scleroderma, is a multi-system, autoimmune disease, characterised by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs, and production of auto-antibodies. MDM is an advisory board member for Boehringer-Ingelheim, Galapagos, Corbus, and Eicos.

How can we improve the management of systemic sclerosis?

Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis.

What is a baseline investigation in systemic sclerosis?

Baseline investigations form part of the essential early investigation of systemic sclerosis. Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: application of a latent linear trajectory model.

Is there a difference between systemic sclerosis and localised scleroderma?

The differential diagnosis of systemic sclerosis. Other scleroderma-like diseases are an important consideration that might require expert assessment and treatment. Localised forms of scleroderma are usually distinct from systemic sclerosis but, occasionally, there can be confusion, especially for generalised morphea.

Systemic sclerosis (SSc) is an autoimmune inflammatory condition. It results in potentially widespread fibrosis and vascular abnormalities, which can affect the skin, lungs, gastrointestinal tract, heart and kidneys. The skin becomes thickened and hard ( sclerotic ).

What are the possible complications of systemic sclerosis sine scleroderma?

Interstitial lung disease and scleroderma renal crisis are the major complications. In systemic sclerosis sine scleroderma, patients have systemic sclerosis–related antibodies and visceral manifestations of the disease but no skin tightening. Immunologic mechanisms and heredity (certain human leukocyte antigen subtypes) play a role in etiology.

What is the pathophysiology of systemic sclerosis?

Systemic sclerosis (SSc) is an autoimmune inflammatory condition. It results in potentially widespread fibrosis and vascular abnormalities, which can affect the skin, lungs, gastrointestinal tract, heart and kidneys. The skin becomes thickened and hard (sclerotic).

What is the difference between diffuse systemic sclerosis and limited systemic sclerosis?

Diffuse systemic sclerosis can affect skin all over the body and is more likely to affect the internal organs. Limited systemic sclerosis is a milder form that only affects the skin on the face, lower arms, hands, lower legs and feet. It normally progresses slowly. Limited systemic sclerosis can affect the lungs and the digestive system too.

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