Systemic sclerosis (SSc) is a chronic rheumatic disease with one of the highest mortalities within rheumatology. It is an autoimmune, fibro-inflammatory skin disease characterized by a distinctive triad of microvascular damage (vasculopathy), immune activation with autoimmunity, and generalized fibrosis of multiple organs, including the skin and lungs. The great heterogeneity of disease cause and presentation create considerable challenges in developing treatment and treating patients.
How many have Systemic Sclerosis?
The prevalence of SSc varies considerably around the world. Northern Europe and parts of Asia have a prevalence of >150 per million, whereas southern Europe, North America, and Australia have a higher prevalence of up to 443 per million. As with other autoimmune diseases, women are about six times likely to get SSc than men.
How is Systemic Sclerosis treated?
There is no cure or disease-modifying drug for SSc, but symptoms and inflammation can be treated using NSAIDs, steroids or biologics. The type of treatment depends on the symptoms and organs involved. Several novel drugs are in development targeting either the inflammatory and/or the fibrotic axis.
How is Systemic Sclerosis diagnosed?
A diagnostic blood-based test does not exist, and it is up to the individual physician to diagnose patients based on their symptoms. Skin fibrosis assessed by the modified Rodnan Skin Score (mRSS) and the presence of vascular damage, including Raynaud’s Phenomenon, is included in the diagnostic assessment. Furthermore, the presence of auto-antibodies and pulmonary involvement (either presented as interstitial lung disease or pulmonary arterial hypertension) is included for diagnose.
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