What is systemic sclerosis?
skin thickening due to increased collagen
Is systemic sclerosis more common in women or men?
women
What other condition commonly (90%) occurs with systemic sclerosis?
Raynauds
What are the two types of SS?
Diffuse
Limited
What is diffuse SS?
involvment of trunk, face, arms, legs - WHOLE BODY
What is limited SS?
involvement of only below the elbows and knees
What is the common signs of SS?
calcium deposits in skin Raynauds pulmonary hypertension oesophageal dysfunction scelerodactyly - thickening of skin on fingers and hands talangiectasia
What is the pathophysiology behind SS?
immune system is acitvated causing endothelial cell activation and damage which activates fibroblasts.
These fibroblasts produce high levels of collagen
How is SS managed?
yearly ECHOs and PFTs
treat the comorbidities