Epidemiologi ved systemisk sklerose (sklerodermi)
DOI:
https://doi.org/10.5324/nje.v18i1.66Abstract
TSystemic sclerosis is an inflammatory rheumatic disease affecting small arteries, microvessels and connective tissue. Pathological findings include fibrosis, vasculopathy and production of autoantibodies. Clinically, two distinct subgroups can be identified. Limited cutaneous systemic sclerosis is associated with anticentromere antibodies and the skin lesion never extends proximally to the elbows and knees. Diffuse cutaneous systemic sclerosis exhibits production of anti topoisomerase I antibodies (anti Scl 70)and skin lesion affects also proximal parts of the body. The annual incidence is estimated to 0.2-1.9 per 100 000 inhabitants. The prevalence is approximately 7-15 cases per 100 000 inhabitants. Mortality is significantly increased, mainly due to pulmonal arterial hypertension and interstitial lung disease. A possible increased risk of contracting lung cancer is still debated
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