rheum systemic sclerosis Flashcards
what is systemic sclerosis
chronic disease characterized by abnormal connective tissue growth resulting in hardening and thickening of skin and often inner organs
what are the two types of systemic sclerosis and which is more common
limited and diffuse
limited more common
clinical presentation of limited ssc
CREST syndrome
calcinosis cutis
raynaud phoenomena(vasospasm of small vessels of fingers which often occurs due to cold conditions)
esophageal dysmotility which can lead to dysphagia and gerd which can further lead to barret esophagus
sclerodactyly which is fibrotic thickening of the skin of the fingers and hands. often initially edematous then fibrotic with waxy appeance and has red brown colour
telangeictasia
plus other cutaneous features such as ulcers
clinical presentation of diffuse ssc
widespread disease and cutaneous features
extracutaneous features affecting esophagus causing esophaela dysmotility
lungs interstiial lung disease
heart fibrosis pericarditis myocarditis
renal fibrotic disease causing thickening of renal arteriolar wall leading to decreased blood flow
which ntibody specific to to limited scc and diffuse scc
limited : anti centromere antibody
diffuse: anti scl 70 antibody and anti rna polymerase 3