Scleroderma (AKA systemic sclerosis) Flashcards
what is scleroderma?
aka systemic sclerosis (most patients with scleroderma have systemic sclerosis) (you can get a localised version of scleroderma that only effects the skin)
*hardening of the skin
an autoimmune inflammatory and fibrotic connective tissue disorder. cause is unclear. notably affects the skin
two main patterns:
Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
what is scleroderma?
aka systemic sclerosis (most patients with scleroderma have systemic sclerosis) (you can get a localised version of scleroderma that only effects the skin)
*hardening of the skin
an autoimmune inflammatory and fibrotic connective tissue disorder. cause is unclear. notably affects the skin
two main patterns:
Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
limited cutaneous systemic sclerosis
a more limited version of systemic sclerosis. used to be called “CREST syndrome”
C – Calcinosis R – Raynaud’s phenomenon E – oEsophageal dysmotility S – Sclerodactyly T – Telangiectasia
diffuse cutaneous systemic sclerosis
involves features of CREST plus many internal organs
Cardiovascular problems, particularly hypertension and coronary artery disease.
Lung problems, particularly pulmonary hypertension and pulmonary fibrosis.
Kidney problems, particularly glomerulonephritis and a condition called scleroderma renal crisis.
features of scleroderma
scleroderma- shiny, tight skin without the normal folds. hands and face
sclerodactyly: skin changes in the hands, tightens around joints and restrictive motion and function. fat pads are lost and skin can break and ulceate
telangiectasia: dilated small blood vessels on the skin. tiny veins dilated. fine, thready appearance
calcinosis: calcium deposition under skin (fingertips)
raynaurds: finger tips go white then blue caused by vasoconstriction
osteophageal dysmotility: connective tissue dysfucnction. GERD and oesophagitis.
systemic and pulmonary HTN
systemic and pulmonary arterial system
pulmonary fibrosis- dry cough, SOB
scleroderma renal crisis- HTN and renal failure
autoantibodies in systemic sclerosis
Antinuclear antibodies (ANA) are positive in most patients with systemic sclerosis. They are not specific to systemic sclerosis.
Anti-centromere antibodies are most associated with limited cutaneous systemic sclerosis.
Anti-Scl-70 antibodies are most associated with diffuse cutaneous systemic sclerosis. They are associated with more severe disease.
nail fold capillaroscopy
magnify and examine the area where the skin meats the fingernails at the base of the nailfold
examine the health of the peripheral capillaries
abnormal- avascular, micro haemorrhage
how is systemic sclerosis diagnosed?
clinical features
antibodies
nail fold capillaroscopy
management of systemic sclerosis
conservative: avoid smoking stretching emollinents avoid cold triggers physiotherapy and OT MDT
medical:
in diffuse - steroid and immunosupressant
nifedipine (raynaurds) PPI analgesia for joint pain antibiotics for skin infection antihypertensive (ACEi) treat pulmonary artery htn supportive mx for pulmonary fibrosis
limited cutaneous systemic sclerosis
a more limited version of systemic sclerosis. used to be called “CREST syndrome”
C – Calcinosis R – Raynaud’s phenomenon E – oEsophageal dysmotility S – Sclerodactyly T – Telangiectasia
diffuse cutaneous systemic sclerosis
involves features of CREST plus many internal organs
Cardiovascular problems, particularly hypertension and coronary artery disease.
Lung problems, particularly pulmonary hypertension and pulmonary fibrosis.
Kidney problems, particularly glomerulonephritis and a condition called scleroderma renal crisis.
features of scleroderma
scleroderma- shiny, tight skin without the normal folds. hands and face
sclerodactyly: skin changes in the hands, tightens around joints and restrictive motion and function. fat pads are lost and skin can break and ulceate
telangiectasia: dilated small blood vessels on the skin. tiny veins dilated. fine, thready appearance
calcinosis: calcium deposition under skin (fingertips)
raynaurds: finger tips go white then blue caused by vasoconstriction
osteophageal dysmotility: connective tissue dysfucnction. GERD and oesophagitis.
systemic and pulmonary HTN
systemic and pulmonary arterial system
pulmonary fibrosis- dry cough, SOB
scleroderma renal crisis- HTN and renal failure
autoantibodies in systemic sclerosis
Antinuclear antibodies (ANA) are positive in most patients with systemic sclerosis. They are not specific to systemic sclerosis.
Anti-centromere antibodies are most associated with limited cutaneous systemic sclerosis.
Anti-Scl-70 antibodies are most associated with diffuse cutaneous systemic sclerosis. They are associated with more severe disease.
nail fold capillaroscopy
magnify and examine the area where the skin meats the fingernails at the base of the nailfold
examine the health of the peripheral capillaries
abnormal- avascular, micro haemorrhage
how is systemic sclerosis diagnosed?
clinical features
antibodies
nail fold capillaroscopy
