Connective Tissue Diseases: Systemic Sclerosis Flashcards
what is there a triad of
autoimmunity, fibrosis and vascular endothelial changes
fibrosis causes subsequent atrophy of the skin and subcutaenous tissue
what is usually responsible for death
renal and lung changes
pulmonary hypertension - 12% deaths
what is a common early finding and is unlikely to have SS without
Raynaud’s
what are the 3 phases of cutaenous involvement
oedematous
indurative
atrophic
- skin becomes thickened and tight
major features
centrally located skin sclerosis that affects face arms and neck
minor features
- Sclerodactyly (skin thickening) of the fingertips and atrophy of the underlying tissues
- Internal organ fibrosis and/or vascular damage (lungs, heart, G.I. tract ± kidneys)
what features are needed to make a diagnosis
1 major and 2 minors
what happens when the skin of the face is affected
pinching of skin of nose - beaking
what can be seen on the face along with beaking of the nose
telangectasia
calcinosis
Calcinosis (skin can breakdown and discharge chalky material (calcium)).
lung involvement
- Pulmonary hypertension, pulmonary fibrosis and accelerated hypertension leading to scleroderma renal crisis (10% patients).
renal involvement
Scleroderma renal crisis is characterised by accelerated hypertension, rapidly progressive kidney failure and proteinuria.
GI involvement
- Gut involvement leading to dysphagia, dyspepsia, malabsorption and bacterial overgrowth of small bowel
- Importantly oesophageal dysmotility
- Lower G.I.: bloating, distension, nausea, vomiting, pain, diarrhoea, constipation
what can gut invovlement lead to
oesophageal dysmotility
limited
- formerly CREST syndrome
- skin involvement confined to face, hands and forearm
- organ involvement tends to occur later
