Week 2 - systemic sclerosis Flashcards
what is systemic sclerosis?
systemic autoimmune disease associated with raynauds, fibrosis and subsequent atrophy of skin subcutaneous tissue
how does systemic sclerosis cause changes in internal organs?
it causes excessive collagen deposition
how does systemic sclerosis cause death generally?
lung and renal changes (e.g pulmonary hypertension)
what cutaneous features are often present with systemic sclerosis?
raynauds sclerodactyly skin sclerosis skin atrophy telangiectasia calcinosis in digits
what are the 3 phases of cutaneous involvement?
- oedematous
- indurative
- atrophic
what are the major and minor cutaneous features of systemic sclerosis?
minor - central sclerosis of skin on arms, face and/or neck
major - sclerodactyly and atrophy of fingertips and bilateral lung fibrosis
how can systemic sclerosis manifest in organs?
lungs - pulmonary fibrosis, pulmonary hypertension
renal - accelerated hypertension leads to renal crisis
GI - dysphagia, malabsorption, bacterial overgrowth
MSK - inflammatory arthritis
limited systemic sclerosis?
limited to face, hands, forearms and feet (below knees/elbows)
late organ involvement
anti centromere antibody
diffuse systemic sclerosis?
extends above knees and elbows
more rapid onset
faster organ involvement
anti Scl70 antibody associated
how is systemic sclerosis diagnosed?
must have 1 major and 2 minor cutaneous features
auto antibodies - anti centromere and anti Scl70`
how is systemic sclerosis treated?
no definitive treatment - treat symptoms
renal - ACE inhibitors
raynauds/digit ulcers - CCBs, iloprost, bosentan
GI - PPIs
ILD - immunosuppression - cyclophosphamide