Week 2 - systemic sclerosis

Question 1

what is systemic sclerosis?

Answer 1

systemic autoimmune disease associated with raynauds, fibrosis and subsequent atrophy of skin subcutaneous tissue

Question 2

how does systemic sclerosis cause changes in internal organs?

Answer 2

it causes excessive collagen deposition

Question 3

how does systemic sclerosis cause death generally?

Answer 3

lung and renal changes (e.g pulmonary hypertension)

Question 4

what cutaneous features are often present with systemic sclerosis?

Answer 4

raynauds
sclerodactyly
skin sclerosis
skin atrophy
telangiectasia
calcinosis in digits

Question 5

what are the 3 phases of cutaneous involvement?

Answer 5

1. oedematous
2. indurative
3. atrophic

Question 6

what are the major and minor cutaneous features of systemic sclerosis?

Answer 6

minor - central sclerosis of skin on arms, face and/or neck

major - sclerodactyly and atrophy of fingertips and bilateral lung fibrosis

Question 7

how can systemic sclerosis manifest in organs?

Answer 7

lungs - pulmonary fibrosis, pulmonary hypertension
renal - accelerated hypertension leads to renal crisis
GI - dysphagia, malabsorption, bacterial overgrowth
MSK - inflammatory arthritis

Question 8

limited systemic sclerosis?

Answer 8

limited to face, hands, forearms and feet (below knees/elbows)
late organ involvement
anti centromere antibody

Question 9

diffuse systemic sclerosis?

Answer 9

extends above knees and elbows
more rapid onset
faster organ involvement
anti Scl70 antibody associated

Question 10

how is systemic sclerosis diagnosed?

Answer 10

must have 1 major and 2 minor cutaneous features

auto antibodies - anti centromere and anti Scl70`

Question 11

how is systemic sclerosis treated?

Answer 11

no definitive treatment - treat symptoms
renal - ACE inhibitors
raynauds/digit ulcers - CCBs, iloprost, bosentan
GI - PPIs
ILD - immunosuppression - cyclophosphamide