Week 2 - systemic sclerosis Flashcards

1
Q

what is systemic sclerosis?

A

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

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2
Q

how does systemic sclerosis cause changes in internal organs?

A

it causes excessive collagen deposition

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3
Q

how does systemic sclerosis cause death generally?

A

lung and renal changes (e.g pulmonary hypertension)

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4
Q

what cutaneous features are often present with systemic sclerosis?

A
raynauds
sclerodactyly
skin sclerosis
skin atrophy
telangiectasia
calcinosis in digits
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5
Q

what are the 3 phases of cutaneous involvement?

A
  1. oedematous
  2. indurative
  3. atrophic
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6
Q

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

A

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

major - sclerodactyly and atrophy of fingertips and bilateral lung fibrosis

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7
Q

how can systemic sclerosis manifest in organs?

A

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

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8
Q

limited systemic sclerosis?

A

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

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9
Q

diffuse systemic sclerosis?

A

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

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10
Q

how is systemic sclerosis diagnosed?

A

must have 1 major and 2 minor cutaneous features

auto antibodies - anti centromere and anti Scl70`

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11
Q

how is systemic sclerosis treated?

A

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

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