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MULTISYSTEM DISEASE > scleroderma > Flashcards

scleroderma Flashcards

1
Q

localised scleroderma (skin&dermis)

A

morphea
linear morphea
en coupe de Sabre

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2
Q

systemic sclerosis

A

limited systemic sclerosis

diffuse systemic sclerosis

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3
Q

pathophysiology of systemic sclerosis

A

imbalance between local vasodilators and vaso constrictors, activated endothelium, adhesion of inflammatory cells.

platelet aggregation, fibrin deposition and intravascular thrombosis and a fibrotic reaction due to proliferation of myofibroblasts

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4
Q

features of vascular dysfunction

A
  • digital ulceration & pulp atrophy
  • pulmonary vessels stiffer - PAH
  • renal vessels stiffer - systemic HTN
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5
Q

fibrosis features

A
  • fibroblast production of collagen type I & III becomes persistent
  • tissue becomes thicker
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6
Q

features of limited cutaneous systemic sclerosis

A

skin involvement is limited to hands, face, feet and forearms, BEAK nose + small mouth, starts with raynauds (CREST)

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7
Q

antibody in diffuse systemic sclerosis

A

ANAs involved:

scl-70 (highly specific) and RNA polymerase III

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8
Q

signs of systemic sclerosis

A
  • small mouth, beak nose
  • raynauds
  • digital ulceration
  • diffuse skin thickening
  • interstitial lung disease
  • gastrointestinal
  • renal
  • cardiac
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9
Q

investigations in systemic sclerosis

A
  • FBC (normocytic normochromic anaemia_
  • U+E
  • auto antibodies (anti-centromeres in 70%, DcSSC)
  • CXR (calcinassi of hands)
  • barium swallowed (oesophageal motility)
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10
Q

signs of limited sclerosis

A

CREST

  • calcinosis
  • raynauds
  • oesphageal - , acid reflux, gi general
  • scleroactyl - thickening and tightening of skin, distal
  • telangectasia
  • scleroderma of neck and face
  • pulmonary HTN
  • fibrotic lung disease
  • mid gut disease
  • renal crisis (scl-70/RNA polymerase III)
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11
Q

ANAs involved

A

anti-centromere

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12
Q

symptomatic treatment of systemic sclerosis

A

lifestyle advice
vasodilatory drugs (preventing digital ulcers/treating PAH)
PPIs

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13
Q

Reno-protective drugs in systemic sclerosis

A

ACEi

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14
Q

immunosuppression

A

cyclophosphamide/mycophenolate/methotrexate

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15
Q

anti-centromere antibody (ACA)

A 
limited cutaneous disease
microvascular changes (telangectaisa)
PAH
GI disease
raynauds
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16
Q

anti-topoisomerase (Scl-70)

A

diffuse cutaneous disease

ILD

17
Q

anti-RNA polymerase III

A

diffuse cutaneous disease, renal involvement

MULTISYSTEM DISEASE (13 decks)

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