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Course 2-rheum > uncommon rheum diseases > Flashcards

uncommon rheum diseases Flashcards

1
Q

describe Raynaud’s phenomenon (vasospasm)

A

blanching and pain in fingers in cold, due to bad circulation/vessel shutdown. fingers turn white then purple

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2
Q

what are periungual changes?

A

dilated capillary loops in cuticles

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3
Q

what feature can be used to score the extent of scleroderma?

A

firm induration on skin of hand

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4
Q

how does firm induration differ from dactylitis?

A

involves all fingers, doesn’t fluctuate

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5
Q

what are the clinical features of scleroderma?

A
  • Raynaud’s
  • periungual changes
  • firm induration
  • GI involvement
  • lung involvement
  • anti-scl-70+/anti-centromere
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6
Q

what are the 3 phases of skin fibrosis in scleroderma?

A
  • early inflamed phase
  • tethered fibrotic phase
  • late softening phase
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7
Q

CREST syndrome is typical of (limited/diffuse) scleroderma

A

limited

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8
Q

what is the major morbidity in limited scleroderma?

A

vasculopathy (painful digital ulcers, pulmonary HTN)

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9
Q

which Ab is associated with diffuse scleroderma?

A

anti-scl-70

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10
Q

what is a critical feature of diffuse scleroderma?

A

early onset pulmonary fibrosis (50%)

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11
Q

the most common COD in scleroderma used to be ___ but is now___

A

renal failure

pulmonary HTN

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12
Q

what lead to the decreased COD due to renal failure in scleroderma?

A

ACE inhibitors

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13
Q

limited scleroderma has a (better/worse) prognosis than diffused

A

better

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14
Q

what are the principle therapies for scleroderma?

A
  • vasculopathy (Ca2+ channel inhibitor, phosphodiesterase, endothelin)
  • fibrogenesis (target fibroblasts and growth factors)
  • abnormal immune response (immune suppressives)
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15
Q

when is cyclophosphamide used in scleroderma?

A

severe Sx, pulmonary involvement

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16
Q

how can SCT help in scleroderma?

A

improve skin (regress tightening), help gut, improve survival

doesn’t help w organ involvement

17
Q

what is nintedanib and when is it used?

A

anti-fibrotic therapy, lung involvement in scleroderma (doesn’t reverse disease)

18
Q

what are gottron’s papules?

A

red sclay patches over knuckles and extensor surfaces (knee and hips)

19
Q

what are the clinical features of inflammatory myositis?

A 
-chronic inflammation of striated muscles
prox muscle weakness
-involvement of lungs, heart, GI, joints
-high CK, +anti-jo-1 on labs
\+/- rash in V/shawl sign on sun exposure
20
Q

the most common type of adult myositis is:

A

PM

21
Q

what is the difference between the pathogenesis of DM and PM?

A

DM-immune complex, specific Ab, microangiopathy, complement activation

PM-direct T cell mediated muscle attack

22
Q

what are the features of inclusion body myositis?

A

-M>F, onset 50s
-nonreponsive to prednisone
-asymmetrical muscle weakness
myopathic and neuropathic changes on EMG
-mononuclear cell infiltrates and amyloid vacuoles on biopsy (red-rimmed vacuoles)

23
Q

which type of myositis has the best prognosis? which type has the worst?

A

best: DM
worst: inclusion body/PM with anti-SRP

24
Q

what are the features of ANCA vasculitis?

A
  • wrist drop (radial nerve damage)
  • livedo reticularis (vessel blockage-white spots)
  • palpable purpura
  • bloody sputum (med emergency)
  • hema/proteinuria
  • +c-ANCA/PR3-ANCA
  • pulmonary nodules
  • mononeuritis multiplex
  • granulomatosis/polyangiitis

every system is adrift

25
Q

how does vasculitis happen?

A

vessel inflmaed due to cytokines, lumen is reduced, flow is reduced, lead to organ damage and necrosis

  • vessel weakening
  • vessel thinning and aneurysm
26
Q

giant cell arthritis is a (small/med/large) vessel vasculitis

A

large

27
Q

GCA happens in the ____ and can lead to____

A

carotid artery/aorta

blindness

28
Q

targeted therapy for GCA include Abs for this interleukin:

A

IL6

Course 2-rheum (7 decks)

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