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18 ORR > CT Diseases > Flashcards

CT Diseases Flashcards

1
Q

slicc classification criteria for sle

A

more/= 4 criteria (at least 1 clinical and 1 lab) OR biopsy proven sle with positive ana/anti-dna

for long standing disease

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2
Q

2019 acr eular criteria

A

ana >/= 1:80 on HEp-2 cell or equivalent positive test + additive crtieria (>/= 10 points)

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3
Q

clinical domain in acr

A

SOAP BRAIN MD

serositits
oral ulcer
arthritic
photosensitivity
blood disorders
renal disease
ana
immunophenomenon
neurological disorders
malar rish
discoid rash
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4
Q

immunologic criteria in acr

A

ana, anti-dsdna, anti-smith, anti-i1rnp, anti-ro/ss-a, anti-la/ss-b, anti-histone, anti-phospholipid

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5
Q

diagnostics for sle

A

read

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6
Q

all patients with sle should be started on __

A

hydroxychloroquine

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7
Q

other treatments for sle

A
  • corticosteroids
  • iv methylprednisolone pulses
  • oral prednisone
  • plasmapheresis ivig
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8
Q

what is apas

A

autoantibody-mediated acquired thrombophilia characterized by recurrent arterial or venous thrombosis and/or pregnancy morbidity

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9
Q

criteria for apas

A
  • venous/arterial thrombosis
  • ob complications
  • lupus anticoagulant
  • anticardiolipin antibodies
  • anti-b2 gylcoprotein -i antibodies

at least 1 clinical and 1 lab

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10
Q

treatment of apas

A
  • first event: warfarin (2.5-3.5 inr)

- pregnant: low molecular weight heparin with aspirin

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11
Q

what is scleroderma

A

excessive production and accumulation of collagen (fibrosis) in skin and internal organs and injuries to small arteries

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12
Q

clinical presentation of scleroderma

A
  • mucocutaneous telangiectasia
  • raynaud’s digital ischemic ulcers
  • pulmonary hypertension
  • pericarditis
  • skin induration
  • calcinosis cutis
  • hyperpigmentation
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13
Q

diagnosis of scleroderma

A

read

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14
Q

types of scleroderma

A
  • limited cutaneous: indolent onset, limited, slow, anticentromere
  • diffuse cutaneous: rapid onset, diffuse, antitopoisomerase I, anti rna polymerase III
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15
Q

hallmarks of scleroderma

A
  • widespread capillary loss
  • obliterative micoangiopathy
  • combined with fibrosis in the skin and internal organs
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16
Q

diagnosis for scleroderma

A
  • cbc
  • cpk
  • anas
  • antiscl 70, anti centromere ab
  • skin biopsy
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17
Q

treatment of scleroderma

A
  • vascular therapy (arb, clopidogrel, prostacyclin, sildenafil, bosentan)
  • immunosuppressive agents (methotrexate, aza, mycophenolate mofetil and cyclophosphamide)
  • hydroxycholoroquine
  • low dose prednisone
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18
Q

clinical presentation of sjorgen

A
  • ocular symptoms
  • dry mouth
  • ear
  • nose and paranasal sinuses
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19
Q

criteria for sjorgen syndrome

A
  • histopath: focal lymphocytic sialoadenitis
  • (+) anti-ro/ss-a
  • sicca ocular score >/= 5
  • schirmer test = 5 mm per 5 min
  • unstimulated whole salivary flow =0.1 ml/min

+ = more/= 4 and symptoms of ocular/oral dryness

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20
Q

pathogenesis of sjorgen

A
  • mutation of hla-dr and hla-dq
  • environmental factors
  • b-lymphocyte activation = exocrine gland dysfunction and destruction
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21
Q

diagnosis of sjorgen

A
  • esr and cbc
  • anas
  • rheumatoid factor
  • serum igg
  • creatinine clearance
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22
Q

treatment of sjorgen

A
  • dry eyes: artificial tears with cyclic amp or cyclosporine
  • dry mouth: inc water
  • parotid gland: antibiotics, assess for lymphoma
  • arthritis: hydroxychloroquine/ methrotrexate + low dose prednisolone
  • raynaud’s: vascular therapy or vasodilators
  • vasculitis: steroid / immunosuppression
23
Q

what is dermatomyositis

A
  • symmetric, proximal greater than distal weakness
  • heliotrope rash
  • gottron’s sign
  • gottron’s papules
  • v-sign
  • shawl sign
  • telangiectasia
  • sc ca deposits
24
Q

antibodies and labs for dermatomyositis

A
  • mda5, tif1, mi2, nxp2
  • increased muscle enzymes
  • emg: inflammatory myopathy
  • biopsy
25
Q

what is immune mediated necrotizing myopathy

A
  • acute or insidious onset of symmetric, proximal more than distal weakness
  • dysphagia, dysarthria, myalgia
26
Q

labs for immune mediated necrotizing myopathy

A
  • anti hmgcr, anti signal recognition particles
  • elevated ck
  • emg abnormal
  • biopsy: multifocal, necrotic and regenerating muscle fibers with paucity of inflammatory cells
27
Q

what is anti-synthetase syndrome

A
  • anti aminoacyl rna synthetases
  • anti jo1
  • elevated ck
  • perimysial fragmentation
28
Q

what is inclusion body myositis

A
  • slowly progressive weakness and muscle atrophy
  • distal muscles
  • asymmetric
  • dysphagia
  • cn-1a
  • increased cd8
  • biopsy: endomyismal inflammatory infiltrates
29
Q

treatment for inflammatory myopathies

A
  • steroids
  • methylprednisolone IV
  • ivig or rituximab
30
Q

large and medium vessel vasculitis

A

large:

  • takayasu arteritis
  • giant cell arteritis

medium:

  • polyarteritis nodosa
  • kawasaki disease
31
Q

small vessel vasculitis

A
  • immune: hsp, cryoglobulinemic vasculitis, hypocomplementemic urticaria vasculitis
  • anca: granuloma with polyangiitis, eosinophilic granulomatosis with polyangitis, mpa
32
Q

what is granulomatosis with polyangiitis (wegener’s)

A
  • upper and lower respi tracts with glomerulonephritis

- histo: necrotizing vasculitis of small arteries and veins, granuloma formation

33
Q

diagnosis of wegener’s

A
  • upper airway biopsy!!: granulomatous inflammation with necrosis
  • kidney biopsy: pauci-immune gn
  • immuno: antiproteinase 3 anca!
34
Q

what is eosinophilic granulomatosis with polyangiitis (churg-strauss)

A
  • lung predominant
  • histo: granulomatous retraction with eosinophils
  • s/sx: pulmo findings, mononeuritis multiplex peripheral neuropathy, allergic rhinitis and sinusitis
35
Q

diagnosis of churg-strauss

A
  • labs: eosinophilis (>1000), elevated esr and fibrinogen, anti-myeloperoxidase (mpo anca)
  • 4/6: eosinophilia, asthma, pulmo infiltrated non-fixed, neuropathy, paranasal sinus abnormalities, extravascular eosinophils
36
Q

what is microscopic polyangitis

A
  • necrotizing vasculitis with few or no immune complexes affecting small vessels
  • no granulomatous inflammation
  • biopsy: necrotizing inflammation, no granulomas or eosinophils
  • myeloperoxidase p-anca
37
Q

treatment for anca vasculitis

A
  • non organ threatening: methotrexate or mycophenolate mofetil with glucocorticoid
  • organ or life threatening: cyclophosphamide or rituximab with glucocorticoid
  • rapidly progressive renal failure of pulmo hemorrhage: plasmapheresis
38
Q

what is polyarteritis nodosa

A
  • necrotizing inflammation of small and medium sized muscular arteries
  • associated with hepatitis b
  • aneurysmal dilatation (upto 1 cm) are characteristic
39
Q

lab tests for polyarteritis nodosa

A
  • biopsy

- arteriogram: aneurysm

40
Q

criteria for pan

A

3/10

  • weight loss >/= 4 kg
  • livedo reticularis
  • testicular pain
  • myalgia or leg weakness
  • mono/poly neuropathy
  • dbp > 90
  • bun or crea elevated
  • hepbsag or ab
  • arteriogram with aneurym
  • pmn in biopsy
41
Q

treatment for polyarteritis nodosa

A
  • treat underlying cause
  • no major organ involvement: glucocorticoid +/- immunosuppressant
  • organ or life threatening, >5 ffs, rapid progression: glucocorticoid + immunosuppressant
42
Q

what is giant cell arteritis / temporal arteritis

A
  • panarteritis with inflammatory mononuclear cell infiltrates within the vessel wall with giant cell formation
  • proliferation of intima
  • fragmentation of internal elastic lamina
  • classic sx: fever + anemia + inc esr
43
Q

classification of gca

A

more/= 3:

  • > 50 yo
  • new onset headache
  • temporal artery abnormality
  • elevated esr >/= 50 mm/h
  • abnormal tab
44
Q

treatment for gca

A
  • prednisone
  • visual loss: methylprednisolone pulsing
  • low dose aspirin
  • dmards: methotrexate, azathioprine
  • tocilizuzmab
45
Q

what is takayasu’s arteritis

A
  • inflammatory stenotic at aortic arch an branches
  • absent pulses, hpn
  • labs: inc esr, mild anemia, inc immunoglobulin
46
Q

criteria for takayasu’s arteritis

A

3/6

  • = 40 yo
  • claudication of extremities
  • dec pulsation
  • <10 mmhg sbp
  • bruit at subclavian arteries
  • narrowing or occlusion of entire aorta
47
Q

treatment for takayasu’s arteritis

A
  • corticosteroids +/- methrotrexate, leflunomide, mycophenolate mofetil, azathioprine, tocilizumab
48
Q

what is iga vasculitis (henoch schonlein purpura)

A
  • anaphylactoid purpura
  • immune complex deposition (iga)
  • common with strep infection (aso titers)
  • can cause intussusception
49
Q

criteria for iga vasculitis

A

mandatory: purpura or petechiae with lower limb predominance

1/4:

  • skin biopsy: leukocytoclastic vasculitis / kidney biopsy: proliferative gn + iga deposits
  • diffuse abdominal pain with acute onset
  • arthritis or arthralgia
  • proteinuria / hematuria
50
Q

tx for iga vasculitis

A
  • self-limiting
  • oral corticosteroid
  • cyclophosphamide
51
Q

what is behcet’s syndrome

A
  • recurrent oral and genital ulceration + ocular involvement
  • systemic perivasculitis with early neutrophil infiltration and endothelial swelling
  • pseudoaneurysms
52
Q

criteria for behcet’s syndrome

A

recurrent oral ulceration + 2 of:

  • recurrent genital ulcers
  • ocular lesions
  • skin lesions
  • positive pathergy test
53
Q

tx for behcet’s syndrome

A
  • thalidomide
  • apremilast
  • colchicine
  • systemic glucocorticoid (prednisone)
  • azathioprine or cyclosporine

18 ORR (21 decks)

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