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Clin Med 4 > Rheumatology - Vasculitis - Smith > Flashcards

Rheumatology - Vasculitis - Smith Flashcards

1
Q

what are the 2 large vessel vasculitis diseases?

A

Takayasu arteritis and Giant Cell (Temporal) Arteritis

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2
Q

what are the 2 medium vessel vasculitis diseases?

A

Polyarteritis Nodosa and Kawasaki Disease

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3
Q

what are the 3 small vessel vasculitis diseases?

A

Microscopic Polyangitis, Granulomatosis w/polyangitis (Wegener’s granulomatosis), IgA Vasculitis (Henoch-Schonlein)

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4
Q

what is the variable vessel vasculitis?

A

Behcet Disease

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5
Q

who is Takayasu arteritis common in?

A

<50 y/o asian women

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6
Q

what large vessels does Takayasu Arteritis and Giant Cell (Temporal) Arteritis affect?

A

aorta and it’s major branches

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7
Q

systemic sx’s of Takayasu Arteritis?

A

elevations of ESR/CRP during the systemic phase

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8
Q

occlusive sx’s of Takayasu Arteritis?

A
  • Claudication
  • Pulselessness in carotid, brachial, radial, femoral, arteries (Must check these)
  • Bruits
  • Renal vascular HTN
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9
Q

what artery off of the aorta is commonly involved in Takayasu Arteritis? what happens?

A

Subclavian artery

-stenotic lesion proximal to origin of vertebral artery -> subclavian steal syndrome (10mmHg difference in BP b/w arms)

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10
Q

dx of Takayasu Arteritis?

A

Normochromic normocytic anemia of chronic disease

Elevated ESR, CRP

MRI or Angiogram of arteries

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11
Q

tx of Takayasu Arteritis?

A

STEROIDS

  • initial dose: 60 mg/day
  • taper over several weeks by 10mg/day to lowest dose (10mg/day), then take them off it

Other meds: MTX, Cyclophosphamide

Angioplasty or bypass grafts if irreversible arterial stenosis

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12
Q

who gets Giant Cell (Temporal) Arteritis?

A

> 50 y/o (avg 79/yo) women from Olmstead County, MN or Scandinavia

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13
Q

what artery is involved in Giant Cell Temporal Arteritis?

A

Temporal artery

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14
Q

what disease is Giant Cell Temporal Arteritis associated with?

A

Polymyalgia Rheumatica (PMR)

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15
Q

sx’s Giant Cell Temporal Arteritis?

A

Pt. >50 y/o with:

  • abrupt onset of visual disturbances
  • polymyalgia rheumatic sx’s (shoulder/hip stiffness)
  • unexplained fever or anemia
  • ***Jaw claudication (hurts when eating)
  • Renal vascular HTN
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16
Q

what is the complication of Giant Cell Temporal Arteritis?

A

Complication: VISUAL LOSS

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17
Q

criteria for Giant Cell Temporal Arteritis

A

CRITERIA (3/5)

  1. > 50 y/o
  2. New onset HA
  3. Tender or decr pulse of temporal artery
  4. ESR >50 mm/hr
  5. Bx shows necrotizing arteritis with mononuclear cells or granulomatous process with multinucleated giant cells
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18
Q

what is the GOLD STANDARD for dx of Giant Cell Temporal Arteritis?

A

Temporal artery bx

-***Pts with only sx’s of PMR do NOT require bx

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19
Q

scheduling of temporal artery bx should NEVER interfere with starting ___ b/c pt will become __

A

steroid tx b/c pt will become blind

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20
Q

what is the ESR level in Giant Cell Temporal Arteritis?

A

ESR >70 or >100

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21
Q

imaging for Giant Cell Temporal Arteritis

A

Angiography, Doppler U/S, MRI/A

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22
Q

tx for Giant Cell Temporal Arteritis NOT complicated by visual loss?

A

Prednisone 60mg/day, for 2 weeks, reduced to 50mg/day and then to 40mg/day at end of month (dose then gradually reduced each 1-2 weeks, but tx can last 12-16 months)

-Low-dose ASA 81mg PO/daily to reduce risk of visual loss, TIA, CVA

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23
Q

tx for Giant Cell Temporal Arteritis with visual loss?

A

IV METHYLPREDNISOLONE (1000mg IV daily x 3 days), then follow with PO Prednisone 60mg/daily

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24
Q

who gets Polyarteritis Nodosa?

A

Male, peak in 6th decade

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25
Q

what medium vessels do Polyarteritis Nodosa and Kawasaki disease affect?

A
  • splenic
  • renal
  • hepatic
  • mesenteric
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26
Q

what viruses are associated with the pathogenesis of Polyarteritis Nodosa?

A

HBV and HCV

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27
Q

which Vasculitis is NOT associated with ANCA/ANCA negative?

A

Polyarteritis Nodosa

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28
Q

which vasculitis SPARES the lungs, but affects the kidneys?

A

Polyarteritis Nodosa

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29
Q

sx’s of Polyarteritis Nodosa

A

HTN (renin mediated b/c affects kidneys)

Peripheral neuropathies (cog dysfunction, seizures, decreased alertness)

Mononeuropathy multiplex affecting nerves (radial, ulnar) w/ both motor and sensory deficits

skin of legs VERY painful and swollen

GI: abd pain (Early sx in w/meneteric arteritis)

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30
Q

what is an early sx of Polyarteritis Nodosa?

A

GI: abd pain in pts with mesenteric arteritis (bowel infarct rare, but surgical emergency)

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31
Q

criteria for Polyarteritis Nodosa?

A

CRITERIA (AT LEAST 3):

  1. Unexplained weight loss >4kg
  2. Livedo reticularis
  3. Testicular pain or tenderness
  4. Myalgias (excluding shoulder/hip), weakness of muscles, tenderness of leg muscles, or polyneuropathy
  5. New-onset diastolic >90mmHg
  6. Elevated serum BUN
  7. HBV antibody or antigen
  8. Characteristic arteriographic abnormalities
  9. Bx of small or medium-sized artery containing polymorphonuclear cells
  10. Mononeuropathy or polyneuropathy
32
Q

labs for Polyarteritis Nodosa?

A

Bx, ANCA negative, ANA (r/o Lupus) – negative, CXR to exclude diseases that affect the lungs, EKG

33
Q

tx for Polyarteritis Nodosa mild disease

A

Prednisone 1mg/kg (60-80mg) PO daily x4 weeks then taper

34
Q

tx for Polyarteritis Nodosa moderate-severe disease

A

Prednisone w/IV cyclophosphamide 600mg/kg q2 weeks for 3 doses

35
Q

tx for Polyarteritis Nodosa severe disease

A

Methylprednisolone 7-15mg/kg IV daily x3 days, then begin PO tx

36
Q

who is Kawasaki disease seen in?

A

infants and young children

37
Q

what MAIN vessel does Kawasaki disease affect? what does it cause if NOT treated?

A

Coronary arteries -> causes coronary artery aneurysms if NOT treated

38
Q

Key finding for Kawasaki disease?

A

Fever lasts >5 days w/tx

39
Q

oral mucous membrane changes sx’s of Kawasaki disease?

A
  • strawberry tongue
  • injected pharynx
  • fissured lips
40
Q

dx of Kawasaki disease?

A

Baseline ECHO obtained at dx (repeated at 2 & 6 weeks to evaluate for CA involvement)

Clinical dx

41
Q

tx for Kawasaki disease?

A

*IVIG 2g/kg administered as a single infusion over 8-12 hours (decreased rates of coronary artery aneurysm incidence)

Aspirin (30-50 mg/kg daily divided into 4 doses; once fever absent for 48 hrs, switch to low dose aspirin of 3-5 mg/kg/day, for antiplatelet effect)

Observe pt for 24hrs to confirm resolution of fever

42
Q

what small vessels do Microscopic Polyangitis, Wegener’s granulomatosis, IgA Vasculitis (Henoch-Schonlein) affect?

A

capillaries, venues, or arterioles

43
Q

what is the M/C cause of pulmonary renal syndrome?

A

Microscopic Polyangitis

44
Q

what is Pulmonary renal syndrome?

A

Caused by Microscopic Polyangitis

  • Necrotizing glomerulonephritis common
  • Pulmonary capillaritis occurs
  • Granulomatous inflammation is absent
45
Q

sx’s of Microscopic Polyangitis

A
  • Pulmonary hemorrhage, Interstitial lung fibrosis (DOESN’T SPARE THE LUNGS)
  • Splinter hemorrhages (b/c small vessels)
46
Q

diagnostics for Microscopic Polyangitis

A

***ANCA positive

UA: hematuria, proteinuria, RBC casts

Chest Ct

47
Q

tx for Microscopic Polyangitis

A

Cyclophosphamide PO plus Prednisone 1mg/kg (max 60-80mg) PO daily
-continue these meds for 3-6 months

48
Q

what is the CLASSIC TRIAD of Wegener’s granulomatosis?

A

(1) Upper Resp/Nose sx’s
(2) Lower Resp. Tract sx’s
(3) Glomerulonephritis

49
Q

what are the Upper Resp/Nose sx’s of Wegener’s?

A
  • Saddle-ridge deformity (cartilage in nose necrotizes and collapses); perforation of nasal septum
  • Nasal congestion, sinusitis, otitis media, mastoiditis
50
Q

which vasculitis has saddle-ridge deformity of nose?

A

Wegener’s

51
Q

what are the Lower Resp sx’s of Wegener’s?

A

cough, dyspnea, hemoptysis

52
Q

what is the glomerulonephritis sx of Wegener’s?

A

hematuria, rapidly progressing

53
Q

Triad of eye sx’s of Wegener’s?

A

(1) Proptosis
(2) Ptosis (drooping of upper eyelid)
(3) Ophthalmoplegia (paralysis of muscles w/in surrounding eye)

54
Q

labs of Wegener’s

A

Serum:

  • slight anemia
  • mild leukocytosis
  • elevated acute phase reactants (ESR, CRP)

Urine (if kidney involvement):

  • proteinuria
  • sediment has red cells
  • red cell casts
55
Q

what will Chest CT of Wegener’s show?

A

-infiltrates, nodules, masses, cavities

56
Q

imaging for Wegener’s?

57
Q

tx for Wegener’s?

A

Cyclophosphamide PO plus Prednisone 1mg/kg (max 60-80mg) PO daily

-immune suppressor w/prednisone

58
Q

who gets IgA Vasculitis (Henoch-Schonlein)?

A

Children 5-7 y/o (M/C systemic vasculitis in children)

59
Q

when does IgA Vasculitis (Henoch-Schonlein) occur?

A

Occurs in FALL, WINTER SPRING (SUMMER OFF – LIKE KIDS)

60
Q

what is IgA Vasculitis (Henoch-Schonlein)?

A

VASCULITIS W/IgA-1 DOMINANT IMMUNE DEPOSITS

61
Q

what’s the CLASSIC TETRAD of IgA Vasculitis (Henoch Schonlein)?

A
  1. Palpable Purpura
  2. Arthralgia/arthritis (2nd M/C sx)
  3. Abdominal pain: colicky and GI bleeding
  4. Renal disease
62
Q

where are the palpable purpura for IgA Vasculitis (Henoch Schonlein)?

A

lower extremities (M/C)

63
Q

what is the 2nd most common sx of IgA Vasculitis (Henoch Schonlein)?

A

arthralgia/arthritis

64
Q

where do arthralgia/arthritis occur in IgA Vasculitis (Henoch Schonlein)? what are they like?

A

lower extremity large joints (knees and ankles)

-non-deforming, oligoarticular, transient

65
Q

what is the M/C abd pain of IgA Vasculitis (Henoch Schonlein)?

A

Intussusception

66
Q

what is the M/C renal disease of IgA Vasculitis (Henoch Schonlein)?

A

hematuria with/without RBC casts and no/mild proteinuria

67
Q

what is the EULAR criteria for IgA Vasculitis (Henoch Schonlein)?

A

-MANDATORY: Purpura (palpable and in clusters)

Pts MUST have ≥1 of the following:

  1. Abdominal pain (diffuse, w/acute-onset)
  2. Arthritis or arthralgia (acute-onset)
  3. Renal involvement (proteinuria, hematuria)
  4. Leukocytoclastic vasculitis or proliferative glomerulonephritis, with predominant IgA deposition
68
Q

tx of IgA Vasculitis (Henoch Schonlein)?

A
  • Oral hydration
  • Rest

Symptomatic relief of joint and abdominal pain
-NSAIDS – naproxen 10-20mg/kg daily-max dose 750 2x/day

Severe abdominal pain that interferes w/PO intake
-Prednisone 1-2 mg/kg PO daily, max dose 60mg daily

***Complication of renal disease – follow-up with pt for screening of urinary abnormalities and elevated BP every 3 months

69
Q

what’s the TRIAD of Behcet disease?

A

Aphthous oral ulcers, genital lesions, and recurrent eye inflammation (ant/post uveitis)

70
Q

what do aphthous ulcers feel like for pt?

71
Q

genital ulcers of Behcet disease? (male and female)

A

Male: painful genital lesions that form on the scrotum, similar to to oral lesions, but deeper

Female: painful genital ulcers that develop on the vulva

72
Q

sx’s of uveitis (anterior and posterior)

A
  • Anterior: ***pain, blurry vision, light sensitivity, tearing, or redness of eye
  • Posterior: ***painless, so more dangerous and vision-threatening b/c often causes fewer sx’s while damaging retina
73
Q

lung sx’s of Behcet disease?

A

-Aneurysms of arteries in the lung, rupture which may lead to massive, life-threatening lung hemorrhage

74
Q

GI sx’s of Behcet disease?

A
  • Ulcerations may occur anywhere in the GIT from the mouth to the anus
  • Terminal ileum and cecum are common sites (hard to distinguish from Crohn’s disease)
75
Q

tx of Behcet disease?

A
  • STEROIDS*
  • Initial glucocorticoid dose: 60mg/day  tx for vasculitis
  • Colchicine 0.6mg PO tid and thalidomide 100mg PO daily for the Aphthous ulcers
  • Cyclophosphamide for severe ocular or CNS sx’s

Clin Med 4 (11 decks)

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