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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
what medium vessels do Polyarteritis Nodosa and Kawasaki disease affect?
- splenic - renal - hepatic - mesenteric
26
what viruses are associated with the pathogenesis of Polyarteritis Nodosa?
HBV and HCV
27
which Vasculitis is NOT associated with ANCA/ANCA negative?
Polyarteritis Nodosa
28
which vasculitis SPARES the lungs, but affects the kidneys?
Polyarteritis Nodosa
29
sx's of Polyarteritis Nodosa
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)
30
what is an early sx of Polyarteritis Nodosa?
GI: abd pain in pts with mesenteric arteritis (bowel infarct rare, but surgical emergency)
31
criteria for Polyarteritis Nodosa?
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
labs for Polyarteritis Nodosa?
Bx, ANCA negative, ANA (r/o Lupus) – negative, CXR to exclude diseases that affect the lungs, EKG
33
tx for Polyarteritis Nodosa mild disease
Prednisone 1mg/kg (60-80mg) PO daily x4 weeks then taper
34
tx for Polyarteritis Nodosa moderate-severe disease
Prednisone w/IV cyclophosphamide 600mg/kg q2 weeks for 3 doses
35
tx for Polyarteritis Nodosa severe disease
Methylprednisolone 7-15mg/kg IV daily x3 days, then begin PO tx
36
who is Kawasaki disease seen in?
infants and young children
37
what MAIN vessel does Kawasaki disease affect? what does it cause if NOT treated?
Coronary arteries -> causes coronary artery aneurysms if NOT treated
38
Key finding for Kawasaki disease?
Fever lasts >5 days w/tx
39
oral mucous membrane changes sx's of Kawasaki disease?
- strawberry tongue - injected pharynx - fissured lips
40
dx of Kawasaki disease?
Baseline ECHO obtained at dx (repeated at 2 & 6 weeks to evaluate for CA involvement) Clinical dx
41
tx for Kawasaki disease?
*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
what small vessels do Microscopic Polyangitis, Wegener's granulomatosis, IgA Vasculitis (Henoch-Schonlein) affect?
capillaries, venues, or arterioles
43
what is the M/C cause of pulmonary renal syndrome?
Microscopic Polyangitis
44
what is Pulmonary renal syndrome?
Caused by Microscopic Polyangitis - Necrotizing glomerulonephritis common - Pulmonary capillaritis occurs - Granulomatous inflammation is absent
45
sx's of Microscopic Polyangitis
- Pulmonary hemorrhage, Interstitial lung fibrosis (DOESN’T SPARE THE LUNGS) - Splinter hemorrhages (b/c small vessels)
46
diagnostics for Microscopic Polyangitis
***ANCA positive UA: hematuria, proteinuria, RBC casts Chest Ct
47
tx for Microscopic Polyangitis
Cyclophosphamide PO plus Prednisone 1mg/kg (max 60-80mg) PO daily -continue these meds for 3-6 months
48
what is the CLASSIC TRIAD of Wegener's granulomatosis?
(1) Upper Resp/Nose sx's (2) Lower Resp. Tract sx's (3) Glomerulonephritis
49
what are the Upper Resp/Nose sx's of Wegener's?
- Saddle-ridge deformity (cartilage in nose necrotizes and collapses); perforation of nasal septum - Nasal congestion, sinusitis, otitis media, mastoiditis
50
which vasculitis has saddle-ridge deformity of nose?
Wegener's
51
what are the Lower Resp sx's of Wegener's?
cough, dyspnea, hemoptysis
52
what is the glomerulonephritis sx of Wegener's?
hematuria, rapidly progressing
53
Triad of eye sx's of Wegener's?
(1) Proptosis (2) Ptosis (drooping of upper eyelid) (3) Ophthalmoplegia (paralysis of muscles w/in surrounding eye)
54
labs of Wegener's
Serum: - slight anemia - mild leukocytosis - elevated acute phase reactants (ESR, CRP) Urine (if kidney involvement): - proteinuria - sediment has red cells - red cell casts
55
what will Chest CT of Wegener's show?
-infiltrates, nodules, masses, cavities
56
imaging for Wegener's?
Chest CT
57
tx for Wegener's?
Cyclophosphamide PO plus Prednisone 1mg/kg (max 60-80mg) PO daily -immune suppressor w/prednisone
58
who gets IgA Vasculitis (Henoch-Schonlein)?
Children 5-7 y/o (M/C systemic vasculitis in children)
59
when does IgA Vasculitis (Henoch-Schonlein) occur?
Occurs in FALL, WINTER SPRING (SUMMER OFF – LIKE KIDS)
60
what is IgA Vasculitis (Henoch-Schonlein)?
VASCULITIS W/IgA-1 DOMINANT IMMUNE DEPOSITS
61
what's the CLASSIC TETRAD of IgA Vasculitis (Henoch Schonlein)?
1. Palpable Purpura 2. Arthralgia/arthritis (2nd M/C sx) 3. Abdominal pain: colicky and GI bleeding 4. Renal disease
62
where are the palpable purpura for IgA Vasculitis (Henoch Schonlein)?
lower extremities (M/C)
63
what is the 2nd most common sx of IgA Vasculitis (Henoch Schonlein)?
arthralgia/arthritis
64
where do arthralgia/arthritis occur in IgA Vasculitis (Henoch Schonlein)? what are they like?
lower extremity large joints (knees and ankles) -non-deforming, oligoarticular, transient
65
what is the M/C abd pain of IgA Vasculitis (Henoch Schonlein)?
Intussusception
66
what is the M/C renal disease of IgA Vasculitis (Henoch Schonlein)?
hematuria with/without RBC casts and no/mild proteinuria
67
what is the EULAR criteria for IgA Vasculitis (Henoch Schonlein)?
-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
tx of IgA Vasculitis (Henoch Schonlein)?
- 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
what's the TRIAD of Behcet disease?
Aphthous oral ulcers, genital lesions, and recurrent eye inflammation (ant/post uveitis)
70
what do aphthous ulcers feel like for pt?
PAINFUL
71
genital ulcers of Behcet disease? (male and female)
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
sx's of uveitis (anterior and posterior)
- 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
lung sx's of Behcet disease?
-Aneurysms of arteries in the lung, rupture which may lead to massive, life-threatening lung hemorrhage
74
GI sx's of Behcet disease?
- 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
tx of Behcet disease?
* **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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