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21 Cards in this Set

  • Front
  • Back
What are the two large vessel vasculitides?

Two medium vessel vasculitides?
Large: Takayasu's and temporal arteritis

Medium: PAN and Kawasaki's
Small vessel vasculitides are divided into two groups. Give the groups and the individual diseases.
Immune-complex:
Hypersensitivity vasculitis
HSP
Cryoglobulinemic

ANCA (paucimmune)
Wegener's
Churg-Strauss
Microscopic polyangiitis
What vasculitis is suggested by:

sinus involvement?

bloody diarrhea?
sinus: Wegener's; also Churg-Strauss

bloody diarrhea: HSP
What vasculitis is suggested by:

testicular tenderness?

mononeuritis multiplex?
testicular tenderness: PAN

mononeuritis: PAN, Wegener's, Churg-Strauss
Why is albumin low in vasculitis?
Albumin in a NEGATIVE acute phase reactant, which goes down with systemic inflammation.
What test is highly specific for Wegener's?
c-ANCA, against proteinase 3
You find positive cryoglobulinemia in labs. What next?
rule out hepatitis C.
What vasculitides have low complement?
Cryoglobulinemia, nearly always

PAN in 25% of cases

occasionally in hypersensitivity vasculitis.
p-ANCA against myeolperoxidase suggests what two vasculitides?
Churg-Strauss, microscopic polyangiitis
What if you have p-ANCA but not against myeloperoxidase?
Consider inflammatory bowel disease, infection, and other systemic inflammatory conditions besides vasculitis.
What are the most sensitive exam findings for temporal arteritis?
scalp tenderness
reduction in TA pulse + tenderness

visual abnormalities are less sensitive, but must be acted upon
always check for jaw claudication too
What is the treatment for temporal arteriitis?
high dose prednisone 20 mg 3 x per day
treatment continues for SIX MONTHS at least, and low dose maintenance may be needed after that; tapering usually begins after ESR is normalized (down 5 mg Q 1-2 weeks to 30 mg once a day)
Alternate day regimens DO NOT WORK
Epidemiology of Takayasu's arteriitis
girls and young women 10-30
Clinical features of Takayasu's arteriitis
Bruits #1 (80%)
upper extremity claudication
decreased upper extremity pulses
asymmetric blood pressure

note that there are nonspecific symptoms in the early stage: fever, arthralgia, weight loss; patients under 20 will often present in this stage.
Treatment of Takayasu's arterittis

Treatment for relapsing Takayasu's
Same as for temporal arteriitis:
high dose prednisone, 20 mg 3 times per day

Methotrexate for relapsing condition; also mycophenolate, cyclophosphamide
What are the most common organ symptoms of PAN?
>50%: focal necrotizing glomerulonephritis
severe hypertension (due to GN)
mononeuritis muliplex

50%: myalgia, arthralgia
palpable purpura, livedo

<30% liver function abnormalities
abdominal pain
testicular pain

more rare:
T/F: PAN will usually show thrombocytosis and low albumin.
true.
How is PAN diagnosed?
tissue biopsy if possible (sural nerve, liver, rectum, testicle)
if affected area cannot be biopsied, visceral angiogram
What disease conditions are associated with PAN?
HEPATITIS B
Other viruses: CMV, EBV, parvovirus
Autoimmune disorders: RA, SLE, PM-DM
medications: sulfa, allopurinol
Hairy cell leukemia
How is PAN treated?
if no Hepatitis B:
high dose corticosteroids (prednisone, or if severe, methylprednisone)
Cyclophosphamide or other cytotoxics are added if there is major organ involvement

if Hepatitis B, steroids alone allow virus to persist, so add:
plasmapheresis to remove immune complexes,
antivirals against hep B: interferon-2a, lamivudine
Can Buerger's disease affect patients who chew tobacco or smoke pipes?
yes.