vasculitis syndromes

Question 1

what is anca?

Answer 1

antibody directed against certain proteins of the cytoplasmic granules of neutrophils and monocytes (high in Wegener’s and microscopic polyangiitis)

Question 2

vasculitis syndromes for which immune complex formation is primary source of damage

Answer 2

Henoch-Schonlein purpura, vasculitis ass. w/ collagen vascular diseases, serum sickness and cutaneous vasculitis syndromes, hepatitis C ass. cryoglobulinemic vasculitis, polyarteritis nodosa-like vasculitis ass. w/ hep B

Question 3

vasculitis syndromes ass. w/ ANCA

Answer 3

granulomatosis w/ polyangiitis (wegener’s), Churg-Strauss syndrome, microscopic polyangiitis

Question 4

vasculitis syndromes ass. w/ pathogenic T cell responses and granuloma formation

Answer 4

GCA, Takayasu’s, granulomatosis w/ polyangiitis, Churg-Strauss syndrome

Question 5

general signs of vasculitis

Answer 5

palpable purpura, pulmonary infiltrates, microscopic hematuria, chronic inflammatory sinusitis, mononeuritis multiplex, unexplained ischemic events, and glomerulonephritis w/ e/o multisystem disease

Question 6

conditions that can mimic vasculitis

Answer 6

infections (bacterial endocarditis, DGI, pulmonary histoplasmosis, occidiodomycosis, syphilis, lyme, RMSF, whipple’s)
coagulopathies/thrombotic microangiopathies (anti-phospholipid antibody syndrome, TTP)
neoplasma (atrial myxoma, lymphoma, carcinomatosis)
drug toxicity (cocaine, amphetamine, ergot alkaloids, methysergide, arsenic)
sarcoidosis
atheroembolic disease
antiglomerular basement membrane disease (Goodpasture’s)
amyloidosis
migraine

Question 7

definition of granulomatosis w/ polyangiitis

Answer 7

(wegener’s)

granulomatous vasculitis of the upper and lower respiratory tracts w/ glomerulonephritis

Question 8

pathology of wegener’s

Answer 8

necrotizing vasculitis of small arteries and veins together w/ granuloma formation

Question 9

upper airway involvement in wegener’s

Answer 9

paranasal sinus pain and drainage
purulent or bloody nasal discharge
nasal septal perforation

Question 10

lower airway involvement in wegener’s

Answer 10

asymptomatic iniltrates

cough, hemoptysis, dyspnea, chest discomfort

Question 11

eye involvement in wegener’s

Answer 11

half of patients

conjunctivitis, dacrocystitis, episcleritis, etc

Question 12

renal disease in wegener’s

Answer 12

accounts for most of the mortality
proteinuria, hematuria, RBC casts
renal failure if not addressed

Question 13

labs in wegener’s

Answer 13

high esr
anemia, leukocytosis
mild hypergammaglobulinemia (high IgA)
90% have + antiproteinase-3 ANCA

Question 14

diagnosis of wegener’s

Answer 14

biopsy w, necrotizing granulomatous vasculitis

Question 15

side effects of cyclophosphamide

Answer 15

BM suppression, cystitis, bladder carcinoma, teratogenicity, GI intolerance, opportunistic infections, pulmonary fibrosis

Question 16

side effects of methotrexate

Answer 16

GI intolerance, stomatitis, BM suppression, hepatotoxicity (can lead to fibrosis or cirrhosis), pneumonitis, teratogenicity, opportunistic infections

Question 17

side effects of azathioprine

Answer 17

GI intolerance, BM suppression, hepatotoxicity, opportunistic infections, hypersensitivity

Question 18

treatment of wegener’s

Answer 18

cyclophosphamide = major

also, methotrexate and steroids

Question 19

definition of microscopic polyangiitis

Answer 19

necrotizing vasculitis w/ few or no immune complexes
glomerulonephritis, pulmonary capillaritis
absence of granulomatous inflammation differentiates it from wegener’s
ANCA+

Question 20

signs of microscopic polyangiitis

Answer 20

glomerulonephritis (can be fatal)
hemoptysis (sign of alveolar hemorrhage)
mononeuritis multiplex, GI and cutaneous vasculitis
if upper airway disease present, likely wegener’s

Question 21

labs in microscopic polyangiitis

Answer 21

75% + for antimyeloperoxidase ANCA antibodies

high esr, anemia leukocytosis, thrombocytosis

Question 22

pathophys of vasculitis / immune complexes

Answer 22

immune complexes deposit in vessels, auto-immune reaction, and then the vessel lumen is compromised with ischemic changes in the tissues supplied by the involved vessel

Question 23

diagnosis of microscopic polyangiitis

Answer 23

histologic e/o vasculitis or pauci-immune glomerulonpheritis w/ the right clinical features

Question 24

definition of churg-strauss

Answer 24

also called allergic angiitis and granulomatosis

asthma, peripheral and tissue eosinophilia, extravascular granuloma formation, and vasculitis of multiple organ systems

Question 25

signs of Churg-Strauss

Answer 25

constitutional
severe asthmatic attacks, pulmonary infiltrates
mononeuritis multiplex
allergic rhinitis and sinusitis
heart disease
skin disease (purpura, cutaneous and subcutaneous nodules)

Question 26

labs in Churg-Strauss

Answer 26

eosinophilia
high ES, fibrinogen, or alpha2 globulins
ANCA, usually anti-myeloperoxidase

Question 27

treatment of churg-strauss

Answer 27

steroids

cyclophosphamide

Question 28

definition of polyarteritis nodosa

Answer 28

multisystem, necrotizing vasculitis of small and medium sized muscular arteries in which involvement ofthe renal and visceral arteries is characteristic

Question 29

classic pathology of polyarteritis nodosa

Answer 29

kidney pathology: arteritis w/o glomerulonephritis

Question 30

giant cell arteritis

Answer 30

inflammation of medium and large sized arteries; usually involves the branches of the carotid (esp temporal artery)

Question 31

what is GCA associated with?

Answer 31

polymyalgia rheumatica (seen in 40-50% of patients with GCA)

Question 32

signs of polymyalgia rheumatica

Answer 32

stiffness, aching and pain in the muscles of the neck, shoulders, lower back, hip and thighs

Question 33

signs of GCA

Answer 33

fever, anemia, high ESR50 yrs

scalp pain and claudication of jaw and tongue

Question 34

complication of GCA

Answer 34

ischemic optic neuropathy, which can lead to sudden blindness

Question 35

diagnosis of GCA

Answer 35

biopsy of temporal artery

u/s of temporal artery may also be helpful

Question 36

diagnosis of polymyalgia rheumatica

Answer 36

typical symptoms of stiffness, aching, and pain in the muscles of the hip and shoulder girdle, an increased ESR, the absence of clinical features suggestive of GCA, and a prompt response to low-dose prednisone

Question 37

what is the treatment for both GCA and PR?

Answer 37

steroids

Question 38

takayasu’s arteritis

Answer 38

inflammatory and stenotic disease of medium and large sized arteries with strong predilection for the aortic arch and its branches