Vasculitis

Question 1

What are the systemic manifestations of vasculitis?

Answer 1

fever, malaise, fatigue, weakness, anorexia, weight loss

Question 2

What are the skin manifestations of vasculitis?

Answer 2

purpura, ulcers, nodules, necrosis

Question 3

What are pulmonary manifestations of vasculitis?

Answer 3

infiltrates, nodules, hemoptysis

Question 4

What are oropharyngeal manifestations of vasculitis?

Answer 4

sinusitis

Question 5

What are CV manifestations of vasculitis?

Answer 5

MI, CHF, HTN, claudication

Question 6

What are MSK manifestations of vasculitis?

Answer 6

arthralgia, arthritis, myalgia

Question 7

What are renal manifestations of vasculitis?

Answer 7

hematuria, proteinuria, casts, HTN, acute renal failure

Question 8

In general, _____ is critical to diagnosing vasculitis

Answer 8

biopsy

Question 9

What other tests might be helpful in diagnosing vasculitis?

Answer 9

chest X ray
liver tests- elevated
creatinine- elevated
UA- proteinura, hematuria
ANCA
HBV/ HCV
ANA
RH

Question 10

What treatment is used for vasculitis?

Answer 10

corticosteroids +/- azothioprine, methotrexate, mycophenolate mofetil

Question 11

Diagnose:
New headache, jaw claudication, blindness, polymyagia rheumatica Temporal artery tenderness, loss of pulse
ESR> 50 mm
Temporal artery biopsy shows a granulomatous or mononuclear infiltrate in the artery, fragmentation of the internal elastic lamina

Answer 11

giant cell arteritis

• age generally >50
  usually monophasic
  start steroids immediately

Question 12

Diagnose:
young female
claudication of extremities, pulseless on exam
bruit over subclavian artery/ abdominal aorta
abnormal arteriogram

Answer 12

Takayasu’s arteritis

Question 13

Diagnose:
HBV+ 
weight loss
vascular purpura, livedo reticularis
myalgia, arhtralgia
one or more mononeuropathies
HTN
focal segmetnal vasculitis on arteriogram
biopsy shows necrotizing vasculitis

Answer 13

Polyarteritis nodosa

Question 14

Diagnose:
HCV+
palpable purpura, myalgia, arthralgia
serum cryoglbulins, RH, hypocomplementemia

Answer 14

cryoglobulinemic vasculitis

Question 15

Diagnose:
Sinusitis, epistaxis, otitis media
Chest x-ray w/ nodules, infiltrates, cavities
Proteinuria, hematuria, RBC casts
c-ANCA+
Pauci-immune glomerulonephritis
Granulomatous inflammation within or adjacent to an artery wall

Answer 15

Granulomatosis with polyangiitis

Question 16

How is granulomatosis with polyangiitis treated?

Answer 16

Rituximab

Question 17

Diagnose: 
pulmonary infiltrates on chest X ray
proteinuria, hematuria, RBC casts
p-ANCA and MPO+
pauci-immune glomerulonephritis
necrotizing vasculitis on biopsy

Answer 17

microscopic polyangiitis

Question 18

Diagnose: 
hx of asthma, allergic rhinitis
eosinophilia
non-fixed pulmonary infiltrates
cardiomyopathy, MI
p-ANCA+
extravascular eosinophils on biopsy

Answer 18

eosinophilic granulomatosis with polyangiitis

Question 19

Diagnose: 
painful recurrent oral and genital ulcers
uveitis, retinal vasculitis
erythema nodosum, severe acne
DVTs
aseptic meningitis

Answer 19

Bechet’s disease

Question 20

Describe the epidemiology of Bechet’s disease

Answer 20

More common in Mediterranean heritage, more severe in men

onset in 2nd or 3rd decade of life

Question 21

What aspects of Bechet’s disease increase mortality?

Answer 21

bleeding pulmonary artery and gastrointestinal aneurysms, CNS involvement

Question 22

How is Bechet’s disease treated?

Answer 22

Local corticosteroids for ulcers

azothioprine for eye involvement

Question 23

Diagnose:

stiffness and soreness in the shoulders and hips, worse in the morning

Answer 23

polymyalgia rheumatica

Question 24

Describe the epidemiology of polymyalgia rheumatica

Answer 24

more common in N Europeans, onset 50-80 years

associated with giant cell arteritis

Question 25

Polymyalgia rheumatica is extremely sensitive to __________

Answer 25

low dose prednisone