What in the Stem would lead you to believe it is this Dz?

Question 1

Immune complex-assoc. vasculitis

Answer 1

Lupus
Drug Hypersensitivity (PCN)

Question 2

Antineutrophil cytoplasmic antibodies

Answer 2

Antiprotinase 3 (PR3-ANCA)
Antimyeloperoxidase (MPO-ANCA) - Neutrophils
Pauci-immune

Question 3

Giant cell (temporal) arteritis

Answer 3

Aorta
Giant cells
Clinical Hx: >40 yo +/- polymyalgia rheumatica
Facial pain, Headache
Double vision - ophthalmic artery involvement - may lead to vision loss
T-cell mediated (CD4)
Temporal arteries

Question 4

Churg-Strauss syndrome

Answer 4

Eosinophils required - Hypereosinophilia
Granulomas
Asthma, Allergic rhinitis
Similar to PAN and MP
MPO-ANCA (but <1/2 show +)
Palpable purpura, GI tract bleeding, renal disease (glomerulosclerosis)
Cardiomyopathy

Question 5

Takayasu arteritis

Answer 5

Aortic arch + pulmonary, coronary, renal arteries
Similar to Giant cell arteritis, granulomatous
Ocular disturbances
Pulseless Dz
Weak upper extremity pulses and low blood pressure
<50 yo, historically Japanese
Visual (including blindness) and neuro deficits seen

Question 6

Polyarteritis nodosa

Answer 6

Chronic HEP-B (HBsAg/Ab) 
Renal, heart, liver, GI 
Classically Young adult 
Branch Points
Segmental transmural necrotizing inflammation 

Sx:
Rapidly accelerating HTN
Abd pain, bloody stools
Diffuse myalgia, peripheral neuritis

Question 7

Buerger Dz (Thromboangiitis obliterans)

Answer 7

Thrombosis required 
Tibial and Radial arteries 
Veins and nerves = pain!
Clinical Hx: Young male smoker, <35 yo, Israeli, indian, japanese
Hypersensitivity to tobacco
Raynaud phenomenon
Intermittent/instep claudication 
Superficial nodular phlebitis 
Extremity ulcerations = gangrene

Question 8

Kawasaki Dz

Answer 8

80% < 4 yo
Coronary arteries
Aneurysms –> thrombosis/rupture –> acute MI
Erythema of conjunctiva, mouth, palms, soles
Desquamative rash
Cervical LN enlargement
IVIg + aspirin lowers MI risk

Question 9

Microscopic Polyangiitis

Answer 9

Necrotizing - arterioles, capillaries, venules
Lesions of same age - palpable cutaneous purpura
Renal glomeruli - necrotizing glomerulonephritis
Lung capillaries
MPO-ANCA
Fibroid necrosis
Leukocytoclastic vasculitis, hypersensitivity vasculitis
Fragmented PMNs (NEUTROPHILS)
Hemoptysis, hematuria, proteinuria

Question 10

Bechet Dz

Answer 10

Orogenital ulcers 
Uveitis 
HLA-B51 
Neutrophilic 
Mortality related to severe neurologic involvement or rupture of aneurysms

Question 11

Granulomatosis with Polyangiitis

Answer 11

Necrotizing granulomas - upper and lower resp tracts
Focal necrotizing, CRESCENTRIC, glomerulonephritis
PR3-ANCA
T-cell mediated hypersensitivity
Males more
Pneumonitis/sinusitis
Renal Dz
Nasopharyngeal ulceration
Upper resp = Geographic pattern of central necrosis
Lower rep = granulomas with cavitation

Question 12

Infectious vasculitis

Answer 12

Pseudomonas, aspergillus, mucor
Vascular invasion
Mycotic aneurysms