vasculitis

Question 1

Large Vessel Vasculitis:

Temporal (giant cell) artheritis

Answer 1

• generally elderly females
• unilateal headache
• jaw claudication
• may lead to irreversiable blindess due to opthalmic after occlusion
• assoc with polymyalgia rheumatic
• most commonly affects branches of carotid artery
• focal granulomatough inflammation
• increased ESR
• treat with high dose of corticosteroids prior to temporal artery biopsy

Question 2

Large Vessel Vasculitis:

Takayasu arteritis

Answer 2

asian females < 40
“pulseless disease” (weak upper extremity pulses)
fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances

granulomatous thickening and narrowing of aortic arch! and proximal great cells
increase ESR
treat with corticosteroids

Question 3

Medium Vessel Vasculitis:

Polyarteritis nodosa

Answer 3

young adults
Hep B seropositivity in 30% of patients
fever, weight loss, malaise, headaches
GI: melena, abdominal pain
HTN, neurologic dusnction, cutanous eruptions, renal damage

typically involves: renal and visceral vessels NOT pulmonary arteries
Immune complex mediated!
Transumural inflammation of the arterial wall with fibrinoid necrosis
innumerable microaneurysms and spasm on arteriogram
treat: corticosteroids and cyclophosphamide

Question 4

Medium Vessel Vasculitis:

Kawasaki disease

Answer 4

asian children <4
fever, cerival lymphadenitis, conjunctival injection, changes in lips/oral mucosa Strawberry tongue!
hand foot erethema, desquaminating rash

may develop coronary artery aneurisms, thrombosis then mi then rupture =(

treat with IV IG and aspirin (look aspirin in a kid whoaaa)

Question 5

Medium Vessel Vasculitis:
Buerger disease (thromboangiitis obliterans)

Answer 5

heavity smokers!!! males <40
intermittent claudication may lead to gangrene, autoamputation of digits, superficial nodular phlebitis
raynauds

segmental thrombosing vasculitis
STOP SMOKING!

Question 6

Small Vessel Vasculitis: Granulomatosis with polangitis (Wegener)

Answer 6

upper respiratory tract: perforation of nasal septum, chronic sinusits, otitis media, mastoiditis

lower resp: hempotysis, cough, dyspnea
renal: hematuria, red cell casts

triad:
focal nectrozing vasculitis
necrtoizing granulomas in the lung and upper airway
nectroziing glomerulonephritis

PR2 anca or c ANCA (anti proteinase 2)

treat with chyclophoshamide, corticosteroids

Question 7

Small Vessel Vasculitis:

microscopic polangitis

Answer 7

necrotizing vasculitis commonly involving lung, kidney, and skin with pauci immune glomerulonephritis and palpable purport. similiar presentation to wegeners but without nasopharyngeal involvement

NO GRANULOMA!
MPO-ANCA/p-ANCA (anti myloperoxidase)
treat with cyclophosphamide and corticosteroids

Question 8

Small Vessel Vasculitis:

Churgg-Strauss syndrome

Answer 8

Asthma, sinsutiis, palpable purpura, peripheral neuropathy can also involve heart, GI, kidneys (pauci immune glomerulonephritis)
Granulomatous! nectrozing vasculitis with eosinophilla
MPO-ANCA/p-ANCA, increase IgE

Question 9

Small Vessel Vasculitis:

Henoch-Schonlein purpura

Answer 9

most common childhood sytemic vasculitis
often follows URI
classic triad:
skin: palpable purpura on buttocks/legs
arthralgias
GI: abdominal pain, melena, multiple lesions of same age

Vasculitis is secondary to IgA complex depositions assoc with IgA nephropathy