vasculitides

Question 1

henoch schonlein purpura

Answer 1

Immune compplex mediate

most common childhood systemic vasculitis

often follows gi or URI

classic triad:
skin: palpable purpura on buttocks/legs
arthlagias
gi: abdominal pain/melena
hematuria

vasculitis is 2nd to iga deposition

associated with iga nephropathy (berger disease) also occurs after respiratory or gi infection.

self limited but may reccur, treat with corticosteroid

may slowly progress to renal failure

mucosal infection increases iga production

Question 2

eosinophillic granulomatosis with polyangiitis (churg strauss syndrome)

Answer 2

pauci immune

asthma, sinusitis, skin nodules or palpable purpura.
peripheral neuropathy (foot/wrist drop)
can involve heart (cadiomyopahty), gi (bleeding), kidneys, lungs

extravascular granulomatous necrotizing vasculitis with eosinophilia.

mpo/p-anca, increased ige, peripheral eosinophilia

Question 3

microscopi polyangiitis

Answer 3

necrotizing vasculitis (fibrinoid necrosis) involving lungs, kidneys, and skin with pauci immune glomerulonephirtis and palpable purpura. presentation similar to wegner but without nasopharyngeal involvement.

no granulomas.

mpo/p-anca

treat with cyclophoasphamide, corticosteroids

leukocytoclastic vasculitis - neutrophil fragmentation

Question 4

granulomatosis with polyangiitis (wegener)

Answer 4

paucii immune

middle aged male

URI: perforation of the nasal septum, mucosal ulceration, chronic sinusitis, otitis media, mastoiditis.

LRT: hemoptysis, cough, dyspnea.

Renal: hematuria, red cell casts

palpable purpura

Triad: focal necrotizing vasculitis
adjacent granulomas in the lung and upper airway
necrotizing glomerulonephritis

pr3 anca/c anca (anti proteinase 3)

treat with cyclophosphamide, corticosteroids

Question 5

Buerger disease (thromboangiitis obliterans)

Answer 5

heavy smokers, males < 40 years old.
intermitten foot claudication -> can lead to gangrene. autoamputation of digits. superficial nodular phlebitis (tender nodules over the course of a vein).
raynaud phenomenon early -> leading to ulcers
tibial and radial arteries often involved
painful
segmental thrombosing vasculitis (necrotizing)(thrombosis cuts blood flow off to hands and feet, microabscesses, can invade vein and nerve and encasement of all three)
treatment is smoking cessation

Question 6

kawasaki disease (mucocutaenous lymph node syndrome)

Answer 6

asian children < 4 years old
conjuctival injection, rash (polymorphous -> desquamating), adenopathy (cervical), strawberry toungue (oral mucositis), fever. CRASH and burncan develop coronary anerysm -> thrombosis or rupture and death.
treat with IVIG and aspirin

Question 7

Polyarteritis nodosa

Answer 7

unique because immune complex mediated
fibrinoid necrosis (necrotizing vasculitis)
usually middle aged men
heb B sero + in 30 percent
fever, weight loss, mailais, headache
GI: abdominal pain and melena (mesenteric involvement)
Hypertension, neurologic dusfunction, cutaneous eruptions, renal damage (causes htn, most common cause of death)
typical involves renal and visceral vessels but spares lungs
transmural inflamation of the arterial wall with fibrinoid necrosis (all stages exist)
innumerable renal microaneuryms and spasms on arteriogram
treat with cyclophosphamide and corticosteroids

early lesions:
transmural with fibrinoid necrosis
eventually heals with fibrosis leading to string of pearls on imaging

Question 8

Takayasu arteritis

Answer 8

usually asian females < 40 years
pulseless disease “weak upper extremity pulses”
fever, neat sweats, arthritis, myalagias, skin nodules, ocular disturbances

granulomatosis thickening and narrowing of the aortic arch and proximal great vessels
increased esr
treat with corticosteroids

neurologic symptoms
weak pulse in one arm

Question 9

giant cell (temporal) arteritis

Answer 9

usually elderly females
unilateral headache (temporal artery), jaw claudication
may lead to irreversible blindness due to opthalmic occlusion. associated with polymalgi rheumatica (flu like symptoms with muscle pain)
most commonly affects branches of carotid artery
focal granulomatosis inflammation
increaased esr
treat with high dose corticosteroids prior to temporal artery biopsy

giant cells and intimal fibrosis
lesions are segmental requires biopsy of a long segment