Vasculitic Disorders Flashcards
What can cause vasculitic disorders?
- type III hypersensitivity (immunocomplex)
- type II hypersensitivity (antigen-Ab)
- ANCA
- Direct invasion by all classes of microbial pathogens
What is ANCA?
antibodies against cytoplasmic components of neutrophils
What is cANCA associated with?
Wegener granulomatosis
What is pANCA associated with?
microcopis polyangiitis
churg strauss syndrome
What is observed with small vessel vasculitis?
palpable purpura
acute inflammation
What are examples of small vessel vasculitis?
henoch schonelein purpura
microscopic polyangiitis
What is associated with medium sized vasculitis?
Thrombosis, infarction, aneurysm of MUSCULAR artery
What are examples of medium sized vasculitis?
polyarteritis nodosa
kawasaki disease
What is commonly seen in large vessel vasculitis?
loss of pulse/stroke
What are examples of large vessel vasculitis?
Takayasu arteritis
giant cell
What is Takayasu arteritis?
PULSELESS DISEASE
Granulomatous
LARGE vessel vasculitis
involvies the AORTIC ARCH
What pop is Takayasu arteritis most commonly observed in?
young asian women and children
What are the clinical findings and tx for Takayasu arteritis?
Absent upper extremity pulse
Discrepancy in bp between arms
visual defects
stroke
tx- corticosteroids
What is a giant cell arteritis?
Granulomatous
Large vessel
involves superficial and temporal opthalmic arteries
What population is giant cell arteritis most commonly observed in?
adults >50
What are common clinical findings and tx for giant cell arteritis?
Temporal HA, jaw claudication
Blindness on ipsilateral side
Polymyalgia rheumatica
Increased ESR
tx. corticosteroids
What is polyarteritis nodosa?
Necrotizing
Medium sized vessel
involves renal, coronary, mesenteric arteries
What population is polyarteritis nodosa most commonly seen in?
Middle aged men
Asssoc. with hep B
What are clinical findings and tx for polyarteritis nodosa?
Vessels are at ALL stages of acute and chronic inflammation
Fever
Focal vasculitis> aneurysms
Organ infarction
Angiography and biopsy
tx: corticosteroids, cyclophosphamide
What is kawasaki disease?
Necrotizing
Medium vessel
coroanry arteries
What pop is kawasaki disease seen in?
Children G)
Children of asian descent have highest incidence
What are clinical findings associated with kawasaki disease?
Fever, erythema, edema of hands and feet
strawberry appearing tongue
Abnormal ECG (acute MI)
Tx: ASPIRIN, IV immunoglobulin
What is Buerger disease?
Medium sized
digital vessel thrombosis
damage to neurovascular compartment
What pops does buerger disease occur in?
Ashkenazi jews
Men 25-50 who smoke cigarettes
What are the common clinical findings and tx for buerger disease?
Resting pain of forefoot and ulcers/gangrene of foot/toes
Upper limpb ischemia w/ ulceration and gangrene, raynaud phenomenon
tx- smoking cessation, vasodilators
What is raynaud disease?
medium sized
digital vessels in fingers and toes, tip of nose and ears
What pop is raynaud disease occur in MC?
young women
A young woman presents with paroxysmal digital color changes, and ulceration/gangrene of the fingers. What does she have?
Raynaud disease
tx- avoid cold temps, Ca channel blockers
A man with SLE presents with systemic sclerosis and CREST syndrome as well as digital vasculitis with vessel fibrosis and dystrophic calcification. What does he have?
Raynaud phenomenon
What is raynaud phenomenon?
medium sized
digital vessels in fingers, toes, nose, ears
Who is affected by raynaud phenomeon?
Adult men and women
secondary to other diseaes (systemic sclerosis, CREST syndrome, SLE)
A 41 year old man presents with necrotizing granulomas in the skin, nasopharynx, lungs and kidneys. He also has c-ANCA antibodies. What does he have?
Wegener granulomatosis
Equally common in men and women
What is wegener granulomatosis?
Necrotizing
meidum and small sized vessel
involves lung (infarctions) and renal vessels (glomerulonephritis)
A child presents with palpable purpura, glomerulonephritis and p-ANCA bodies. The vessels are at the SAME stage of inflammation. What do they have?
Microscopic polyangitis
What is microscopic polyangitis?
Small vessel
involves skin, lung, brain, GI tract, kidneys
What precipitates Microscopic polyangitis?
Drugs, infections and immune disorders (SLE)
Can occur in children and adults
tx- corticosteroids and cyclophosphamide
A pt with an autoimmune disease presents with allergic rhnitis, asthma, p-ANCA antibodies and eosinophilia. What do he have?
Churg strauss syndrome
What is churg strauss syndrome?
small vessel
skin, lung, heart vessels
What pop is churg strauss most commonly seen in?
Adults around 51 with autoimmune diseases
tx- corticosteroids
A 14 year old man presents with palpable purpura of the buttocks and lower extremities, polyarthritis, glomerulonephritis, and abdominal pain and vomiting. He recently had a viral URI. What does he have
Henoch Schonlein purpura
tx- corticosteroids
What is henoch sconlein purpura?
Type III hypersensitivity
small vessel
involves skin, GI tract, renal, joint vessels
A woman presents with palpable purpura, acral cyanosis of nose and ears and raynaud phenomenon. She also has glomerulonephritis and arthritis. What does she have?
Cryoglobulinemia
tx- immunosupressives
What is cryoglobulinemia?
(proteins in plasma that gel at cold temperatures particular in areas exposed to cold temp-fingers, nose, etc.)
Small vessels
Skin, GI, renal vessels
More common in females than males
A 24 yr old female presents with fever and petechiae that begins on the palms and spreads to the trunk. What does she have?
Infectious vasculitis (RMSF)
Small vessels
skin vessels
tx- IV penicillan G
