Vasculitides Flashcards
Gaint cell (temporal) arteritis: who and what age?
Female >50
Gaint cell (temporal) arteritis: symptoms? (3)
unilateral headache
jaw claudication
possible temporal artery tenderness
Gaint cell (temporal) arteritis: important complication?
irreversible blindness die to AION (anterior ischemic optic neuropathy)
irriversible blindness in Gaint cell (temporal) arteritis: cause?
anterior ischemic optic neuropathy (AION)
= interruption of blood flow to the front (anterior) of the optic nerve
Gaint cell (temporal) arteritis: associated with which other disease?
polymyalgia rheumatica
which arteritis is associated with PMG?
Gaint cell (temporal) arteritis
which arteritis is most common in female >50?
Gaint cell (temporal) arteritis
unilateral headache, tenderness of temporal artery, jaw claudication
what can it be?
Gaint cell (temporal) arteritis
which arteritis may lead to irreversible blindness due to AION?
Gaint cell (temporal) arteritis
Gaint cell (temporal) arteritis: which artery branches most commonly affected?
carotid branches
Gaint cell (temporal) arteritis: what kind of inflammation?
focal granulomatous
Gaint cell (temporal) arteritis: which inflammation marker very high?
ESR
Gaint cell (temporal) arteritis: levels of which cytokine correlate with disease activity?
IL-6
Gaint cell (temporal) arteritis: treatment?
high dose glucocorticocoids
Gaint cell (temporal) arteritis: when to apply treatment, before or after biopsy?
before
Gaint cell (temporal) arteritis: diagnosis?
temporal artery biopsy
Gaint cell (temporal) arteritis: why start treatment before biopsy?
to prevent blindness
Takayasu arteritis: in who and what age?
asian female <40
Takayasu arteritis: symptoms?
weak upper extremity pulses
fever, night sweats, arthritis, myalgias
skin nodules and ocular disturbances
other name for Takayasu arteritis?
pulselessness disease
pulselessness disease: what is this?
takayasu
takayasu arteritis: pathophysiology?
granulomatous thickening/narrowing of aortic arch and proximal great vessels
35 y/o chinese woman
weak upper extremity pulses,
night sweats, myalgias, skin nodules
elevated ESR
suggestive of?
takayasu
takayasu: which inflammation marker elevated?
ESR
takayasu: treatment?
glucocorticoids
granulomatous thickening/narrowing of aortic arch and proximal great vessels
which disease?
takayasu
what are the large vessel vasculitides?
giant cell arteritis
takayasu
what are the medium vessel vasculitides?
Buerger, Kawasaki, polyarteritis nodosa
Buerger: in who?
males <40
heavy tabacco smoking
Buerger: other name?
thromboangiitis obliterans
Buerger: symptoms?
intermittent claudication
may lead to gangrene, autoamputation of digits, superficial nodular phlebitis
Raynaud phenomen often present
man 35, heavy tobacco smoking history
intermittent claudication, gangrene toe, Raynaud phenomen
suggestive of?
Buerger
Buerger: pathophysiology?
segmental thrombosing vasculitis
vein and nerve involvement
Buerger: treatment?
smoking cessation
segmental thrombosing vasculitis
vein and nerve involvement
what is this?
Buerger
Kawasaki: in who?
Asian kids <4
vasculitis common in asian kids <4?
kawasaki
kawasaki: symptoms?
CRASH and burn
Conjunctivitis
Rash
Adenopathy
Strawberry tongue
Hand and foot changes
and burn: fever
3 y/o japanese kid
fever, cervical adenopathy, desquamating rash, red and swollen feet and hands
suggestive of which vasculitis?
kawasaki
rash in kawasaki?
polymorphous, desquamating (shedding/scraping off)
what kind of adenopathy in kawasaki?
cervical
hand and foot changes in kawasaki?
swollen and erythema
conjunctivitis in kawasaki?
bilateral non-exudative (no fluids) bulbar
is the conjunctivitis in kawasaki unilateral or bilateral?
bilateral
what was kawasaki formerly called?
mucocutaneous lymph node syndrome
kawasaki: complications?
coronary artery aneurysms; thrombosis or rupture can cause death
what can cause death in kawasaki?
coronary artery aneurysms
kawasaki: treatment?
IVIG and aspirin
polyarteritis nodosa: in who?
middle-aged males
polyarteritis nodosa: seropositive for what in 30% of the cases?
Hepatitis B
in which arteritis 30% of patients seropositive for Hep B?
polyarteritis nodosa
polyarteritis nodosa: involvement of which systems?
cardiovascular, GI, neuro, skin, renal
polyarteritis nodosa: symptoms?
GI: abdominal pain, melena
Hypertension, neurologic dysfunction, skin sores, renal damage
52 y/o man with hypertension, abdominal pain, melena, skin sores, proteinuria and hematuria
Hep B seropositivity
suggestive of?
polyarteritis nodosa
what system is NOT involved in polyarteritis nodosa?
pulmonary
which arteries are typically involved in polyarteritis nodosa?
renal and visceral
polyarteritis nodosa: renal angiogram/ateriogram findings?
innumerable renal microaneurysms, and spams
‘string of pearl’ appearance
‘string of pearl’ appearance on renal angiogram
which disease?
polyarteritis nodosa
polyarteritis nodosa: treatment?
glucocorticoids
cyclophosphamide
polyarteritis nodosa: what is it?
small and medium-sized arteries swollen and damaged
serious vasculitis
Behcet syndrome: in who?
people with Turkish and eastern mediterranean descendent
Behcet syndrome: symptoms?
aphtous ulcers, genital ulceration, uveitis, erythema nodosum
what is erythema nodosum?
tender, red bumps, usually found symmetrically on the shins
what kind of small vessel vasculitis can cause uveitis?
behcet
what can behcet be precipitated by?
HSV (herpes simplex virus) or parvovirus
HSV and parvovirus can trigger which vasculitis?
behcet
how long do behcet flares last?
1-4 weeks
which vasculitis comes in flares?
behcet
1-4 weeks
turkish man with genital ulcers, erythema nodosum and uveitis
suggestive of?
behcet
what kind vasculitis is behcet?
immune complex
with which HLA type is behcet associated?
HLA-B51
which vasculitis is associated with HLA-B51?
behcet
cutaneous small vessels vasculitis: when does it occur?
7-10 days after medication or infection
cutaneous small vessels vasculitis: which medication can cause it?
penicillins
cephalosporins
sulfonamides
phenytoin
allopurinol
penicillins
cephalosporins
sulfonamides
phenytoin
allopurinol
can cause which vasculitis?
cutaneous small vessel vasculitis
cutaneous small vessel vasculitis: which infections can cause this?
HCV (hep C), HIV
cutaneous small vessel vasculitis: symptoms?
palpable purpura
(7-10 days after certain infection or medication)
cutaneous small vessel vasculitis: visceral involvement?
no
which small vessel vasculitis has no visceral involvement?
cutaneous small vessel vasculitis
person with palpable purpura, no visceral symptoms
8 days after taking antibiotics
which vasculitis?
cutaneous small vessel vasculitis
person with palpable purpura
9 days after HCV infection
which vasculitis?
cutaneous small vessel vasculitis`
what kind of vasculitis is cutaneous small vessel vasculitis?
immune complex mediated leukocytoclastic
leukocytoclastic vasculitis: other name?
cutaneous small vessel vasculitis
cutaneous small vessel vasculitis: other name?
leukocytoclastic vasculitis
what is leukocytoclasis?
infiltrating neutrophils causing vascular damage
cutaneous small vessel vasculitis
churg strauss: other name?
eosinophilic granulomatosis with polyangiitis
eosinophilic granulomatosis with polyangiitis: other name?
churg-strauss
eosinophilic granulomatosis with polyangiitis: symptoms?
asthma
sinusitis
skin nodules or purpura
peripheral neuropathy (wrist/foot drop)
eosinophilic granulomatosis with polyangiitis: which other organs can be involved?
heart, BI, kidney
how do call eosinophilic granulomatosis with polyangiitis with renal involvement?
pauci-immune glomerulonephritis
eosinophilic granulomatosis with polyangiitis: pathophysiology?
granulomatous, necrotizing vasculitis with eosinophilic involvement
eosinophilic granulomatosis with polyangiitis: antibodies?
P-ANCA/MPO-ANCA
IgE increased
eosinophilic granulomatosis with polyangiitis: which ANCA?
P-ANCA
Churg-strauss: which cells particularly involved?
eosinophilesc
churg-strauss: symptoms?
asthma
sinusitis
skin nodules or purpura
foot/wrist drop (neuropathy)
person with sinusitis, wrist drop, and skin purpura
P-ANCA positive, elevated IgE
suggestive of?
churg strauss (eosinophilic granulomatosis with polyangiitis)
which small vessel vasculitis has increased IgE?
eosinophilic granulomatosis with polyangiitis
granulomatosis with polyangiitis: which 3 systems are involved?
upper respiratory tract
lower respiratory tract
renal
granulomatosis with polyangiitis: symptoms upper respiratory tract?
perforation nasal septum
chronic sinusitis
otitis media
mastoiditis
granulomatosis with polyangiitis: symptoms lower respiratory tract?
hemoptysis
cough
dyspnea
granulomatosis with polyangiitis: renal symptoms?
hematuria, red cell clasts
person with chronic sinusitis, otitis media, couching, hemoptysis and hematuria
C-ANCA is positive
suggestive of?
granulomatosis with polyangiitis
involvement of lower/upper respiratory tract and kidneys
C-ANCA positive
suggestive of which vasculitis?
granulomatosis with polyangiitis
pathological triad of granulomatosis with polyangiitis?
focal necrotizing vasculitis
necrotizing granulomas in lung/upper airway
necrotizing glomerulonephritis
granulomatosis with polyangiitis: which ANCA?
C-ANCA/PR3-ANCA
granulomatosis with polyangiitis: CXR?
large nodular densities
in which vascilitis large nodular densities on CXR?
granulomatosis with polyangiitis
granulomatosis with polyangiitis: treatment?
glucocorticosteroids in combination with rituximab or cyclophosphamide
glucocorticosteroids in combination with rituximab or cyclophosphamide
treatment for what?
granulomatosis with polyangiitis
what does rituximab do?
kills b-cells
Immunoglobulin A vasculitis: other name?
henoch schonlein purpura
henoch schonlein purpura: other name?
immunoglobulin A vasculitis
Immunoglobulin A vasculitis: often follows what?
URI (upper resp infection)
most common childhood systemic vasculitis?
Immunoglobulin A vasculitis
henoch schonlein purpura (HSP)
Immunoglobulin A vasculitis: classic triad?
HSP
Hinge pain (arthralgia)
Stomach pain
Palpable purpura
Immunoglobulin A vasculitis: stomach pain associated with what?
intussusception
(invaginatie)
Immunoglobulin A vasculitis: where are the palpable purpura?
buttocks and legs
child with arthalgia, purpura on buttocks and legs, stomach pain
suggestive of?
immunoglobulin A vasculitis
henoch schonlein purpura: which immunoglobins are involved?
IgA immune complexes
Hinge pain (arthralgia)
Stomach pain
Palpable purpura
what is this?
immunoglobin A vasculitis
or henoch schonlein purpura
immunoglobin A vasculitis: treatment?
supportive care
possibly glucocorticoids
henoch schonlein purpura: treatment?
supportive care
possibly glucocorticoids
microscopic polyangiitis: presentation?
lung: couch, hemoptysis, dyspnea
kidney: hematuria, red clasts
skin: palpable purpura
similar to granulomatosis with poylangiitis without nasopharyngeal involvement
microscopic polyangiitis: which 3 systems are involved?
lung
kidney
skin
microscopic polyangiitis: skin symptoms?
palpable purpura
microscopic polyangiitis: lung symptoms?
coughing, hemoptysis, dyspnea
microscopic polyangiitis: renal symptoms?
hematuria and red cell clasts
how do call kidney involvement in microscopic polyangiitis?
pauci-immune glomerulonephritis
microscopic polyangiitis: granulomas?
no
microscopic polyangitis: which ANCA?
P-ANCA (MPO-ANCA)
what is P-ANCA?
anti-myeloperoxidase
(also anti-MPO)
microscopic polyangiitis: treatment?
cyclophosphamide, glucocorticoids
which vasculitides have ANCA?
- eosinophilic granulomatosis with polyangiitis
- granulomatosis with polyangiitis
- microscopic polyangiitis
which vasculitis have P-ANCA
- eosinophilic granulomatosis with polyangiitis
- microscopic polyangiitis
which vasculitide has C-ANCA?
granulomatosis with polyangiitis
which vasculitide is similar to granulomatosis with polyangiitis, but without nasopharyngeal involvement?
microscopic polyangiitis
Mixed cryoglobulinemia: often due to what?
infections
especially HCV (Hep C)
Mixed cryoglobulinemia: triad?
palpable purpura, weakness, arthralgias
Mixed cryoglobulinemia: other involvement (outside triad)?
peripheral neuropathy, renal disease (glomerulonephritis)
what are cryoglobulins?
immunoglobulins that precipitate in the cold
Mixed cryoglobulinemia: what kind of vasculitis?
immune complex
mix of IgG and IgM
Mixed cryoglobulinemia: mix of which immunoglobulins?
IgG and IgM
mix of IgG and IgM immuno complex deposition
which vasculitis?
Mixed cryoglobulinemia
palpable purpura, weakness, arthralgias
HCV seropositive
suggestive of?
Mixed cryoglobulinemia