Flashcards in Vasculitis (Postlew) Deck (54)
Primary finding in vasculitis
inflammation within blood vessel walls
What arteries does Polyarteritis Nodosa affect?
(spares arterioles, venules, capillaries)
Polyarteritis Nodosa is associated with...
Hep B (30% of patients)
Polyarteritis Nodosa: epidemiology
slight male predominance
Polyarteritis Nodosa: Clinical Manifestations
--kidney involvement (= HTN and renal failure)
--cardiac involvement (MI, CHF, pericarditis)
--skin (livedo reticularis)
Polyarteritis Nodosa: lab findings
↑ESR, ↑CRP, hematuria, ↑creatinine
Cutaneous Polyarteritis Nodosum is associated with...
How is Buerger’s Disease characterized?
Granulomatous inflammation, which predominantly affects vascular supply to the lower limbs
Buerger’s Disease: epidemiology
young adult M, tobacco smokers
Which CT disorders can be associated with IC-mediated vasculitis?
SLE, Sjögren's syndrome, and RA
What is the most prominent feature in Immune Complex-Mediated Small Vessel Vasculitis?
Cutaneous involvement of small blood vessels
What is the classic cutaneous symptom associated with Immune Complex-Mediated Small Vessel Vasculitis?
(pustules, vesicles, urticaria, small ulcerations may also be found)
Hypersensitivity Vasculitis usually occurs secondary to...
drug exposures or infections
How is Hypersensitivity Vasculitis characterized?
IC deposition in capillaries, postcapillary venules and arterioles
Clinical manifestations of Hypersensitivity Vasculitis?
--colicky abdominal pain
Henoch-Schonlein Purpura: epidemiology
90% in children
What vessels does Henoch-Schonlein Purpura affect?
small vessels, primarily postcapillary venules
What is characteristically present in Henoch-Schonlein Purpura?
Henoch-Schonlein Purpura: classic tetrad
purpura, arthritis, abdominal pain, GN
Henoch-Schonlein Purpura: lab findings
↑ESR, ↑CRP, hematuria, ↑creatinine, RBC casts present (if renal involvement)
Cryoglobulinemia Vasculitis is most often associated with...
long-standing hep C infection
How are types II/III of Cryoglobulinemia Vasculitis different than type I?
IgM and IgG (polyclonal)
associated with hep C, Sjogrens, SLE
result in IC-mediated vasculitis
Cryoglobulinemia Vasculitis: clinical manifestations
--palpable purpura with ulcers
--neurological involvement, peripheral neuropathy
--Digital ischemia (often secondary to cold_
Cryoglobulinemia Vasculitis: lab findings
↑ESR, ↓C4, (+) RF
What happens if heparin is in collection tubes when trying to dx Cryoglobulinemia Vasculitis?
can → false (+) cryofirinogen
Cryoglobulinemia Vasculitis: pathology
leukocytoclastic vasculitis with IgM + C3 deposits in/around small and medium sized vessels
What vessels does Giant Cell Arteritis (Temporal) affect?
large sized arteries originating from the arch of the aorta
How is Giant Cell Arteritis characterized?
Giant Cell Arteritis: epidemiology
Age >50, Females 3:1, Northern European ancestry
Giant Cell Arteritis: potentially serious complication
thoracic aortic aneurysms
Clinical Manifestations of GCA?
--Systemic symptoms (fever, weight loss, fatigue)
--Visual disturbances (can progress to sudden blindness; due to ↓blood to ophthalmic a)
--Jaw or tongue claudication
--Tender, swollen temporal artery
GCA: lab findings?
markedly elevated ESR
If you think a patient may have Giant Cell Arteritis, what is the first thing you should do?
begin trx to prevent blindness; do NOT wait for biopsy
What vessels does Takayasu’s Arteritis affect?
large-sized arteries + involvement of aortic arch + branches (+ coronary/pulm arteries)
How is Takayasu’s Arteritis characterized?
Takayasu’s Arteritis: epidemiology
adolescent girls and young women of Asian descent
What vessels does Kawasaki Disease affect?
small and medium-sized muscular arteries, especially coronary arteries
Kawasaki Disease: epidemiology
Children, Asian ancestry
Kawasaki Disease is known to cause...
acquired heart disease (**esp in US and Japan)
What causes most of the mortality in Kawasaki Disease?
(diseases causes pericardial effusion, myocarditis, aneurysms, MI)
Clinical Manifestations of Kawasaki Disease?
fever, rash, strawberry tongue, erythema of palms/soles, edema of hands/feet, conjunctival injection...
What occurs in the second week of Kawasaki Disease?
Cutaneous vasculitis in Connective Tissue Diseases is associated with...
hypocomplementemia and ↑↑ANAs
Pathology associated with Connective Tissue Disease Associated Vasculitis?
IgG and C3 deposition in/around dermal blood vessels +/- IgM
What are the hallmarks of Rheumatoid Vasculitis?
--Pupuric lesions +/- concomitant medium vessel vasculitis
--Deep cutaneous ulcer over malleoli
Rheumatoid Vasculitis typically occurs in patients with what condition?
nodular, joint-destructive, RF(+) RA
Clinical manifestations associated with Microscopic Polyangiitis?
--lung involvement + pulm hemorrhage
Microscopic Polyangiitis: lab findings
RBC casts in urine
ANCA positive (usually p-ANCA)
Granulumatosis With Polyangiitis: Clinical Manifestations (classic triad?)
**ENT, respiratory tract and kidney involvement
glomerulonephritis, sinusitis, nasal ulcerations, saddle nose, +/- pulm infiltrates or nodules
Most notable lab finding associated with Granulumatosis with Polyangiitis?
Allergic Granulomatosis With Polyangiitis: lab findings
↑ESR, eosinophilia, eosinophiluria, RBC casts, proteinuria
Allergic Granulomatosis With Polyangiitis: Clinical manifestations
Systemic symptoms (fever, weight loss, fatigue)
Lungs: dyspnea, cough
Neurological: peripheral neuropathy
GU: eosinophilic granulomas of urinary tract and/or prostate
Bechet’s Disease: clinical manifestations
--Oral and genital ulcers
--Cutaneous lesions (pyoderma gangrenousum-like lesions, pathergy lesions)