Vasculitis and Related Conditions Flashcards
What symptoms may tip you off to systemic vasculitis?
constitutional symptoms (cytokine release)
systemic illness
multiple organs involved
vessel inflammation leads to tissue ischemia in many areas of the body
How is vasculitis diagnosed?
clfinicopathologic diagnosis- biopsy tissue must be used to confirm the diagnosis in most forms
many other conditions can mimic vasculitis and of many of those conditions will be mad worse by immunosuppression
What are some generalized symptoms of systemic vasculitis?
palpable purpura (non-blanching), can progress with necrotic centers
List potentially helpful tests in systemic vascultitis.
tissue biopsy, angiogram (depending on size of vessel)
CXR, UA with miscroscopy, serum creatinine and LFTs (check organ involvement)
cryoglobulins- sludging of blood when cold due to immune complexes that preciptitate
C3 and C4, ANA, RF, ANCA
Hep B and C serology can be helpful in treatment
What is the prognosis for patients with systemic vasculitis?
small and medium vessel forms are often fatal without treatment
large vessel forms of vasculitis are associated with significant morbidity but limited mortality
early diagnosis and treatment are critical
How are small and medium vessel vasculitis managed?
small/med: high-dose corticosteroids and immunosuppressive meds
large: corticosteroids
What are the most common symptoms/signs of Giant cell arteritis?
new onset headache constitutional symptoms proximal myalgia jaw claudication scalp tenderness visual symptoms
signs: fever, artery induration, bruit or diminished pulse, ischemic retinopathy,
tender, non-pulsatile temporal artery
labs: ESR++, anemia, thrombocytosis
What is the standard treatment for GCA?
begin high dose steroids immediately, obtain a biopsy as soon as possible (long section due to skip lesions)
What are the signs and symptoms of Granulomatosis with polyangitis (Wegner’s)?
CXR: infiltrates, nodules, cavitary lesions
UA: red cell casts (nephritis), p-ANCA+, elevated creatinine, pouch-immune glomerulonephritis
anemia of chronic disease, elevated sed rate
List the p-ANCA positive arteritises.
polyarteritis nods
Eosinophilic granulomatosis w/ polyangiitis
(IBD, CLD, HIV, Lupus)
c-ANCA is positive in granulomatosis with polyangiitis
What are the striking features of Takayasu’s artiritis?
effects women <40yo
cladication of the extremities (pulse-less limbs)
bruit over subclavian, or abdominal aorta
abnormal arteriogram or MRA
tx. with initially high-dose steroids
What are the principle organs that are effected in polyarteritis nodosa (PAN)?
skin- purpura
nerve: mononeuropathy/multiple mononeuropathies
kidney: HTN, elevated creatinine, proteinuria w/o RBC casts
**associated with Hep B
treated with steroids +/- cyclophophamide
What are the striking features of cryoglobulinemic vasculitis?
palpable purpura LFT abnormalities >90% have Hep C elevated creatinine, proteinuria low C4, RF
tx with steroids + anti-viral +/- plasmapheresis AND rituximab (best for cryo)
What organs does microscopic polyangiitis effect primarily and how do you treat it?
lungs: pulmonary infiltrates
kidneys: proteinuria, hematuria, RBC casts, pauci-immune glomerulonephritis
treatment with steroids + rituximab/cyclophophamide
What symptoms are associated with eosinophilic granulomatosis with polyangiitis?
asthma, allergic rhinitis, nasal polyposis, eosinophilia (non-fixed pulmonary infiltrates)
cardiomyopathy, myocardial infarction
