Vasculitic Syndromes Flashcards
Giant cell arteritis- epidemiologies
females of north european descent, >50
Giant cell arteritis- Sx
it’s in the temporal artery so headaches, jaw pain, blurry/double vision
Giant cell arteritis- Dx
bilateral temporal artery biopsy, increase in ESR, multinucleated giant cells
Giant cell arteritis- Tx
prednisone
Takayasu arteritis- what is it
also known as aortic arch syndrome or pulseless disease. Vasculitis causes an inflammation which damages the aorta and the its main branches leading to arterial stenosis or aneurysms.
Takayasu arteritis- patient population
females between 15 and 40 years old in Asia, Latin America, and Eastern Europe
Takayasu arteritis- Sx
abscence of peripheral pulses, ischemia to head, fever, vascular bruits
Takayasu arteritis- Dx
increase in ESR and aortic arch thickening
Takayasu arteritis- Tx
Corticosteroids
What is pylyarteritis nodosa (PAN)?
vasculitis of medium-sized vessels
Is PAN involved with glomerulonephritis?
No
What infection is PAN assocated with?
Hep B and C
PAN- Dx
increase in BUN and Hep B antigen
What is Microscopic polyangitis (MPA)?
vasculitis of small-sized vessels
Is MPA involved with glomerulonephritis?
Yes (+lung involvement)
MPA- Dx
Increase in P-ANCA
What are the diseases associated with secondary polyarteritis?
hepatitis C infection, rheumatoid arthritis, Sjogren syndrome, mixed cryoglobulinemia, hairy cell leukemia, myelodysplastic syndrome, and other hematologic malignancies
What is the confirmatory test used to demonstrate vasculitis?
Angiography or biopsy of involved tissue showing vasculitis
What are the agents used in the treatment of vasculitis?
Early diagnosis and corticosteroid therapy. Cytotoxic or antimetabolite drugs such as cyclophosphamide, methotrexate, and azathioprine are used in combination with corticosteroids
Churg Strauss vasculitis- why it’s different than PAN
The presence of granulomas as well as the abundance of eosinophils distinguishes this disease from PAN
Churg Strauss vasculitis- population
males with a median age of 38
Churg Strauss vasculitis- Sx
1) a history of, or current symptoms of, asthma, 2) peripheral eosinophilia (>1.5 x 10^9 eosinophils/L), 3) systemic vasculitis of at least 2 extra-pulmonary organs.
Churg Strauss vasculitis- Dx
Granulomatous, necrotizing vasculitis w/ eosinophilia. Elevated IgE & ↑ P-ANCA
Churg Strauss vasculitis- Tx
Corticosteriod w/ or w/o cytotoxic agents
Buerger- population
young smokers
Buerger disease- Sx
ischemic injury to fingers and toes, can lead to amputation
Buerger disease- Tx
STOP SMOKING YOU IDIOT
Primary Angitis of CNS- cause
a form of vasculitis where the body attacks the arteries of the brain and spinal cord. It has a chronic fluctuating and progressive course
Primary Angitis of CNS- Sx
headache, maybe some mild CNS, rarely coma or stroke
Primary Angitis of CNS- Dx
Cerebral angiography and biopsy of CNS tissues, including the leptomeninges
Primary Angitis of CNS- Tx
Short, benign course: use corticosteroids and calcium-channel blockers to prevent vasospasm
Wegener Granulomatosis- 3 signs
upper and lower respiratory tract necrotizing granulomatous inflammation and focal segmental necrotizing glomerulonephritis
Wegener Granulomatosis- population
40-50 year olds. Slight male predominance.
Wegener Granulomatosis- Dx
positive cytoplasmic (c-) ANCA test
Wegener Granulomatosis- Tx
Corticosteroids
What are the 4 diseases with a + p-ANCA test?
Idiopathic crescentic glomerulonephritis
Microscopic polyarteritis nodosa
Churg-Strauss syndrome
Wegener granulomatosis
What is the most common cause of isolated cutaneous vasculitis?
drugs
What are clinical manifestations of isolated cutaneous vasculitis?
urticaria, palpable purpura, livedo reticularis, or skin ulceration.
Schonlein-Henoch vasculitis- presentation
arthritis, gastrointestinal pain, kidney inflammation, and purpura in kids
Schonlein-Henoch vasculitis- Dx
IgA deposition in vessel walls and normal complement levels.
Type I Cryoglobulins- 3 cancers
Multiple myeloma, chronic lymphocytic leukemia and Waldenstrom’s macroglobulinemia are all cancers of this type
Type I Cryoglobulins- Sx
headaches, visual disturbances, nosebleeds, Raynaud phenomenon, and ischemic ulceration
Type I Cryoglobulins- Lab findings
increased IgM
Type II cryoglobulinemia- 3 associated diseases
chronic infections (most commonly hepatitis C), autoimmune disorders, and sometimes lymphoma
Type II cryoglobulinemia- presentation
nonsystemic small vessel vasculitis with palpable purpura, urticaria, and cutaneous ulceration
Type II cryoglobulinemia- lab findings
↑erythrocyte sedimentation rate, ↑immunoglobulin levels, positive rheumatoid factor
