vasculitis syndromes Flashcards
what is anca?
antibody directed against certain proteins of the cytoplasmic granules of neutrophils and monocytes (high in Wegener’s and microscopic polyangiitis)
vasculitis syndromes for which immune complex formation is primary source of damage
Henoch-Schonlein purpura, vasculitis ass. w/ collagen vascular diseases, serum sickness and cutaneous vasculitis syndromes, hepatitis C ass. cryoglobulinemic vasculitis, polyarteritis nodosa-like vasculitis ass. w/ hep B
vasculitis syndromes ass. w/ ANCA
granulomatosis w/ polyangiitis (wegener’s), Churg-Strauss syndrome, microscopic polyangiitis
vasculitis syndromes ass. w/ pathogenic T cell responses and granuloma formation
GCA, Takayasu’s, granulomatosis w/ polyangiitis, Churg-Strauss syndrome
general signs of vasculitis
palpable purpura, pulmonary infiltrates, microscopic hematuria, chronic inflammatory sinusitis, mononeuritis multiplex, unexplained ischemic events, and glomerulonephritis w/ e/o multisystem disease
conditions that can mimic vasculitis
infections (bacterial endocarditis, DGI, pulmonary histoplasmosis, occidiodomycosis, syphilis, lyme, RMSF, whipple’s)
coagulopathies/thrombotic microangiopathies (anti-phospholipid antibody syndrome, TTP)
neoplasma (atrial myxoma, lymphoma, carcinomatosis)
drug toxicity (cocaine, amphetamine, ergot alkaloids, methysergide, arsenic)
sarcoidosis
atheroembolic disease
antiglomerular basement membrane disease (Goodpasture’s)
amyloidosis
migraine
definition of granulomatosis w/ polyangiitis
(wegener’s)
granulomatous vasculitis of the upper and lower respiratory tracts w/ glomerulonephritis
pathology of wegener’s
necrotizing vasculitis of small arteries and veins together w/ granuloma formation
upper airway involvement in wegener’s
paranasal sinus pain and drainage
purulent or bloody nasal discharge
nasal septal perforation
lower airway involvement in wegener’s
asymptomatic iniltrates
cough, hemoptysis, dyspnea, chest discomfort
eye involvement in wegener’s
half of patients
conjunctivitis, dacrocystitis, episcleritis, etc
renal disease in wegener’s
accounts for most of the mortality
proteinuria, hematuria, RBC casts
renal failure if not addressed
labs in wegener’s
high esr
anemia, leukocytosis
mild hypergammaglobulinemia (high IgA)
90% have + antiproteinase-3 ANCA
diagnosis of wegener’s
biopsy w, necrotizing granulomatous vasculitis
side effects of cyclophosphamide
BM suppression, cystitis, bladder carcinoma, teratogenicity, GI intolerance, opportunistic infections, pulmonary fibrosis
side effects of methotrexate
GI intolerance, stomatitis, BM suppression, hepatotoxicity (can lead to fibrosis or cirrhosis), pneumonitis, teratogenicity, opportunistic infections
side effects of azathioprine
GI intolerance, BM suppression, hepatotoxicity, opportunistic infections, hypersensitivity
treatment of wegener’s
cyclophosphamide = major
also, methotrexate and steroids
definition of microscopic polyangiitis
necrotizing vasculitis w/ few or no immune complexes
glomerulonephritis, pulmonary capillaritis
absence of granulomatous inflammation differentiates it from wegener’s
ANCA+
signs of microscopic polyangiitis
glomerulonephritis (can be fatal)
hemoptysis (sign of alveolar hemorrhage)
mononeuritis multiplex, GI and cutaneous vasculitis
if upper airway disease present, likely wegener’s
labs in microscopic polyangiitis
75% + for antimyeloperoxidase ANCA antibodies
high esr, anemia leukocytosis, thrombocytosis
pathophys of vasculitis / immune complexes
immune complexes deposit in vessels, auto-immune reaction, and then the vessel lumen is compromised with ischemic changes in the tissues supplied by the involved vessel
diagnosis of microscopic polyangiitis
histologic e/o vasculitis or pauci-immune glomerulonpheritis w/ the right clinical features
definition of churg-strauss
also called allergic angiitis and granulomatosis
asthma, peripheral and tissue eosinophilia, extravascular granuloma formation, and vasculitis of multiple organ systems
signs of Churg-Strauss
constitutional
severe asthmatic attacks, pulmonary infiltrates
mononeuritis multiplex
allergic rhinitis and sinusitis
heart disease
skin disease (purpura, cutaneous and subcutaneous nodules)
labs in Churg-Strauss
eosinophilia
high ES, fibrinogen, or alpha2 globulins
ANCA, usually anti-myeloperoxidase
treatment of churg-strauss
steroids
cyclophosphamide
definition of polyarteritis nodosa
multisystem, necrotizing vasculitis of small and medium sized muscular arteries in which involvement ofthe renal and visceral arteries is characteristic
classic pathology of polyarteritis nodosa
kidney pathology: arteritis w/o glomerulonephritis
giant cell arteritis
inflammation of medium and large sized arteries; usually involves the branches of the carotid (esp temporal artery)
what is GCA associated with?
polymyalgia rheumatica (seen in 40-50% of patients with GCA)
signs of polymyalgia rheumatica
stiffness, aching and pain in the muscles of the neck, shoulders, lower back, hip and thighs
signs of GCA
fever, anemia, high ESR50 yrs
scalp pain and claudication of jaw and tongue
complication of GCA
ischemic optic neuropathy, which can lead to sudden blindness
diagnosis of GCA
biopsy of temporal artery
u/s of temporal artery may also be helpful
diagnosis of polymyalgia rheumatica
typical symptoms of stiffness, aching, and pain in the muscles of the hip and shoulder girdle, an increased ESR, the absence of clinical features suggestive of GCA, and a prompt response to low-dose prednisone
what is the treatment for both GCA and PR?
steroids
takayasu’s arteritis
inflammatory and stenotic disease of medium and large sized arteries with strong predilection for the aortic arch and its branches
