2nd Exam: Vasculitis Flashcards
Vasculitis:
group of systemic disease w variable Cx ft
TF? Vasculitis affects a single organ.
F.
Causes of vasculitis:
infection or immune-mediated usually
Pathology of vasculitis:
endo damage, thrombosis/ clot formation, inflammation, fibrinoid necrosis, thickening, narrowing
How to dx vasculitis:
biopsy, BEFORE corticosteroids, otherwise inflammation may not be present
Fibrinoid necrosis:
necrosis of vessel wall, not really fibrin, lumen may narrow and change blood flow
3 mechs of vasculitis:
immune complex forms deposits, ANCA related, T cell mediated (granulomas)
Consequences of vasculitis:
aneurysms, hemorrhage, edema, ischemia
Cx aspects of systemic vasculitis:
inc ESR, CRP, acute phase reactants, fever, chills, malaise, weight loss
What happens after immune complexes form?
lodge in wall, inflammation
ANCA sf:
antineutrophil cytoplasmic antibody
How to differentiate bw the types of vasculitis?
1 disease, 1 vessel usually
Large vessel vasculitis:
Takayasu / giant cell arteritis
Medium-sized vessel vaculitis;
Kawasaki disease, polyarteritis nodosa
Small-vessel vasculitis:
H-S purpura and cryoglobulinemic (Goodpasture syndrome, isolated Cutaneous leukoclastic angiitis, microscopic polyangitis, Wegener granulomatosis and Churg-Strauss syndrome)
Goodpasture syndrome
rare AI d, Ab’s that attack lungs and kidneys, isolated
Temporal (cranial) arteritis:
oft women 65+, large elastic arteries, branches of carotid aa., aorta, temporal a., pathology represents panarteritis (granulomatous inflammation early, fibrosis and nonspecific changes late, granuloma disappears), not entire length of artery, often nodular, swollen, seen visually, all layers thickened
How to dx temporal (cranial) arteritis:
biopsy
Early granulomatous inflammation is diagnostic of:
temporal areteritis
giant cells indicate:
granulomatous reaction
neutrophils will be found in this region in temporal arteritis:
Below intima
Cx ft’s of temporal arteritis:
high ESR (often lower than 80), throbbing unilateral headache, scalp pain near the artery, fever, visual disturbances later, ophthalmic a. d. - ischemic optic n., oft preceded by amaurosis fugax, (transient loss of vision in 1 or both eyes), blindnesss wo tx
Tx for temporal arteritis:
corticosteroids, prevents blindness
Polyarteritis nodosa:
young to middle aged, over 30yo, males 2:1, affects muscular, not elastic aa., transmural, segmental different aged nodules, deposits in vessel wall (Ig and complement), Hep B ag in deposits 30% of time, Hep B ag may be found in blood, vessels, many aa. are affected, smaller diameter vessels, they may bleed
TF? Polyarteritis nodosa is a granulomatous disease.
F.
Transmural is aka:
panarteritis
cell that lacks polyarteritis nodosa, not granulomatous.
giant cell
Cells present in a polymorphous infiltrate:
PMN’s, lymphos
Possible effects of polyarteritis nodosa:
thrombosis, infarcts, ischemia
In early lesions of polyarteritis nodosa:
fibrinoid necrosis
What does necrosis look like in wall of affected vessel?
fibrin
What happens after PAN w fibrinoid and organizing thrombus?
inflammatory process attacks thrombosis, ongoing disease, many different temporal aspects, one healing while another in chronic phase
Cx fts of PAN:
Puzzling, can masquerade, polyneuropathy, kideny disease due to arteritis, not glomerulonephritis (hypertension, renal insufficiency), GI d. w hemorrhage or infarction, pulmonary involvement rare
If the lungs are involved, rule out this:
PAN
This rash looks like acute RF:
livedo reticularis
Livedo reticularis:
related to vasculitits, mottled reticulated vascular pattern, lace-like purplish discoloration of the skin, looks a little like rheumatic fever
erythema marginatum:
circular, serpiginous (wavy margin), erythematous rash
Focal dilations of vessel:
aneurysm, round protuberence, inflammation
To dx aneurysm if many organs are involved:
angiogram
ANCA-assoc vasculopathies:
Wegner’s granulomatous, microscopic polyangitis, Churg-Strauss syndrome, antineutrophil antibodies syndrome
Characteristic ANCA-assoc vasculopathy:
Wegner’s granulomatous
Churg-Strauss syndrome:
asthma, lung d., skin d.
antineutrophil antibodies syndrome:
Ab’s against granules in PMNs, higher Abs w acute or recurrent disease, c-ANCA = diffuse pattern, granules, (Abs vs proteinase 3), p-ANCA = perinucleuar pattern (Abs vs myeloperoxidase (MPO))
To determine if antineutrophil antibodies syndrome is getting worse:
Ab titer
This ANCA is Abs to granules, entire cytoplasm of neutrophils appears stained:
c-ANCA
This ANCA has a perinuclear pattern:
p-ANCA
To dx p-ANCA:
immunofluorescence microscopy (IFM)
ANCAs are generated vs enzymes in the:
azurophilic granules of PMNs
Abs against enzyme in the azurophilic granules of PMNs result in:
degranulation of PMNs (tissue destruction), adherence of polys to endo (tissue destruction), Abs cause PMNs to damage vessels and tissue (tissue and vessel destruction)
How do degranulated PMNs cause tissue damage?
contain enzymes, stimulate formation of free radical, damage
Wegner’s granulomatosis, preferred name:
Granulomatosiss w polyangitis
Granulomatosiss w polyangitis:
usually 40yo men, most have + c-ANCA in serum, necrotizing vasculitis, small aa. and vv., vascualar or extra-vascular granulomas
Temporal arteritis and polyarteritis nodosa both involve what structures?
vessels, not tissues
Wegener’s granulomatosis involves these structures:
vessels OR tissues, i.e., lungs, nose, hard palate, renal disease
Staining pattern of Wegener’s granulomatosis:
C diffusing (ACNAs)
Cx ft of Wegener’s granulomatosis:
Sinuses, lungs, then kidneys: involves sinuses, nasopharynx (95%) - cough, hemoptysis, dyspnea, lung (90%,), kidney disease (77%) - proteinuria, hematuria, failure
To test for Wegener’s granulomatosis:
immunofluorescence microscopy, c-ANCA pattern (diffuse staining bc there are so many granules bound by Abs)
Large, necrotic, cavitating granuloatous lesions are characteristic of this:
Wegener’s lung in Wegener’s granulomatosis:
Cells found in the outer wall of Wegener’s lung vessels:
chronic inflammation: lymphos, plasma cells, Giant cells
Issue related to Wegener’s Lung:
vasculitis, granulomas
To dx Wegener’s from a nasal lesion:
biopsy
Wegener’s renal disease is a disease of:
glomerulus, not vessel/ vasculitits
Are granulomas in the kidney common or rare?
rare
Wegener’s renal diseases:
crescentic/ RPGN (Rapidly progressive glomerulonephritis), glomerular, no immune deposits (pauci-immune vasculitis)
2 types of Wegener’s renal disease:
crescentic or RPGN
Pauci-immune vasculitits:
associated w min evidence of hypersensitivity upon immunofluorescent staining for IgG, no immune deposits
Henoch-Schonlein Purpura:
small vessel acute vascularitis with PMNs, 4-7yo, in spring, oft after URI, esp strep, features in common w IgA nephropathy
Most common systemic vasculitis in children:
small vessel acute vascularitis with PMNs (H-S Purpura)
Vasculitis that might be triggered by infection:
H-S Purpura
Are granulomas seen w small vessel acute vascularitis with PMNs?
no
Cx ft of H-S Purpura:
palpable dot-like purpura (blood in skin), GI pain, diarrhea, bleeding, protein and blood in urine, pulm d and neuropathy rare, often resolve on own
Rx for H-S Purpura:
corticosteroids
Pathologoy of H-S purpura:
inc serum IgA, immune-complex deposits (IgA + unknown Ab), acute vasculitits
To dx H-S Purpura
fluorescence micro
IgA antibodies in wall of vessel, no other vasculitis looks like this
Progression of H-S purpura
polys, degranulate, apoptosis, disappear, fragment
Is H-S purupura bigger or smaller than petechiae:
bigger
H-S purpura is due to:
leukocytoclastic vacsulitis
leukocytoclastic vasculitis:
acute necrosis, tiny vessels of skin (leads to bleeding), wall infiltrated by PMNs w nuclear dust (clastic), can see nuclei very well, blue dots smaller than nuclei
vasculitis in the dermis:
leukocytoclastic vasculitis
H-S purpura renal d.:
glomerulus, deposited throughout body, especially skin
H-S purpura renal d. looks like:
IgA nephropathy, deposits of IgA in the mesangium
