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This Concept Map, created with IHMC CmapTools, has information related to: Vasculitides, The Vasculitides Large Vessel Vasculitis Takayasu Arteritis- womenមyo -gran inflammation of Ao, brr -ocular disturbances, weak ULimb pulses -fibrous thickening of Ao arch MORPHOLOGY-mostly just Ao arch 1/3 w/ rest of Ao, brr, pulm aa. -irreg thickening, intimal wrinkling -aa. to upper w/ intimal thickening-pulseless dz -coronary, renal aa. can be affected Early-adventitial mononuc infiltrate -perivasc cuffing of vaso vasorum Later-mononuc in media, some w/ granuloma -giant cells and patchy necrosis possible -sim to GCA-diff'ate based on clinical, AGE -->collagenous fibrosis -if Ao root affected->dilate-Ao insufficiency -could narrow cor. ostia->MI CLINICAL-weak pulses, low BP in UL, HTN else -cold, numb fingers -ocular-visual defects, retinal hemor, blindness -neuro-dizziness, weakness, hemiparesis -distal Ao->claudication of legs, visual/neuroSx -pulm aa->pulm HTN, cor pulmonale -variable course, can be quiescent/stable for awhile, The Vasculitides Small Vessel Vasculitis Cutaneous Leukocytoclastic Angiitis -small vessel vasculitis like MPA -localized to skin -not systemic, no kidney involvement, The Vasculitides Small Vessel Vasculitis Churg-Strauss Syndrome -allergic granulomatosis/angiitis -p-ANCA in 70% -strong ass'ion w/ allergic rhinitis, bronchial asthma, eosinophilia -EOSINOPHIL infiltrate perivasc tissues -intra-/extravasc granulomas in: lungs, heart, spleen, periph nerves, skin -identical to classic PAN, microscopic polyang -infrequent renal dz -M&M due to coronary arteritis, myocarditis, The Vasculitides Small Vessel Vasculitis Essential Cryoglobulinemic Vasculitis -small vessel vasculitis like MPA -ANCA not present -cryoglobulin immune deposits -skin, glomeruli, The Vasculitides Other Disorders CT d/o: RA-rheumatoid vasculitis -sm-med aa in multiple organs ->infarction in viscera, aortitis -w/ high RF titer, rheumatoid nodules SLE Malignancies-usually lymphoproliferative, The Vasculitides Small Vessel Vasculitis Microscopic Polyangiitis/Polyarteritis (Hypersensitivity/Leukocytoclastic vasculitis) -aa'oles, caps, venules, even med aa (unlike PAN) -all lesions of same age, p-ANCA in majority (80%) -in skin, muc memb, lungs, brain, heart, GI, kidneys -necrotizing GNephritis(90%), pulm capillaritis common ->hemoptysis, hematuria, proteinuria, bowel pain, mm. -ppting cause-rxn to Ag-drugs (PCN), streptococci, tumor MORPHOLOGY-sim to PAN-but musc, large aa spared -uncommon macroscopic infarcts -segmental fibrinoid necrosis in media -or just inflam w/ neutrophil frags (leukocytoclasia) -leukocytoclastic angiitis esp in post cap venules -Ig, complement in skin lesions -PAUCI-IMMUNE INJURY (few Igs on IF stain) CLINICAL -can show up in Henoch-Schonlein purpura, essential mixed cryoglobulinemia, ConnT issue d/o ->ANCA not present Tx-mostly just remove offending agent, some need Tx, The Vasculitides Medium-Sized Vessel Vasculitis Kawasaki Disease-young kids, infants Mucocutaneous LN syn -large, med, sm aa, cor aa. -acute-fever, conjunc/oral erythema, hands/feet edema/erythema -rash, desquamation -cervical LNs enlarged 20% develop cardiac sequelae -(-)Sx, cor aa. ectasia, aneurysm ->rupture, thrombosis, MI, death-1% -leading cause of acqired ht dz in kids PATHOGENESIS-unknown cause -immunoregulatory defect -T-cell/mac activation -autoABs to endoth, sm mm., -polyclonal B-cell hyperactivity, cytokines -SELF-LIMITING MORPHOLOGY-resembles PAN -necrosis, inflam of entire thickness -less fibrinoid necrosis Cor aa. lesions-mild intimal to severe necrosis of wall Acute-subsides spontaneously or w/ Tx SUBSIDING PHASE->aneurysm-> ->rupture,death, The Vasculitides Small Vessel Vasculitis Henoch-Schonlein Purpura -small vessel vasculitis like MPA -ANCA not present -IgA-dominant immune response -skin, gut, glomeruli ->arthralgias, arthritis, The Vasculitides Small Vessel Vasculitis Wegener Granulomatosis-peaks in 40s -necrotizing vasculitis Triad: 1)Acute necrotizing granulomas-of URT &/or LRT 2)Focal necrotizing or granulomatous vasculitis -sm-med vessels - aa, a'oles, caps, venules -esp in lungs, upper airways 3)Renal dz-often focal or necrotizing, crescentic, glomerulitis "Limited WG" if not full triad-unaffected kidneys PATHOGENESIS-very sim to PAN, serum sickness ->likely a hypersensitivity-occasional ICs -also supported by response to immune supp. -c-ANCA in serum (90%) w/ active dz -rising c-ANCA titer suggests relapse MORPHOLOGY- -mucosal granulomas to ulcerative lesions in URT (nose, palate, pharynx) -rimmed by necrotizing grans and vasculitis -almost identical to acute PAN -cavitation in lungs from coalesced focal grans -Diff Dx-mycobacteria, fungal -grans w/ lympho, plasma cell, macs, giants in rim -lesions->fibrosis, organization RENAL- -Early, milder-acute focal prolif, necrosis in glom -hematuria, proteinuria-responds to Tx -Advanced-diffuse necrosis, crescent formation -progressive renal failure CLINCAL- -persistent pneumonitis, bilateral nodules/cavitations -chronic sinusitis, mucosal ulcerations, renal dz -skin rashes, mm/jt pain, neurites, fever Tx-immunosupp, cyclophosphamide, prednisone -unTx-malignant 80% death w/in 1 year, The Vasculitides Medium-Sized Vessel Vasculitis (Classic) Polyarteritis Nodosa-YOUNG ADULTS -systemic vasculitis-transmural -renal, visceral aa, not pulm NO glomerulonephritis MORPHOLOGY-med-small aa in any organ -not lung -esp kidney, heart, liver, GI, NS, skin -very segmented lesions-often at bifurcs ->irregular aneurysmal dilation, rupture -palpable nodule, hypoperfusion, bleed HISTO-transmural inflam w/ PMNs, monos -acute-then replaced by monos and ... -fibrinoid necrosis of inner half->adventitia -thrombosed lumen possible -all haphazard, uncoordinated findings CLINICAL-can be in kids, older too -acute, subacute, chronic, remittent Sx-malaise, FUO, wt loss, rapid-onset HTN -abd pain, melena, myalgias, periph neuritis -RENAL INVOLVEMENT but not GN b/c no caps 30% w/ HepB Ag in serum, little ANCA ass'n DX-becrotizing arteritis on Bx of involved organ -angiography shows aneurysm/occlusion in 50% TX-corticosteroids+cyclophosphamide-90% remis -fatal in most unTx cases, The Vasculitides Non-infectious Vaculitis, in general Immunologic origins->systemic necrotizing vasculitides Widespread distribution-mostly smaller than aa. Initiated via: Immune Complexes-resembles Arthus, serum sickness -Accumulate in vessel walls, but xport there unknown -Pauci-immune=fewvasc immune deposits, other MOA -DNA-antiDNA in SLE-ass'd vasculitis -IgM, IgG, complement in cryoglobulinic vasculitis -drug hypersensitivity-ᡂ% vasc skin lesions-PCN, streptokinase -hepB Ag HBsAg-antiHBsAg in serum and vasc (w/ comp) -esp in PAN; ->remission of vasc, not HepB -HepC->glomerulonephritis w/ HCV/RNA in glomeruli Antineutrophil Cytoplasmic ABs-ANCAs -against neutrophil primary gran contents, lysosomal ->binding of ANCA->degranulation, tissue destruction -detected by IF stain -c-ANCA-cytoplasmic-proteinase 3 (PR-3)-Weg's -p-ANCA-perinuclear-MPO-in C-G, microscopic PA -ANCA mech is Dx marker - mech unknown Other -ABs to endoth cells->SLE, Kawasaki -Goodpasture's->glomerulitis via anti-bsment memb ABs -Viruses can cause via T-cell action, IFN-g (herpes, coxsac), Wegener Granulomatosis-peaks in 40s -necrotizing vasculitis Triad: 1)Acute necrotizing granulomas-of URT &/or LRT 2)Focal necrotizing or granulomatous vasculitis -sm-med vessels - aa, a'oles, caps, venules -esp in lungs, upper airways 3)Renal dz-often focal or necrotizing, crescentic, glomerulitis "Limited WG" if not full triad-unaffected kidneys PATHOGENESIS-very sim to PAN, serum sickness ->likely a hypersensitivity-occasional ICs -also supported by response to immune supp. -c-ANCA in serum (90%) w/ active dz -rising c-ANCA titer suggests relapse MORPHOLOGY- -mucosal granulomas to ulcerative lesions in URT (nose, palate, pharynx) -rimmed by necrotizing grans and vasculitis -almost identical to acute PAN -cavitation in lungs from coalesced focal grans -Diff Dx-mycobacteria, fungal -grans w/ lympho, plasma cell, macs, giants in rim -lesions->fibrosis, organization RENAL- -Early, milder-acute focal prolif, necrosis in glom -hematuria, proteinuria-responds to Tx -Advanced-diffuse necrosis, crescent formation -progressive renal failure CLINCAL- -persistent pneumonitis, bilateral nodules/cavitations -chronic sinusitis, mucosal ulcerations, renal dz -skin rashes, mm/jt pain, neurites, fever Tx-immunosupp, cyclophosphamide, prednisone -unTx-malignant 80% death w/in 1 year Lymphomatoid Granulomatosis Commonly confuse w/ Wegener Pulm infiltration of nodules -evolving lymphoprolif d/o- -50% develop lymphoid malignancy-NHL -lymphoid, plasmacytoid cells -not a true vasculitis -yet 1/3 w/ sim lesions in kidneys, brain, liver, The Vasculitides Other Disorders Infectious Arteritis -most frequent cause of localized arteritis -usually bac or fungal (aspergillosis, mucormycosis) -often w/ bacterial pneumonia, or near TB rxns, abscesses -sometimes from hematogenous spread of bad-septicemia -embolism from infective endocarditis -can -> mycotic aneurysms ->induce thrombosis->infarction to already-infx tissue, The Vasculitides Large Vessel Vasculitis Giant Cell (Temporal) Arteritis-MOST COMMON ᡪyo -acute, chronic -granulomatous arteritis of Ao, main brr -esp extracranial brr of carotid-temporal a. -also ophthalmic (blindness), vertebral -ass'd w/ polymyalgia rheumatica MORPHOLOGY-short segs w/ nodular thickenings -can -> slit-like lumen, thrombosis 2 histo patterns 1)granulomatous in 2/3 pts-inner 1/2 of media -mononuc infiltrate, giant cells 2)absent granulomas, nonspecific panarteritis -lymphos, macs, PMNs, eos-NO GIANTS -occasional fibrinoid necrosis BOTH->collagenous thickening of vessel wall -can look just like aging aa. PATHOGENESIS-unknown cause-TCI suspected CLINICAL-fatigue, wt loss, fever -facial pain; severe, unilateral HA along aa. -vessel painful to palpation -abrupt visual Sx in 1/2 pts-diplopia to total loss -visceral vessels->myocardial, GI, neuro problems DX-biopsy, histologic confirmation-2-3cm Bx -(-)Bx doesn't r/o-1/3 are negative TX-anti-inflammatory agents Healing->intimal thickening