Vasculitis Flashcards
Vasculitis in general
Presence of inflmmation in BV walls with reactive damage to surrounding structures
Loss of integrity compromoises blood flow leading to necrosis and ischemia
Large
Medium
SMall
Large - aorta of major branches
Medium - main visceral arteries and branches
Small - intraparenchymal arteries, etc.
Patho of vasculitis
Activation of CM path in large vessel with promonnet granuloma formation…antigens deposited in vascular walls and activate CD4 T cells…monocyte recruitment mediates endothelial damage
AB of vasculitis
ANCA - anti-neutropghil cytoplasm AB
All seem to be intitiated by inlammevent in resp tract
Previously shielded neirtophil antigens are exposed and get ANCAs
AB formation activates neutrophils and B cells
Aberrant T cells result in granuloma formation
Kawasaki follows pattern of IC antigen-mediated illness resulting in stimulation of CD8 T cells and specific IgA formation by plasma cells
Epi of vasculitis
IgA is most common childhood (peak at 5-7 yrs)
Giatn cell is most common in adults (over 50)
CM of vasculitis
Constitutional sx promiinent and preced others
Subacute course - weeks to months…Kawasaki is exception which is acute
Most have strkining inflammation with ESR and CRP elevated
Pain is often present
Multisystem dz
Sx of
Giant cell Takayasu Medium vessel Jawasaki Polyarteritis nodosa Small veseel
Branches of carotid most commonly temporal so jaw claudication, diminisehd temporal pulse and presence of bruti
Aoritc arch so arm claudication, asymmetric BP, absent peripheral pulses
Poor tissue and organ profusion
Coronaies so asymptomatic
Momneuritis multilex, GI bleeding and pain, and renal insufficency and cutaneous ulcers
Eye, kidney, lung
Giant cell and polymyalgia rheumatica general
Giant cell - inflammation of one or more brnahces of carotid…mostly temporal
Antigens deposited in vascular walls and activate CD4s…monocytes recruit things to damage vessel walls
Macros responsible for granuloma formation
CM of GCA and PR
GCA - high, spiking fevers, and headaches over 50 y/o…scalp pain, jaw claudication…temporal arteyr enlarged or without a pulse…bruits may be heard…anterior ischemic optic neuropathy is a risk
PMR - symmetric pain, aching, morning stiffness of shoulder, neck, hips, and thights…also all over 50…maybe decreased ROM of shoulders and hips…most of the time tenderness and weakness absent
Dx of GCA and PMR
GCA - over 100 ESR…temporal artery biopsy demonstrating panarteritis with inflamm mononuclear infiltrates with giant cell ofrmation
PMR - iover 50 ESR…clinical and supported if resolved by GCs
Anemia of chronic dz common in both
Negative testing for autoanditbodies is expected
Managment of GCA and PMR
GCA- preserve vision and reduce sx…immeditely start on high dose prednisone whilebiopsy arranged
PMR - low dose prednisone
IgA vasculitis in general and comps
Henoch-shonlein purpura
Fall and winter in children
Almost all get palpable purpura
1/2 get migratory oligoarticular arthritis/arthralgias of hips/knees/ankles
25% ab pin
1/2 with renal dz with hemato/proeinuria
IgA presentation/dx, anageent, prognosis
Skin biopsy shoews leukocytoclastic vasculitis with IgA deposition
Kidney biopsy shows IgA deposition
Most just get adquate hydration…if renal, then give GCs, immunosuppressants, or cyclophosphamide
Generally self-limited dz with spontaneous resolution over the next month
Kawasaki dz general
Most under 5 y/o
Predom in coronary
Main manifestation is fever for at least 5 days
Non-exudative conjucntiivities Oral mucosal changes Skin rash of polymorphous red rash with desquamation Extremitiy changes (edema of hands/feet) Non-surppurative cervical adenitis
Most resolve over 2-3 weeks…25% get coronary anyeursm
IVIG and oral aspirin is tx
Granulomatosis with polyangiitis general
Wegeners
Small vessel involvement in upper and lower respiratoy tract and kindye
95% upper airway involvement with sinus pain and blood/purulet nasal discharge
Pulm involvement in about 90%
Renal dz in about 80%
Skin lesions in about 50%
Dx with positive ANXA with more specific antiproteinase-3 AB…patholigc necrotizing granulmatous vasculitis
Tx is high dose GCs with or without cyclophosphamide
MPA`
Microscopic polyangitis
Necrotizing vasculitis with few or no immune complexes (unlike PAN) and no granulomatous (unlike GPA)
Predominant kidney and nervous involvement
Renal in 80%
2/3 have mononeuritis multiplex
Alveolar hemorrhage in about 15%
C-ANCA association in about 75%….unlike GPA, more specific AB is myeloperoxidase…pauci-immune glomeruloneprhitis or vasculitis
Tx is idential to GPA
Eosinophilis granulomatosis with polyangitis
EDGPA - Chrug-strauss)
Asthma, peripheral and tissue eosinophilia and multisystem vasculitis
Pulmonary in 90%
Neuro in 75%
May get recurrent allergic rhinits/sinusitis
Peripheral esosionphilia (over 1000)…1/2 test positive of C-ANCA, specificaly anti-myeloperoxidase AB (like MPA)
Necrotizing vasculitis with eosinophil infiltration of tissue
High dose GCs alone
PAN
Polyarteritis nodosa
Renal and viscerla arteries most common
Pulmonary involvement is absent and more GI involvement
Diagnosis is necortizing vasculitis with prominent neutrophil infiltration of the tissue
Aggressive therapy with GCs and cyclophopsphamide
Takayasu arteritis
Large veels (aortic arch and its branches)
More prevalent in adolescent girls and young women
Arm claudication, Raynaud, diminished absent pulse
Dx made with arteriography that demonstrates irregular vessel walls, areas of stenosis, post-stenotic dilation and aneurysm formatin
High dose GCs are mainstay
zcryoglobulinemic vasculitis
Cold-precip oligo or polyclonal ABs
Most commonly with Hep C
Palpable purpua, arthrtisi, peripheral neuropathy, and glomeruloneprhots
Cryoglobulins and RF tested should be positive
Low C3, C4, CH50
Test for hep C and tx the Hep C with antiviral
