Vasculitis Flashcards
Pathogenesis of Vasculitis
Deposition of circulating immune complexes
activation of complement
damage to vessel walls
leukocytoclastic changes
necrosis and thrombosis
distribution and symptoms determined by vessel size
Clinical manifestations of large vessels
Limb claudication Asymmetric BP absence of pulses bruits aortic dilation
Clinical manifestations of medium vessels
cutaneous nodules ulcers arthritis livedo reticularis digital gangrene mononeuritits multiplex microaneurysms
Clinical manifestations of small vessels
purpura vesiculobullous lesions urticaria alveolar hemorrhage arthritis cutaneous extravascular necrotizing granulomas splinter hemorrhages uveitis epislceritis scleritis
Constitutional symptoms of vasculitis
fever
weight loss
malaise
large vessel vasculitis
takayasu arteritis
giant cell arteritis (temporal arteritis)
medium vessel vasculitis
polyarteritis nodosa
kawasaki disease
small vessel vasculitis - ANCA associated
Microscopic polyangiitis
Granulomatosis w/ polyangiitis(wegeners)
Eosinophilic granulomatosis w/ polyangitis (churg-strauss)
small vessel vasculitis - Immune complex
Anti - GBM disease
cryoglobulinemic vasculitis
IgA vasculitis (henoch - schonlein)
Hypocomplementemic urticarial vasculitis (anti C1q)
variable vessel vasculitis
Behcets disease
Cogan’s syndrome (igG4-RD?)
single organ vasculitis
Cutaneous leukocytoclastic angiitis
primary CNS vasculitis (string of beads)
vasculitis assd w/ systemic disease
lupus vasculitis
rheumatoid vasculitis
sarcoid vasculitis
Vasculitis assd w/ probably etiology
hep C assd cryoglobulinemic vasculitis hep B assd vasculitis syphilis assd vasculitis drug assd immune complex vasculitis drug assd ANCA-assd vasculitis cancer assd vasculitis
constitutional vasculitis symptoms
fever, wt loss, fatigue, weakness
MSK vasculitis symptoms
arthralgia/itis, myalgia
Cutaneous vasculitis symptoms
palpable purpura!, nodules, urticaria, livedo reticularis, phlebitis, ischemia
Pulmonaryvasculitis symptoms
hemorrhage, nodules, inflitration, cavities
renal vasculitis symptoms
nephritis, infarction, hypertension
Neurologic vasculitis symptoms
cephalgia, mononeuritis multiplex, stroke
Lab vasculitis symptoms
anemia, increased ESR, abnormal LFT, hematuria
Palpable purpura indicate
idiopathic 50% Infection 20% necrotizing vasculitis Inflammatory 20% leukocytoclastic vasculitis Medications 5% malignancy 5%
Treatment of vasculitis
Induce remission - glucocorticoids, cyclophosphamide(P. jiroveci prophylaxsis)
maintenance: AZA/MTX/mycophnolate mofetil
Rituximab - maintaining remission
Takayasu arteritis criteria
Age < 40
2 phases - early inflammatory/ late occlusive
decreased brachial artery pulse (>10 diff b/w arms)
bruits over subclavian/aorta
arteriogram - dilation/aneurysma/narrowing in aorta/main branches
claudication of extremities
Takayasu treatment
“pulseless disease”
steroids
arteries affected by temporal arteritis
inflammation of BV in face aortic aneurysms or stenosis of arch vessels aortic insufficiency murmor or bruits temporal ophthalmic facial carotid can cause blindness
disease assd with temporal arteritis
polymyalgia rheumatica - PMR
aches/pains in shoulders, hip/thigh, stiffness
proximal pain and stiffness - no weakness
Giant cell arteritis
Age > 50, FUO, paraneoplastic PMR - proximal pain and stiffness abnormal artery biopsy, temporal artery abnormality(tender/decreased pulse) Elevated ESR >50, anemia, low albumin Increased Alk phos mistaken for tension headache till go blind jaw gets tired chewing
Giant cell arteritis diagnosis
temporal biopsy for granulomatious thickening
normal -> MRI, CT angiogram for vessel narrowing
Polyarteritis nodosa
Hypertension D BP > 90 Livedo reticularis Abdominal pain Testicular pain/tenderness Mono(foot drop)/polyneuropathy heart/brain involvement (not lungs)
Labs for polyarteritis nodosa
azotemia, anemia
hep B virus
skin/nerve/muscle/testicle biopsy
angiogram of renal/mesenteric/hepatic artery
polyarteritis nodosa treatment
induce remission - pulse cyclophosphomide and steroids
maintenance: azthioprine, MTX, mycophenolate mofetil
Kawasaki Disease - small/med vessel vasculitis
Warm CREAM Warm - fever > 5d + 4 of 5 Conjunctivits Rash Erythema palms and soles Adenopathy, cervical Mucous Membrane(dry red strawberry tongue)
Kawasaki treatment and complications
High dose ASA or IVIG
Coronary artery aneurysm
Myocarditis (do an echo)
Histopathology of small vessel vasculitis - immune complex assd
Histopathology of skin with PMN’s and mononuclear cells leading to fibrinoid necrosis and WBC fragments
Urticarial vasculitis
urticaria>24 hours=chronic->leukocytoclasitc nuclear debris
fever/angioedemia/reynauds/arthralgias/uveitis/copd
ANTI C1q antibodies!!!! aka anti C1q vasculitis
causes of urticarial vasculitis
viruses(hep b/c), malignancies, CTDs, drugs
treatment of uriticarial vasculitis
acute: steroids and Azathioprine
chronic: mycophenolate mofetil and rituximab
Henoch Shoenlein purpura
purpura 100% arthritis (60-85%) nephropathy 10-50 Gi abdominal pain w/bleeding 85% IgA immune complexes typically children TX: prednisone?
clinical symptoms of mixed cryoglobulinemia
raynauds cutaneous ulceration cold-induced urticaria digital gangrene polyarthralgias purpura nephritis peripheral neuropathy
Mixed cryoglobulinemia
assd w/ hep C, Sjogrens, SLE, SBE, osteomyelitis
POLYCLONAL mixed IgG-IgM complexes
types 2 and 3
LOW C4
C-ANCA vs P-ANCA
C-anca: anti PR-3
P-anca: anti MPO
ANCA vasculitis
assd w/ pauci-immune systemic necrotizing small vessel vasculitis
ANCA activate neutrophils to damage vessel walls
release factors to stim alt complement path - more nphil
may get granulomatous rxn from extravascular neutrophils - necrosis
microscopic polyangiits
looks like wegeners - no upper resp involvement and no granulomas
P - ANCA
microsocopic polyangitis
cyclophosphamide, prednisone, aza, rituximab
granulomatosis w/ polyangitis - wegners
nasal, otic, ocular, oral inflammation
abnormal chest x-ray
urinary sediment (>5RBC or casts) RPGN - RF -> death
oligoarthritis, neuropathy, uveitis, DVTs
ABs to proteinase 3
C-ANCA
granulomas and patchy necrosis in art and veins
eosinophilic granulomatosis w/ polyangitis - Churg-Strauss
Asthma Eosinophilia (> 10% of WBC) Mono or polyneuropathy transitory pulmonary infiltrates paranasal sinus abnormalitis biopsy w/extravascular eosinophils
variable vessel vasculitis - Behcets syndrome
autoimmunity against heat shock proteins
Triad: painful apthous ulcers(mouth/genitals) 3x - 1 year
eye lesions (pan uveitis)-blindness
arthritis
neurologic - meningoencephalitis, seizures
vascular: E nodosum, phlebitis
Pathergy test - 48 hr after serile needlestic - ulcer/blister
Cogans syndrome
like behcets but chlamydia ABs agaist cornea and inner ear- part of IgG4-RD
Behcets
epidemiology: turkish/middle eastern -
HLA B51!!!
cause: strep sanguis(like HSP), herpes, bowel flora
arteries and veins!
CD4 helper response through TH1 path. No auto ABs
Behcets treatment
steroids aza chlorambucil colchicine interferon thalidomide
familial mediterranean fever
abdominal pain and arthritis (hot ankle rash)
episodic fever
pyrin - protease that normally inactivates IL8 and C5a
thus neutrophil cytokines yield fever
inhibits NF-kB and IL 1
colchicine prevents 2ndary amyloidois - deadly
renal amyloidosis indicated by proteinuria
diagnosis - biopsy proven amyloid
- scintography w/ serum amyloid protein
string of beads
primary angiitis of the CNS - single organ vasculitis
cutaneous leukocytoclastic vasculitis(skin)
Drug reaction
Palpable Purpura - most common vasculitis
inflammation and fibrinoid necrosis of vessel walls and cellular debris in tissue of the skin
