Vasculitis Flashcards

(53 cards)

1
Q

Pathogenesis of Vasculitis

A

Deposition of circulating immune complexes
activation of complement
damage to vessel walls
leukocytoclastic changes
necrosis and thrombosis
distribution and symptoms determined by vessel size

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2
Q

Clinical manifestations of large vessels

A
Limb claudication
Asymmetric BP
absence of pulses
bruits
aortic dilation
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3
Q

Clinical manifestations of medium vessels

A
cutaneous nodules
ulcers
arthritis
livedo reticularis
digital gangrene
mononeuritits multiplex
microaneurysms
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4
Q

Clinical manifestations of small vessels

A
purpura
vesiculobullous lesions
urticaria
alveolar hemorrhage
arthritis
cutaneous extravascular necrotizing granulomas
splinter hemorrhages
uveitis
epislceritis
scleritis
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5
Q

Constitutional symptoms of vasculitis

A

fever
weight loss
malaise

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6
Q

large vessel vasculitis

A

takayasu arteritis

giant cell arteritis (temporal arteritis)

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7
Q

medium vessel vasculitis

A

polyarteritis nodosa

kawasaki disease

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8
Q

small vessel vasculitis - ANCA associated

A

Microscopic polyangiitis
Granulomatosis w/ polyangiitis(wegeners)
Eosinophilic granulomatosis w/ polyangitis (churg-strauss)

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9
Q

small vessel vasculitis - Immune complex

A

Anti - GBM disease
cryoglobulinemic vasculitis
IgA vasculitis (henoch - schonlein)
Hypocomplementemic urticarial vasculitis (anti C1q)

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10
Q

variable vessel vasculitis

A

Behcets disease

Cogan’s syndrome (igG4-RD?)

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11
Q

single organ vasculitis

A

Cutaneous leukocytoclastic angiitis

primary CNS vasculitis (string of beads)

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12
Q

vasculitis assd w/ systemic disease

A

lupus vasculitis
rheumatoid vasculitis
sarcoid vasculitis

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13
Q

Vasculitis assd w/ probably etiology

A
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
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14
Q

constitutional vasculitis symptoms

A

fever, wt loss, fatigue, weakness

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15
Q

MSK vasculitis symptoms

A

arthralgia/itis, myalgia

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16
Q

Cutaneous vasculitis symptoms

A

palpable purpura!, nodules, urticaria, livedo reticularis, phlebitis, ischemia

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17
Q

Pulmonaryvasculitis symptoms

A

hemorrhage, nodules, inflitration, cavities

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18
Q

renal vasculitis symptoms

A

nephritis, infarction, hypertension

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19
Q

Neurologic vasculitis symptoms

A

cephalgia, mononeuritis multiplex, stroke

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20
Q

Lab vasculitis symptoms

A

anemia, increased ESR, abnormal LFT, hematuria

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21
Q

Palpable purpura indicate

A
idiopathic 50%
Infection 20% necrotizing vasculitis
Inflammatory 20% leukocytoclastic vasculitis
Medications 5%
malignancy 5%
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22
Q

Treatment of vasculitis

A

Induce remission - glucocorticoids, cyclophosphamide(P. jiroveci prophylaxsis)
maintenance: AZA/MTX/mycophnolate mofetil

Rituximab - maintaining remission

23
Q

Takayasu arteritis criteria

A

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

24
Q

Takayasu treatment

A

“pulseless disease”

steroids

25
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 ```
26
disease assd with temporal arteritis
polymyalgia rheumatica - PMR aches/pains in shoulders, hip/thigh, stiffness proximal pain and stiffness - no weakness
27
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 ```
28
Giant cell arteritis diagnosis
temporal biopsy for granulomatious thickening | normal -> MRI, CT angiogram for vessel narrowing
29
Polyarteritis nodosa
``` Hypertension D BP > 90 Livedo reticularis Abdominal pain Testicular pain/tenderness Mono(foot drop)/polyneuropathy heart/brain involvement (not lungs) ```
30
Labs for polyarteritis nodosa
azotemia, anemia hep B virus skin/nerve/muscle/testicle biopsy angiogram of renal/mesenteric/hepatic artery
31
polyarteritis nodosa treatment
induce remission - pulse cyclophosphomide and steroids | maintenance: azthioprine, MTX, mycophenolate mofetil
32
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) ```
33
Kawasaki treatment and complications
High dose ASA or IVIG Coronary artery aneurysm Myocarditis (do an echo)
34
Histopathology of small vessel vasculitis - immune complex assd
Histopathology of skin with PMN's and mononuclear cells leading to fibrinoid necrosis and WBC fragments
35
Urticarial vasculitis
urticaria>24 hours=chronic->leukocytoclasitc nuclear debris fever/angioedemia/reynauds/arthralgias/uveitis/copd ANTI C1q antibodies!!!! aka anti C1q vasculitis
36
causes of urticarial vasculitis
viruses(hep b/c), malignancies, CTDs, drugs
37
treatment of uriticarial vasculitis
acute: steroids and Azathioprine chronic: mycophenolate mofetil and rituximab
38
Henoch Shoenlein purpura
``` purpura 100% arthritis (60-85%) nephropathy 10-50 Gi abdominal pain w/bleeding 85% IgA immune complexes typically children TX: prednisone? ```
39
clinical symptoms of mixed cryoglobulinemia
``` raynauds cutaneous ulceration cold-induced urticaria digital gangrene polyarthralgias purpura nephritis peripheral neuropathy ```
40
Mixed cryoglobulinemia
assd w/ hep C, Sjogrens, SLE, SBE, osteomyelitis POLYCLONAL mixed IgG-IgM complexes types 2 and 3 LOW C4
41
C-ANCA vs P-ANCA
C-anca: anti PR-3 | P-anca: anti MPO
42
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
43
microscopic polyangiits
looks like wegeners - no upper resp involvement and no granulomas P - ANCA
44
microsocopic polyangitis
cyclophosphamide, prednisone, aza, rituximab
45
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
46
eosinophilic granulomatosis w/ polyangitis - Churg-Strauss
``` Asthma Eosinophilia (> 10% of WBC) Mono or polyneuropathy transitory pulmonary infiltrates paranasal sinus abnormalitis biopsy w/extravascular eosinophils ```
47
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
48
Cogans syndrome
like behcets but chlamydia ABs agaist cornea and inner ear- part of IgG4-RD
49
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
50
Behcets treatment
``` steroids aza chlorambucil colchicine interferon thalidomide ```
51
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
52
string of beads
primary angiitis of the CNS - single organ vasculitis
53
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