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MSK 2 > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

Vasculitis in general

A

Presence of inflmmation in BV walls with reactive damage to surrounding structures

Loss of integrity compromoises blood flow leading to necrosis and ischemia

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2
Q

Large
Medium
SMall

A

Large - aorta of major branches

Medium - main visceral arteries and branches

Small - intraparenchymal arteries, etc.

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3
Q

Patho of vasculitis

A

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

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4
Q

AB of vasculitis

A

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

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5
Q

Epi of vasculitis

A

IgA is most common childhood (peak at 5-7 yrs)

Giatn cell is most common in adults (over 50)

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6
Q

CM of vasculitis

A

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

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7
Q

Sx of

Giant cell 
Takayasu
Medium vessel
Jawasaki
Polyarteritis nodosa
Small veseel
A

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

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8
Q

Giant cell and polymyalgia rheumatica general

A

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

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9
Q

CM of GCA and PR

A

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

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10
Q

Dx of GCA and PMR

A

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

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11
Q

Managment of GCA and PMR

A

GCA- preserve vision and reduce sx…immeditely start on high dose prednisone whilebiopsy arranged

PMR - low dose prednisone

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12
Q

IgA vasculitis in general and comps

A

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

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13
Q

IgA presentation/dx, anageent, prognosis

A

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

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14
Q

Kawasaki dz general

A

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

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15
Q

Granulomatosis with polyangiitis general

A

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

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16
Q

MPA`

A

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

17
Q

Eosinophilis granulomatosis with polyangitis

A

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

18
Q

PAN

A

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

19
Q

Takayasu arteritis

A

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

20
Q

zcryoglobulinemic vasculitis

A

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

MSK 2 (24 decks)

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