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    VASCULITIS Syndrome

    BY

    PROF : AbdelRahman A Mokhtar

    2013

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    Case presentation : History

    A 45-year-old man presents with severe dyspneaand cough. He was in excellent health until 4

    weeks ago when he developed a sore throat and

    fever. Over the past 2 weeks, he has noticed

    reddish ulcers on his legs, episodes of dark urine,and migratory arthralgias. He reports a past

    history of heavy alcohol use and acknowledges

    occasional recreational drug use

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    Case : Objective Findings

    Diffuse pulmonary rales andrhonchi

    No detectable heart

    murmurs or S3

    Palpable ulcerative rash

    over the legs

    No synovitis

    Hgb = 9.8; WBC = 23,000;ESR = 68; Creatinine = 2.8

    UA = 50 RBCs with casts

    Oximetry = 85% O2 saturation

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    Approach to MultisystemInflammatory Disease

    How should you approach a patient who presents

    with multisystem inflammatory disease?

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    SLE

    Vasculitis

    V. MimeticDiseases That

    Can Present as

    Vasculitic

    Syndromes

    Diagnostic Considerations in PatientsWith Multisystem Inflammation

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    What is vasculitis:

    PathologistInflammatory destruction ofblood vessels

    Infiltration of vessel wallwith inflammatory cells

    u Leukocytoclasis

    u Elastic membrane disruption

    Fibrinoid necrosis of thevessel wall

    Ischemia, occlusion,thrombosis

    Aneurysm formation

    Rupture, hemorrhage

    Rheumatologist A clinicopathologic

    process characterized by

    inflammatory destruction

    of blood vessels thatresults in occlusion or

    destruction of the vessel

    and ischemia of the

    tissues supplied by that

    vessel.

    Systemic vasculitides

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    Definition

    The vasculitides are a set of related disorderscharacterized by blood vessel inflammation leading

    to tissue or endorgan injury.

    Inflammation of vessels

    with inflammatory cells

    in and/or accentuated

    around the vessel wall

    Accompanied by vascular damage: Leukocytoclasia

    Endothelial cell necroses

    Muscle cell necroses

    Fibrin & thrombi

    Degeneration of collagen

    Differentiated from other

    vascular disorders by

    the presence of

    inflammation of the vessel

    wall as compared to blandvasculopathy

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    Primary Secondary

    ?D. category Example

    Rheumatic diseases RA, SLE, MCTD,DM, RF, SS

    Infectious diseases HBV, HCV, SBE

    Cryoglobulinemia myeloma-associated,

    2ry to lymphoma, HCV

    Vasculitis may be primary or secondary (more common).

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    Vasculitis: cassification

    Large-vessel vasculitisu Aorta and the great vessels (subclavian, carotid)

    u Claudication, blindness, stroke

    Medium-vessel vasculitis

    u Arteries with muscular wall

    u Mononeuritis multiplex (wrist/foot drop),

    mesenteric ischemia, cutaneous ulcers

    Small-vessel vasculitisu Capillaries, arterioles, venules

    u Palpable purpura, glomerulonephritis, pulmonary

    hemorrhage

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    Vasculitis: Long-term Damage

    Large-vessel vasculitisu Blindness, Stroke

    u Claudication: Angina of the arms

    Medium-vessel vasculitisu Foot drop: inability to lift a foot

    u Wrist drop: inability to lift a hand

    u Cutaneous ulcerations

    Small-vessel vasculitis

    u Oxygen dependence

    u Renal insufficiency/failure

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    Classification

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    Wegeners

    Churg-Strauss

    MPA

    Necrotizing Granuloma

    Hypereosinophilia

    Sinusitis

    Subglottic stenosis

    Pulmonary nodulesOrbital pseudotumor

    Asthma

    Pulmonary infiltrates

    Myocarditis

    Pulmonary capillaritisGlomerulonephritis

    Sensory neuropathy

    Mononeuritis multiplex

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    http://egyptinphotos.tumblr.com/image/29926418091
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    ROAD MAP for DIAGNOSSIS

    There are five important questions to ask whenfaced with a patient with possible vasculitis

    (depending on clinical presentation):

    (1) Is this a condition that could mimic thepresentation of vasculitis?

    (2) Is there a secondary underlying cause?

    (3) What is the extent of vasculitis?

    (4) How do I confirm the diagnosis of vasculitis?

    (5) What specific type of vasculitis is this?

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    Diseases That Can Present as VasculiticSyndromes.

    (1) Is this a condition that could mimic the

    presentation of vasculitis?

    When to Consider Vasculitis Mimics (the RedFlags)

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    Clinical Features Suggesting Vasculitis

    Multisystem inflammatory disease Rapidly progressive major organ dysfunction

    Constitutional symptoms (fever, weight loss)

    High ESR, severe anemia, thrombocytosis

    Evidence of small-vessel inflammation:

    In the kidneys = active urinary sediment

    In the lungs = hemoptysis, dyspnea

    In the skin = palpable purpura/hemorrhage Acute neurologic changes

    Footdrop

    Altered mental status

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    Vasculitis Vasculopathy

    Diseases That Can Present asVasculitic Syndromes

    Infectious diseases

    Bacterial endocarditis

    HIV infections Viral hepatitis

    Paraneoplastic

    syndromes Atrial myxoma

    Coagulopathies/thrombotic

    microangiopathies

    Cholesterol emboli

    syndrome

    Toxic drug effects Ergots

    Cocaine

    Amphetamines

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    (the Red Flags)

    Presence of a heart murmur

    Necrosis of lower extremity digits

    Splinter hemorrhages

    Prominent liver dysfunction History of recreational drug use

    History of high-risk sexual activity

    Prior diagnosis of neoplastic disease Unusually high fevers

    (2) I h d d l i

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    (2)Is there a secondary underlyingcause?

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    (3)What is the extent of vasculitis?

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    (4) How do I confirm the diagnosis of vasculitis?

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    (5) What specific type of vasculitis is this?

    Consider tissue biopsyof affected organ to

    determine Vessel size

    Histologic features of vessel

    inflammation

    Vessel wall necrosis

    Granulomas/giant cells

    Immune complex and/orC3 deposition

    Obtain tissue immediately

    Obtain tissue from most

    signi fi cantly involved organLung=usually

    diagnostic

    Kidney=often

    diagnostic

    Skin=sometimes

    diagnostic, but always

    easy to obtai n

    Nasal/sinus mucosa=

    easy to obtain butmay be

    non diagnosticObtain an adequate specimen

    Obtain immunofluorescence studies

    Immunoglobulin depositionC3 deposition

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    Consider angiography of mesenteric or cerebral vesselsas clinically indicated

    Tests suggesting immune complex formation

    and/or deposition Rheumatoid factor and cryoglobulins

    Antinuclear antibodies (ANA)

    Low C3 or C4 levels Tests suggesting necrotizing vasculitis without

    immune complex deposition

    Antineutrophil cytoplasmic antibodies (ANCA)

    Tests suggesting systemic inflammation Erythrocyte sedimentation rate (ESR)

    C-reactive protein (CRP)

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    Antineutrophil Cytoplasmic Antibodies

    ANCA byimmunofluorescence

    methods

    c-ANCA = Wegeners

    disease (60% to 90%)

    p-ANCA = microscopic

    polyangiitis (MPA)

    (50% to 80%),UC (40% to 80%),

    Crohns (10% to 40%)

    Hoffman GS.Arth Rheum. 1998;41(a):15211537.

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    Case 2: History

    A 36-year-old female is seen for migratoryarthritis of 6 months duration. She also reports

    some fatigue and a photosensitive skin rash.

    notes:

    Patchy hair loss 4 months ago that regrew

    Aphthous-like mouth ulcers every 4 to 6 weeks

    A diagnosis of walking pneumonia made last

    month based on symptoms of pleuritic chestpain

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    Case 2: Objective Findings

    Pain with mild synovitisover the MCPs and PIPs

    Rash over her face, legs,

    and trunk Hgb = 12.1; ESR = 33

    UA = 3+ protein

    ANA = 1:640 titer

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