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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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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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