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Vascuitis
Is a clinico pathologic process characterized by inflammation
and damage to blood vessels.
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Approaches to the classification of vasculitis
Clinical syndromes
Vessel size
Laboratory markers
Pathology
Etiology
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Vasculitis: Classification by vessel size
Small vessels (venules, arterioles)
Drug-induced and serum sickness
Henoch-Schönlein purpura
Cryoglobulinemia
Vasculitis associated with
systemic rheumatic diseases
Vasculitis associated with
malignancy
Hypocomplementemic urticarial
vasculitis
Vasculitis associated with
infections
Small and medium muscular
arteries
Classic PAN
Microscopic polyangiitis
Wegener’s granulomatosis
Churg-Strauss vasculitis
Kawasaki syndrome
Rheumatoid vasculitis
SLE
Large arteries
Giant cell or temporal arteritis
Takayasu arteritis
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Vasculitis: Clinical syndromes
Leukocytoclastic or hypersensitivity vasculitis
Classic polyarteritis nodosa
Kawasaki syndrome
Microscopic polyangiitis
Wegener’s granulomatosis
Churg-Strauss vasculitis or allergic granulomatosis
Giant cell or temporal arteritis
Takayasu arteritis
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When to suspect vasculitis: clinical features
Multisystem disease
Unexplained constitutional signs and symptoms
Skin lesions (palpable purpura)
Ischemic vascular changes (gangrene, claudication, Raynaud’s
phenomenon, livedo)
Glomerulonephritis
Mononeuritis multiplex
Myalgia, arthralgia/arthritis
Abdominal (intestinal angina) or testicular pain
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Takayasu's Arteritis (pulseless disease)
- Second or third decade
- F: M 8:1
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Clinical
Constitutional system's +Ischemic feature
. Fever
. Fatigue
. Polyarthralgia
. Muscular pain
Sign and symptoms due to ischemic change depend on the site of vascular lesion:
- Diminished or weak artpulsation}
- Low blood pressure }
- Muscular weakness} In the Arms
- Transient visual disturbance} cerebral ischema
- - Syncope and vertigo }
-Hypertension --------renal artery
- Intermittent claudication-------iliac arteritis
- Vascular murmurs and local tenderness--------stenosis
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ACR classification criteria: Takayasu arteritis
Must have at least 3 of the 6 criteria present.
Age < 40 years at disease onset
Claudication of extremities
Decreased brachial artery pulse
BP difference > 10 mm Hg between arms
Bruit over subclavian arteries or aorta
Arteriogram abnormality: occlusion or narrowing in aorta or main
branches
Sensitivity 90.5% and specificity 97.8%.
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Takayasu disease: arteritis (aortograms)
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Takayasu disease: arteritis (pulmonary angiogram)
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Takayasu disease: arteritis (aortogram)
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Takayasu’s arteritis: aneurysmal dilatation, ascending and
descending aorta (MRI arteriography)
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Polymyalgia rheumatica: epidemiology
Onset after age 50 and usually after 60
Highest incidence in individuals of Northern European extract
Female:male ratio 2:1
Incidence approaches 1% in older populations
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Polymyalgia rheumatica: clinical and laboratory features
Pelvic and shoulder girdle aching
Morning stiffness
Rapid response to low doses of corticosteriods
Anemia
Elevated ESR and C-reactive protein
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Giant cell arteritis
Clinical features
Headache
Temporal artery
abnormality
Jaw claudication
Visual loss; diplopia
Extremity claudication
PMR symptoms
Weight loss, fever
Respiratory symptoms
Laboratory features
Elevated ESR
Elevated CRP
Other acute-phase reactants
Anemia
Elevated alkaline phosphatase
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Relationship of polymyalgia (PMR) to giant cell arteritis
(GCA)
40-60% of patients with GCA have PMR symptoms; in about half of these
individuals, PMR is their first manifestation of GCA
10-15% of patients with PMR have GCA
PMR symptoms can occur before, with, or after GCA symptoms in
patients with GCA
GCA can develop long after onset and treatment of PMR
Treatment of GCA requires larger doses of corticosteroids than does
treatment of PMR
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Polymyalgia rheumatica: differential diagnostic possibilities
Fibromyalgia
Depression
Occult infection
Occult malignancy
Hypothyroidism
Temporal arteritis
Viral myalgia
Rheumatoid arthritis
Polymyositis
Multiple myeloma
Osteoarthritis
Rotator cuff disease
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Giant cell arteritis: forehead
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Giant cell arteritis: retinal ischemia
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Polyarthritis nodosa
-middle age (45 years) -M:F 2.5-1
Pathology: there is fibrinoid necrosis and infarction
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clinical manifestation.
-Fever-Malasia - Weight loss and myalgia
-Neurological-Mononeuritis multiplex (arthritis of the vasa
nervorum)
-Abdominal pain (acute cholecystitis , pancreatitis,appenditis,G1
haemorrhage) -50%
-Renal : -Hematuria -proteinurea
-Hypertension -acute/chronic renal failure
-Cardiac: -coronary arteritis:infarction
-Heart failure -Pericarditis
Skin: -subcutanous hermorrhage -rash-puprpua 20-30%
-livedo reticulars -nodules
-Arthrlgia
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ACR 1990 criteria for classification of polyarteritis nodosa
Must have at least 3 of the 10 criteria present.
Weight loss > 4 kg
Livedo reticularis
Testicular pain or tenderness
Myalgias, weakness, or leg tenderness
Mononeuropathy or polyneuropathy
Diastolic BP > 90
Elevated BUN/creatinine
Hepatitis B virus
Arteriographic abnormality
Biopsy of small or medium artery containing PMN
Sensitivity 82.2% and Specificity 86.6%
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Polyarteritis nodosa: subcutaneous nodules, hand
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Polyarteritis nodosa: wrist drop
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Investigation
-Anemia, leukoeytosis and ESR (up)
-ANCA-ve
-HbsAg +ve %30
-Angiography: microanyrusm.
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Polyarteritis nodosa (angiogram)
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Polyarteritis nodosa: leg (angiogram)
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Wagener granulomatosis:
-Upper + lower respiratory tract + kidney
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-Sinusitis -otitis -Rhinitis -epistaxis -
oralulcers
-Hear loss -nasal ulceration
-Cough -Hemoptysis -shortness of
breath -pleuritis
-Hematourea -renal failure
-Fever -arthrlgia -headache - skinrash -
ocular inflammation.
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ANCA 90%
Chest-x-ary:migratory pattern
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Wegener’s granulomatosis: face
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Wegener’s granulomatosis: face
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Wegener’s granulomatosis: eye involvement
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Wegener’s granulomatosis: lungs (radiograph)
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Wegener’s granulomatosis: lungs (radiograph)
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The chug Strauss syndrome:
Males in their fourth decade
-Rhinitis –B.Asthma - esoinophilia +systemic
vacuities
ANCA 60%
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ACR 1990 criteria for classification of Churg-Strauss
syndrome
Must have at least 4 of the 6 criteria present.
Asthma
Eosinophilia > 10%
Neuropathy, mono or poly
Pulmonary infiltrates, non-fixed
Paranasal sinus abnormality
Extravascular eosinophils
Sensitivity 85% and specificity 99.7%.
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-Microscopic vacuitis (polyangilitis)
ANCA positive
-Lung and Kidney
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Polyarteritis nodosa (PAN) vs. microscopic polyangiitis
(MPA)
PAN
Small/medium muscular artery
Renal vascular nephropathy
No lung involvement
ANCA negative
MPA
Small/medium arteries, arterioles,
venules, capillaries
Glomerulonephritis
Lungs involved in up to one third
ANCA positive
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Hypersensitivity Vasculitis
- Inflammation of small vessels (venules-capillaries and arterioles)
- Exogenous stimuli proved or suspected
. Henoch – schonlein purpura
. Serum sickness
. Drug-related vasculitides
. Infection
- Endogenous antigen
. Neoplasms
. C T D
. Congenital deficiencies of the complement system
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Skin: Palpable purpura (papules, nodules, vesicles,
bullas, ulcers, or recurrent urticaria)
Systemic: Fever –malaise, myalgia , anorexia
Extra cutaneous : joint-renal-G I –peripheral
neuropathy
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ACR classification criteria: Henoch-Schönlein purpura
Must have at least 2 of the 4 criteria present.
Palpable purpura
Age < 20 years at disease onset
Bowel angina (abnormal pain after meals or bowel ischemia usually with
bloody diarrhea)
Granulocytes in walls of arterioles or venules on biopsy
Sensitivity 87.1% and specificity 87.7%
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Palpable purpura: back
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Henoch-Schönlein syndrome: legs
(photomicrograph and clinical)
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Vasculitis: purpuric eruption, feet
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Henoch-Schönlein syndrome: purpura, legs
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Hemorrhagic lesions, ankle and foot
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Dermal vasculitis: ankle and foot
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Livedo reticularis
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ANCA - Associated vacuities
Cyclophosphamide + glucocorticoid for remission induction of
generalized primary small and medium vessel vacuities
Remission – maintenance therapy with a combination of low dose
steroid and either azathioprine , leflunomide or methotrexate
Alternative immunomodulatory therapy choices should be
considered for patients who don’t achieve remission or relapse on
maximal doses of standard therapy
. Intra venous immunoglobulin
. Mycophenolate
. Infliximab
. Rituximab
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High dose gluco corticoid therapy for induction
remission in large vessel vacuities
Imuno suppression (MTX , Azathioprine) for Takaysuo
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Behcet`s syndrome
F:M 2:1
Clinical features
Apthae:
Oral: . herpetic form
. Individual lesion's on buccal mucosa,
Gingival, lips, tongue
. Painful-heal without scaring over 1-3
weeks
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Behçet’s syndrome: ulceration, tongue
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Behçet’s syndrome: ulceration, tongue
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Behçet’s syndrome: ulceration, tongue and labia oris
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Behçet’s syndrome: ulceration, mouth
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Genital:
. Scrotum and penis in males
. Vulva and vague in females
. Similar to oral ulcers
. Tendency toward scaring
. Recur less frequently
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Behçet’s syndrome: ulceration, vulva
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Behçet’s syndrome: scrotal ulcers
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Continuous lesion's :
. Erythema – nodsum-like lesion
. Pyoderma gangrenosum –like lesion
. Acnei form
. Pseudo folliculitis
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Ophthalmologic features:
. Anterior uveitis
. Posterior uveitis
. Hypopyon
. Retinal vascuitis
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Behçet’s syndrome: hypopyon, eyes
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Arthritis:
. Non erosive
. Symmetrical or asymmetrical
. Mono – oligo or poly arthritis
. Knee, wrist, ankles and elbows
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C N S ( neuro- behcet ):
. Brain – stem
. Venous sinus syndrome syndrome
. Headache
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G I :
. Diarrhea
. Abdominal
Vascular:
. Arterial and venous thrombosis
. Aneurysm
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Criteria for diagnosis of Behçet’s disease
Recurrent oral ulceration plus two of the following:
Recurrent genital ulceration
Eye lesions (anterior/posterior uveitis or cells in vitreous or
retinal vasculitis)
Skin lesions (E. Nodosum, pseudofolliculitis, papulopustular
lesions or acneiform nodules)
Positive pathergy test
Sensitivity 91% and specificity 96%
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Behcet’s disease: aneurysms, popliteal and femoral arteries
(angiogram)
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treatment
-skin + mucosal inolvement
-local steroid (mouth + genitalulcers)
-acne –like lesion Topical
-colchicines
-Azathioprine + INF +anti TNF
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Eye
-azathioprin + systemic corticosteroids
-Severe eye disease: cyclosporine A, or infliximab in
combination with azthioprine
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Vessel disease
-DVT . corticosteroid
. Azathioprine
. Cyclophosphamide
. Cyclosporine
- Pulmonary or peripheral aneurysms
. Cyclophosphamid
. Steroid
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Arthritis---------Colchicine
CNS--------------- . Steroid
. INF-&
. Azathioprine
. Cyclophosphamide
. Methotrexate
. TNF antagonists