Is a clinico pathologic process characterized by inflammation ......When to suspect vasculitis:...

transcript

Vascuitis

Is a clinico pathologic process characterized by inflammation

and damage to blood vessels.

Approaches to the classification of vasculitis

Clinical syndromes

Vessel size

Laboratory markers

Pathology

Etiology

Vasculitis: Classification by vessel size

Small vessels (venules, arterioles)

Drug-induced and serum sickness

Henoch-Schönlein purpura

Cryoglobulinemia

Vasculitis associated with

systemic rheumatic diseases

malignancy

Hypocomplementemic urticarial

vasculitis

infections

Small and medium muscular

arteries

Classic PAN

Microscopic polyangiitis

Wegener’s granulomatosis

Churg-Strauss vasculitis

Kawasaki syndrome

Rheumatoid vasculitis

Large arteries

Giant cell or temporal arteritis

Takayasu arteritis

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

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

Takayasu's Arteritis (pulseless disease)

- Second or third decade

- F: M 8:1

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

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%.

Takayasu disease: arteritis (aortograms)

Takayasu disease: arteritis (pulmonary angiogram)

Takayasu disease: arteritis (aortogram)

Takayasu’s arteritis: aneurysmal dilatation, ascending and

descending aorta (MRI arteriography)

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

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

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

Relationship of polymyalgia (PMR) to giant cell arteritis

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

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

Giant cell arteritis: forehead

Giant cell arteritis: retinal ischemia

Polyarthritis nodosa

-middle age (45 years) -M:F 2.5-1

Pathology: there is fibrinoid necrosis and infarction

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

ACR 1990 criteria for classification of polyarteritis nodosa

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%

Polyarteritis nodosa: subcutaneous nodules, hand

Polyarteritis nodosa: wrist drop

Investigation

-Anemia, leukoeytosis and ESR (up)

-ANCA-ve

-HbsAg +ve %30

-Angiography: microanyrusm.

Polyarteritis nodosa (angiogram)

Polyarteritis nodosa: leg (angiogram)

Wagener granulomatosis:

-Upper + lower respiratory tract + kidney

-Sinusitis -otitis -Rhinitis -epistaxis -

oralulcers

-Hear loss -nasal ulceration

-Cough -Hemoptysis -shortness of

breath -pleuritis

-Hematourea -renal failure

-Fever -arthrlgia -headache - skinrash -

ocular inflammation.

ANCA 90%

Chest-x-ary:migratory pattern

Wegener’s granulomatosis: face

Wegener’s granulomatosis: eye involvement

Wegener’s granulomatosis: lungs (radiograph)

The chug Strauss syndrome:

Males in their fourth decade

-Rhinitis –B.Asthma - esoinophilia +systemic

vacuities

ANCA 60%

ACR 1990 criteria for classification of Churg-Strauss

syndrome

Asthma

Eosinophilia > 10%

Neuropathy, mono or poly

Pulmonary infiltrates, non-fixed

Paranasal sinus abnormality

Extravascular eosinophils

Sensitivity 85% and specificity 99.7%.

-Microscopic vacuitis (polyangilitis)

ANCA positive

-Lung and Kidney

Polyarteritis nodosa (PAN) vs. microscopic polyangiitis

Small/medium muscular artery

Renal vascular nephropathy

No lung involvement

ANCA negative

Small/medium arteries, arterioles,

venules, capillaries

Glomerulonephritis

Lungs involved in up to one third

ANCA positive

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

Skin: Palpable purpura (papules, nodules, vesicles,

bullas, ulcers, or recurrent urticaria)

Systemic: Fever –malaise, myalgia , anorexia

Extra cutaneous : joint-renal-G I –peripheral

neuropathy

ACR classification criteria: Henoch-Schönlein purpura

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%

Palpable purpura: back

Henoch-Schönlein syndrome: legs

(photomicrograph and clinical)

Vasculitis: purpuric eruption, feet

Henoch-Schönlein syndrome: purpura, legs

Hemorrhagic lesions, ankle and foot

Dermal vasculitis: ankle and foot

Livedo reticularis

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

High dose gluco corticoid therapy for induction

remission in large vessel vacuities

Imuno suppression (MTX , Azathioprine) for Takaysuo

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

Behçet’s syndrome: ulceration, tongue

Behçet’s syndrome: ulceration, tongue and labia oris

Behçet’s syndrome: ulceration, mouth

Genital:

. Scrotum and penis in males

. Vulva and vague in females

. Similar to oral ulcers

. Tendency toward scaring

. Recur less frequently

Behçet’s syndrome: ulceration, vulva

Behçet’s syndrome: scrotal ulcers

Continuous lesion's :

. Erythema – nodsum-like lesion

. Pyoderma gangrenosum –like lesion

. Acnei form

. Pseudo folliculitis

Ophthalmologic features:

. Anterior uveitis

. Posterior uveitis

. Hypopyon

. Retinal vascuitis

Behçet’s syndrome: hypopyon, eyes

Arthritis:

. Non erosive

. Symmetrical or asymmetrical

. Mono – oligo or poly arthritis

. Knee, wrist, ankles and elbows

C N S ( neuro- behcet ):

. Brain – stem

. Venous sinus syndrome syndrome

. Headache

. Diarrhea

. Abdominal

Vascular:

. Arterial and venous thrombosis

. Aneurysm

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%

Behcet’s disease: aneurysms, popliteal and femoral arteries

(angiogram)

treatment

-skin + mucosal inolvement

-local steroid (mouth + genitalulcers)

-acne –like lesion Topical

-colchicines

-Azathioprine + INF +anti TNF

-azathioprin + systemic corticosteroids

-Severe eye disease: cyclosporine A, or infliximab in

combination with azthioprine

Vessel disease

-DVT . corticosteroid

. Azathioprine

. Cyclophosphamide

. Cyclosporine

- Pulmonary or peripheral aneurysms

. Cyclophosphamid

. Steroid

Arthritis---------Colchicine

CNS--------------- . Steroid

. INF-&

. Azathioprine

. Cyclophosphamide

. Methotrexate

. TNF antagonists

Is a clinico pathologic process characterized by inflammation ......When to suspect vasculitis:...

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