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Vasculitis AppliedAnish Pithadia

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Takayasu Arteritis Younger age asian females

Fibrotic vessels- longer term fibrosis leads to shrinkage of vessels.

Blood supply to upper extremity is

Macrophages, lymphocyte, eosinophils come to the vasa visorum

Systemic - Cytokines are produced (malaise, fever)

Local features- elastic tissue if destroyed and become fibrotic. Loss of elastic

properties. When L v produced systole there is stretch and relaxation. When there is fibrosis and stenosis the pulse in not propagated to the upper

extremities.

NO PULSE but there is blood flow

Ischemia of the upper limbs tingling sensation of upperr limbs can seen as well. Blood flow to CNS is reduced as well dizziness, syncope, neurological

dysfunction

Sometimes there is aortic dilations-As the aortas doesnt recoil there may be

regurgitation of the aortic valve.

Coronary Osteal Stenosis may be seen as well

intimal wrinkling

Rx: corticosteroids

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Giant Cell (temporal arteritis) >50 yr olds +

Descendants of Nordic origins higher risk factor

Granulomatous inflammation in the media of temporal artery

The inflammatory cells(lympho, macro, neutrophils, giant cells

(langherhans cells, foreign body type giant cells) attach

Must biopsy 2-3 cm of the temporal artery

Granuloma formation with multitude of cells

Seen only in 70% of pts

Others have them scattered inflammatory cells

Most common systemic vasculitis in adult

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Artery may be nodular

Ophthalmic artery may be involved and blindness can occur

Rx: corticosteroids

Lab- very high ESR, blindness(can become permanent), claudication (painsthat result in muscle when the muscle is working, but the vasculature to thearea does not relax to supply the area.

Vasa nervosum- some segment of the nerve may become infarcted

ASSOCIATED WITH POLYMYALGIA RHEUMATICA

Pains of proximal girdle hips shoulders and periarticular inflammation Pts cant comb hair, may find it difficult to even stand

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Mucocutaneous lymph node

syndrome (Kawasaki disease) Seen in

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Poly Arteritis Nodosa Severe necrotizing inflammation of visceral vessels

Arterioles, venules, capillaries are not inflamed

Multiple arterial inflammation leading to formation of nodes-

multi-systemic arteritis renal and visceral vessels butspares the pulmonary circulation

The visceral artery has transmural inflammation

Usually theres always some fibrinoid necrosis

30% of the pts have HbsAg +

Not Associated with ANCA!

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When there is antigen antibody complex activation there may bemacrophages, lymphocytes, eosinophil's, along with necrosis

May lead to a weakening of wall and therefore lead to an aneurysm.

Segmental vasculitis

Immune complex vasculitis

Necrotic tissue of the vasculature may slough off ischemia, ulceration, infarction, hemorrhage

CLINICAL

Systemic- fever, malaise, anorexia, weight loss- Increase in ESR found byC reactive protein

Local

Rx: immunosuppression- Corticosteroids, cyclophosphamide

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Thromboanginitis Obliterans-

Buergers disease Severe inflammation of the arteries of the limbs(both hands and feet)

Pain, cyanotic, cold- due to reduced blood supply

Ischemia may precipitate

Ulcers may be present

Spillover to the neighboring arteries, veins and nerves

Strongly associated with men who smoke

Inject tobacco subdermally and you get hypersensitivity reaction

Age of 40 years, India, japan and Israeli descent

The inflammation of the endothelial lining leads to thrombi formation within thevessel lumen. Also giant cells and in the center microabscesses may be

present inside them

Doesn'tt involve the visceral arteries

Usually presents as an episodic attack.

Rx: Stop smoking!

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Microscopic Polyangitis Small Vessel p-ANCA positive

Involves the pulmonary arteries unlike PAN

Palpable Purpura

Henosch-Scholein After resp. infection usually, there is IgA antibodies that are

secreted in the mucosal lining.

They make aggregates with the antigens and deposit aroundthe body under skin(purpura), synovial membrane(arthritis), etc.

Cryoglobulinemia- coagulation precipitated at lowertemperature.

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Wegners Disease Necrotizing medium and small-sized vessel vasculitis

Involved in lung infarctions and renal vessels(glomerulonephritis)

Necrotizing granulomas in skin, upper respiratorytract(nasopharynx-saddle nose deformity, chronic

sinusitis,collapse of trachea), lower respiratory tract(cavitatingnodular lesions)

Necrotizing vasculitis in lungs (infarction and hemoptysis)

C-ANCA antibodies (>90% of cases) correlate erratically with

therapy 3 cs= c-ANCA, corticosteroids, cyclophosphamide

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Churg Strauss Syndrome Microscopic Vasculitis found in allergy prone pts

Pts have allergy, asthma, rhinitis

Multiple vasculitis lesions

Chronic granulomatous anginitis Eosinophil's are increased in the blood!

P-ANCA

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Lab Work and Diagnosis 1. Rule out any

MIMICKERS of

Vasculitis

2. Skin biopsy leastinvasive

1-2 stitches removed

7-10 days later

Leukocytoclasticvasculitis seen to the

right

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Bad Biopsy

Searching for PAN

Not deep enough and only

contains superficial dermis andepidermis.

WHY?

PAN affects medium sized

arteries tat are located deepwithin the dermis

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Good Biopsy

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Magnified previous picture

Inflamed medium sized

vessel

NOT THE WHITE AREAS-

THOSE ARE FAT

LOBULES

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Urine Analysis Almost always done when

suspecting vasculitis in

patients

Presence of red blood cellsor high amounts of protein

in the urine calls for a

biopsy

Local anesthesia and U/S Crescent shaped

glomerulus seen

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Temporal Biopsy Used to diagnose Giant

Cell Temporal Arteritis

Done under anesthesia,

small incision require 2-

3cm taken for lab results

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Lung Biopsy Used for diagnosing vasculitis that may have to do with

the lungs

Ie (Wegners)

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Abdominal Angiogram

Helpful in the diagnosis of

Polyarteritis

Nodosa(PAN)

Diagnosis is based on

out-pouchings that are

seen when the dye is

injected

Diagnostic of PAN

Similar to a heart

catheterization

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ESR and C-Reactive Protein Where is there a physiologic increase in ESR?

Pregnancy- must be ruled out!

ESR increases when there is a high amount of acute

phase proteins that has been stimulated by overproduction of cytokines. This increases the rate at

which RBCs stack up on one another.

SOOOOOOO let me ask a question!

How does a fever start- what the mechanism behind a

fever?

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ANCA Group do anutoantibodies

Minly of IgG type- against antigens in the cytoplasm of

neutrophil granulocytes and monocytes

P-ANCA- peri nucleur- MPO

C-ANCAcytoplasmiic- proteinase 3

Di i t d ith

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Diseases associated with

ANCA Wegners granulomatosis- C ANCA

Microscopic Polyangitis- P-ANCA

Glomerulonephritis- P-ANCA

Churg-Strais- associated with p-ANCA directed against

MPO

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C-ANCA

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