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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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