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Vasculitis Applied

Anish Pithadia

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

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

Artery may be nodular Ophthalmic artery may be involved and blindness can occur Rx: corticosteroids Lab- very high ESR, blindness(can become permanent), claudication (pains
that result in muscle when the muscle is working, but the vasculature to the area 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

Mucocutaneous lymph node syndrome (Kawasaki disease)


Seen in <5 yr olds- 80%

Feet, Hands and Conjunctiva become red


Cardiac complications arise Japan and US- most common cause of acquired heart
disease in young children, some of the babies develop fever, conjunctivitis, skin has rash, hands, feet and soles get red

Vasculitis may be in coronary artery

Oral erythema

Poly Arteritis Nodosa


Severe necrotizing inflammation of visceral vessels

Arterioles, venules, capillaries are not inflamed


Multiple arterial inflammation leading to formation of nodesmulti-systemic arteritis renal and visceral vessels but spares 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!

When there is antigen antibody complex activation there may be


macrophages, 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 by
C reactive protein Local

Rx: immunosuppression- Corticosteroids, cyclophosphamide

Thromboanginitis ObliteransBuergers 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 the
vessel 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!

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 around the body under skin(purpura), synovial membrane(arthritis), etc.

Cryoglobulinemia- coagulation precipitated at lower


temperature.

Wegners Disease
Necrotizing medium and small-sized vessel vasculitis

Involved in lung infarctions and renal vessels(glomerulonephritis)


Necrotizing granulomas in skin, upper respiratory
tract(nasopharynx-saddle nose deformity, chronic sinusitis,collapse of trachea), lower respiratory tract(cavitating nodular lesions)

Necrotizing vasculitis in lungs (infarction and hemoptysis) C-ANCA antibodies (>90% of cases) correlate erratically with
therapy

3 cs= c-ANCA, corticosteroids, cyclophosphamide

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

Lab Work and Diagnosis


1. Rule out any
MIMICKERS of Vasculitis

2. Skin biopsy least


invasive 1-2 stitches removed
7-10 days later Leukocytoclastic vasculitis seen to the right

Bad Biopsy
Searching for PAN
Not deep enough and only contains superficial dermis and epidermis. WHY? PAN affects medium sized arteries tat are located deep within the dermis

Good Biopsy

Magnified previous picture


Inflamed medium sized vessel

NOT THE WHITE AREASTHOSE ARE FAT LOBULES

Urine Analysis
Almost always done when
suspecting vasculitis in patients

Presence of red blood cells


or high amounts of protein in the urine calls for a biopsy Local anesthesia and U/S Crescent shaped
glomerulus seen

Temporal Biopsy
Used to diagnose Giant
Cell Temporal Arteritis

Done under anesthesia,


small incision require 23cm taken for lab results

Lung Biopsy
Used for diagnosing vasculitis that may have to do with
the lungs Ie (Wegners)

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

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 over production 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?

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

Diseases associated with ANCA


Wegners granulomatosis- C ANCA

Microscopic Polyangitis- P-ANCA


Glomerulonephritis- P-ANCA Churg-Strais- associated with p-ANCA directed against
MPO

C-ANCA