Vasculitis Flashcards

1
Q

Signs of vasculitis

A

Fever, myalgias, arthralgias, malaise

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2
Q

Pathogenesis of non’infectious vasculitis

A

Abs to viral proteins form immune complexes that deposit in vascular lesions

30% of pats with poly arthritis bodies have underlying Hep B

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3
Q

Anti-myeloperoxidase (MPO-ANCA)

A

Microscopic polyangiitis and Church-Strauss syndrome

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4
Q

Anti-proteinase-3 (PR3-ANCA)

A

Typically found in polyagiitis with granulomatosis

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5
Q

Giant cell arteritis

A

Most common

Chronic, often granulomatous

Commonly arteries in the head (TEMPORAL ARTERY)

Ophthalmic artery (blindness)

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6
Q

Giant cell arteritis morphology

A

Modular intimal thickenings with reduction of lumen

Medial granulomatous inflammation

Elastic laminate fragmentation

Segmental inflammatory lesions along vessel

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7
Q

Giant cell arteritis clinical

A

MEDICAL EMERGENCY- diagnose and treat to prevent blindness

Affected artery is modular and tender to palpating

Diagnose by biopsy of involved artery

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8
Q

Takayasu Arteritis

A

Rare, granulomatous vasculitis

<50

Women > men

Involves aortic arch

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9
Q

Takayasu Arteritis Clinical

A

Weakening of pulses in the upper extremities, reduced BP

Ocular disturbances

Claudication of legs

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10
Q

Polyarteritis nodoosa

A

Involves small and medium arteries

No ANCA association

About 30% have chronic HepB with HbsAg-HbsAb complexes in affected vessels

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11
Q

Polyarteritis nodosa morphology

A

Segmental necrotizing inflammation of small and medium vessels

Descending distribution Kidneys, heart, liver, GI tract

Impaired perfusion: ulceration, infarcts, atrophy, hemorrhage’s in distribution of affected vessels

Aneurysmal nodules

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12
Q

Acute polyarteritis nodosa

A

Transmittal inflammation with fibrinoid necrosis

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13
Q

Later polyarteritis nodosa

A

Acute inflammation with fibrinoid necrosis

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14
Q

Clinical polyarteritis nodosa

A

Young adults > kids or elderly

Vasculitis in renal arteries
No glomerulonephritis
Major cause of death along with hypertension

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15
Q

Polyarteritis nodosa treatment

A

Corticosteroids and cyclophosphamide

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16
Q

Kawasaki syndrome

A

Large, medium sized and small arteries

Young children and infants (#1 cause of acquired heart disease in children)

Acute, febrile, usually self limited

17
Q

Morphology of Kawasaki syndrome

A

Resembles polyarteritis nodosa
Transmittal inflammation but less fibrinoid necrosis

Range from intimal lesions to severe transmittal destruction

18
Q

Kawasaki syndrome clinical

A

Fever, congested conjunctival, changes in lips and oral mucosa, rash, cervical lymphadenopathy

Edema of hands, feet, erythema of palms and soles

19
Q

Untreated Kawasaki syndrome

A

Asymptomatic vasculitis of coronaries

Giant coronary aneurysm

MI or sudden death

20
Q

Kawasaki syndrome treatment

A

Aspirin, IV immunoglobulin

21
Q

Microscopic Polyangiitis

A

Usually affects arteriolosclerosis, capillaries and venules

All lesions tend to be SAME AGE

MPO-ANCA is usually present

22
Q

Microscopic polyangiitis pathogenesis

A

Some cases from antibody response to antigens such as:drugs, microorganisms, heterologous proteins, tumor antigens

Immune complex decomposition
Triggers secondary immune response (MPO-ANCAs)

Most lesions are pauci-immune

23
Q

Microscopic polyangiitis morphology

A

Segmental fibrinoid necrosis of the media

No granulomatous inflammation

Similar to PAN but muscular and large arteries are spared

May only see infiltrating and fragmenting of neutrophils

24
Q

Microscopic polyangiitis clinical

A

Hemostasis, Hematuria, and proteinuria
Bowel pain and bleeding
Muscle pain or weakness
Palpable purpura

25
Q

Microscopic polyangiitis treatment

A

Immunosuppression, improves long term survival

26
Q

Church Strauss syndrome

A

small vessel necrotizing vasculitis

Associated with allergic rhinitis, bronchial asthma and eosinophilia

Intra and extra-vascular granulomas
Infiltration of vessels and perivascular tissues by eosinophils

27
Q

Granulomatous with Polyagiitis

A

Respiratory tract involvement with acute necrotizing granulomas of upper and/or lower

Focal necrotizing or granulomatous vasculitis of small to med sized vessels

Renal disease

(Limited forms restricted to respiratory tract)

28
Q

Granulomatosis with polyangiitis pathogenesis

A

PR3-ANCA in 95% of patients

29
Q

Granulomatosis with polyangiitis clinical

A

M>F average age 40

Persistent pneumonia is with bilateral modular and cavitary infiltrates

Chronic sinusitis

Mucosal ulceration of the nasopharyngeal

Rapidly fatal if untreated (immunosuppressive, cyclophosphamide)

30
Q

Thromboangiitis obliterans (Buerger Disease)

A

Segmental , thrombosis, acute and chronic inflammation of med sized and small arteries (Tibial and radial arteries)

MEN WHO ARE HEAVY CIGARETTE SMOKERS
Begins before 35

31
Q

Thromboangiitis obliterans morphology

A

The onus contains microabscesses with central focus of neutrophils surrounded by granulomatous inflammation

Inflammation extends to adjacent veins and nerves

32
Q

Thromboangiitis obliterans clinical

A

Superficial modular phlebitis
Cold sensitivity in hands
Instep claudication
Chronic ulceration of toes, feet, fingers