Vasculitis

Question 1

Signs of vasculitis

Answer 1

Fever, myalgias, arthralgias, malaise

Question 2

Pathogenesis of non’infectious vasculitis

Answer 2

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

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

Question 3

Anti-myeloperoxidase (MPO-ANCA)

Answer 3

Microscopic polyangiitis and Church-Strauss syndrome

Question 4

Anti-proteinase-3 (PR3-ANCA)

Answer 4

Typically found in polyagiitis with granulomatosis

Question 5

Giant cell arteritis

Answer 5

Most common

Chronic, often granulomatous

Commonly arteries in the head (TEMPORAL ARTERY)

Ophthalmic artery (blindness)

Question 6

Giant cell arteritis morphology

Answer 6

Modular intimal thickenings with reduction of lumen

Medial granulomatous inflammation

Elastic laminate fragmentation

Segmental inflammatory lesions along vessel

Question 7

Giant cell arteritis clinical

Answer 7

MEDICAL EMERGENCY- diagnose and treat to prevent blindness

Affected artery is modular and tender to palpating

Diagnose by biopsy of involved artery

Question 8

Takayasu Arteritis

Answer 8

Rare, granulomatous vasculitis

<50

Women > men

Involves aortic arch

Question 9

Takayasu Arteritis Clinical

Answer 9

Weakening of pulses in the upper extremities, reduced BP

Ocular disturbances

Claudication of legs

Question 10

Polyarteritis nodoosa

Answer 10

Involves small and medium arteries

No ANCA association

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

Question 11

Polyarteritis nodosa morphology

Answer 11

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

Question 12

Acute polyarteritis nodosa

Answer 12

Transmittal inflammation with fibrinoid necrosis

Question 13

Later polyarteritis nodosa

Answer 13

Acute inflammation with fibrinoid necrosis

Question 14

Clinical polyarteritis nodosa

Answer 14

Young adults > kids or elderly

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

Question 15

Polyarteritis nodosa treatment

Answer 15

Corticosteroids and cyclophosphamide

Question 16

Kawasaki syndrome

Answer 16

Large, medium sized and small arteries

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

Acute, febrile, usually self limited

Question 17

Morphology of Kawasaki syndrome

Answer 17

Resembles polyarteritis nodosa
Transmittal inflammation but less fibrinoid necrosis

Range from intimal lesions to severe transmittal destruction

Question 18

Kawasaki syndrome clinical

Answer 18

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

Edema of hands, feet, erythema of palms and soles

Question 19

Untreated Kawasaki syndrome

Answer 19

Asymptomatic vasculitis of coronaries

Giant coronary aneurysm

MI or sudden death

Question 20

Kawasaki syndrome treatment

Answer 20

Aspirin, IV immunoglobulin

Question 21

Microscopic Polyangiitis

Answer 21

Usually affects arteriolosclerosis, capillaries and venules

All lesions tend to be SAME AGE

MPO-ANCA is usually present

Question 22

Microscopic polyangiitis pathogenesis

Answer 22

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

Question 23

Microscopic polyangiitis morphology

Answer 23

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

Question 24

Microscopic polyangiitis clinical

Answer 24

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

Question 25

Microscopic polyangiitis treatment

Answer 25

Immunosuppression, improves long term survival

Question 26

Church Strauss syndrome

Answer 26

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

Question 27

Granulomatous with Polyagiitis

Answer 27

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)

Question 28

Granulomatosis with polyangiitis pathogenesis

Answer 28

PR3-ANCA in 95% of patients

Question 29

Granulomatosis with polyangiitis clinical

Answer 29

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)

Question 30

Thromboangiitis obliterans (Buerger Disease)

Answer 30

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

Question 31

Thromboangiitis obliterans morphology

Answer 31

The onus contains microabscesses with central focus of neutrophils surrounded by granulomatous inflammation Inflammation extends to adjacent veins and nerves

Question 32

Thromboangiitis obliterans clinical

Answer 32

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