Vasculitis Syndromes

Question 1

Small vessels

Answer 1

Capillaries, arterioles, postcapillary venules

Question 2

Medium vessels

Answer 2

Renal arteries, mesenteric vasculature, coronary arteries

Question 3

Large vessels

Answer 3

Aorta and its proximal branches

Question 4

ANCA association: cANCA

Answer 4

1) Wegener granulomatosis 2) Churg-Strauss

Question 5

ANCA association: pANCA

Answer 5

1) Microscopic polyarteritis 2) Ulcerative colitis 3) Sclerosing cholangitis 4) Crohn disease %) Autoimmune hepatitis type 1

Question 6

ANCA association: ANCA

Answer 6

Cystic fibrosis

Question 7

Predominantly large vessel vasculitis

Answer 7

Takayasu arteritis

Question 8

Predominantly medium vessel vasculitis

Answer 8

1) PAN 2) Kawasaki disease

Question 9

Predominantly small vessel vasculitis

Answer 9

1) Granulomatosis with polyangiitis 2) Eosinophilic granulomatosis with polyangiitis 3) Microscopic polyangiitis 4) HSP 5) Isolated cutaneous luekocytoclastic vasculitis 6) Hypocomplementemic urticarial vasculitis

Question 10

Granulomatosis with polyangiitis is aka

Answer 10

Wegener granulomatosis

Question 11

Eosinophilic granulomatosis with polyangiitis is aka

Answer 11

Churg-Strauss syndrome

Question 12

HSP affects more males vs females

Answer 12

Males

Question 13

Approx 90% of HSP cases occur in what age group

Answer 13

Children, usually between 3 and 10y/o

Question 14

T/F HSP in adults present with more severe and chronic complications

Answer 14

T

Question 15

Common finding in biopsy of HSP is deposition of IgA particularly

Answer 15

IgA1

Question 16

Hallmark of HSP

Answer 16

Palpable purpura

Question 17

Skin lesions of HSP are usually of what distribution

Answer 17

Symmetric and in gravity-dependent areas or pressure points

Question 18

Skin lesions of HSP typically last for how long

Answer 18

3-10 days and may recur up to 4 months after initial presentation

Question 19

Arthritis and arthralgias occur in ___% of children with HSP

Answer 19

75

Question 20

Arthritis in HSP is usually

Answer 20

1) Oligoarticular 2) Predilection for lower extremities 3) Does not lead to deformities 4) Usually resolves in 2 weeks

Question 21

GI manifestations of HSP occur in up to ___% of patients with HSP

Answer 21

80

Question 22

Renal involvement occurs in up to ___% of children with HSP

Answer 22

50

Question 23

HSP ACR classification criteria

Answer 23

2 of the ff must be present 1) Palpable purpura 2) Age at onset less than or equal to 20 y/o 3) Bowel angina (postprandial abdominal pain, bloody diarrhea) 4) Biopsy demonstrating intramural granulocytes in small arterioles and/or venules

Question 24

HSP EULAR criteria

Answer 24

1 or more must be present 1) Abdominal pain 2) Arthiris or arthralgia 3) Biopsy of affected tissue demonstrating predominant IgA deposition 4) Renal involvement (proteinuria >3g/24hr), hematuria or red cell casts

Question 25

An isolated cutaneous leukocytoclastic vasculitis that affects infants <2 yr of age, that resembles HSP clinically

Answer 25

Acute hemorhhagic edema (AHE)

Question 26

Distinguishes AHE from HSP

Answer 26

1) Younger age 2) Nature of the lesions 3) absence of other organ involvement 4) biopsy

Question 27

T/F Platelet count in HSP is low

Answer 27

F, Normal

Question 28

UTZ is done in patients with HSP and GI symptoms to look for

Answer 28

1) Bowel wall edema 2) Intussusception (rarely)

Question 29

___ are most often used to treat significant GI involvement and joint pain in HSP

Answer 29

Steroids (Prednisone 1mkday for 1-2 weeks followed by taper)

Question 30

T/F Prednisone in HSP does not alter overall prognosis nor prevent renal disease

Answer 30

T

Question 31

Major long term complication of HSP

Answer 31

Renal disease

Question 32

Renal disease in HSP can develop up to ___ after diagnosis

Answer 32

6 months, but rarely happens if initial UA is normal

Question 33

Self-limited course of HSP usually lasts ___ on average

Answer 33

4 weeks

Question 34

From 15-60% of children with HSP experience 1 or more recurrences, typically within ___ of diagnosis

Answer 34

4-6 mo

Question 35

T/F HSP: With each relapse, symptoms are usually milder than at presentation

Answer 35

T

Question 36

T/F HSP: Children with a more-severe initial course are at higher risk for relapse

Answer 36

T

Question 37

T/F HSP: The long-term prognosis usually depends upon the severity and duration of gastrointestinal or renal involvement

Answer 37

T

Question 38

Approximately ___% of those with HSP nephritis go on to have end-stage renal disease

Answer 38

8

Question 39

AKA pulseless disease

Answer 39

Takayasu arteritis

Question 40

Takayasu arteritis predominantly affects males vs females

Answer 40

Females

Question 41

Complication of takayasu arteritis that is more common in United States, Western Europe, and Japan

Answer 41

Occlusive complications

Question 42

Complication of takayasu arteritis that is more common in SEA and Africa

Answer 42

Aneurysms

Question 43

MC aortic branches affected by takayasu arteritis

Answer 43

1) Subclavian 2) Renal 3) Carotid

Question 44

___mmHg difference in systolic BP among 4 extremities is indicative of disease

Answer 44

>10

Question 45

Mainstay of therapy of Takayasu arteritis

Answer 45

Glucocorticoids, starting with high doses (Pred 1-2mkday)

Question 46

T/F Cutaneous PAN is limited to the skin

Answer 46

T

Question 47

Vessel involvement in PAN is usually

Answer 47

Segmental and tends to occur at vessel bifurcations

Question 48

Vascular bed that is usually spared in PAN

Answer 48

Pulmonary vasculature

Question 49

Gold standard diagnostic imaging study for PAN

Answer 49

Conventional arteriography

Question 50

Conventional arteriography in PAN reveals

Answer 50

"Beads on a string" pattern (areas of aneurysmal dilation and segmental stenosis)

Question 51

Mainstay of therapy in PAN

Answer 51

Oral (1-2mkday) and IV pulse (30mkday) prednisone

Question 52

GPA and MPA involve what vascular beds

Answer 52

Respiratory tract and kidneys

Question 53

A small vessel necrotizing granulomatous (allergic granulomatosis) vasculitis associated with a history of refractory asthma and peripheral eosinophilia

Answer 53

CSS

Question 54

ANCA-associated vasculitis that can produce a saddle nose deformity

Answer 54

GPA

Question 55

Small vessel vasculitis: IgA-dominant immune deposits

Answer 55

HSP

Question 56

Small vessel vasculitis: Necortizing vasculitis

Answer 56

GPA, CSS, and MPA

Question 57

Small vessel vasculitis: Granulomatous inflammation

Answer 57

GPA and CSS

Question 58

Small cell vasculitis: Asthma and eosinophilia

Answer 58

CSS

Question 59

Predominantly small vessel vasculitis, granulomatous

Answer 59

1) Wegener granulomatosis 2) Churg-Strauss

Question 60

Predominantly small vessel vasculitis, nongranulomatous

Answer 60

1) Microscopic polyangitis 2) HSP 3) Isolated cutaneous leukocytoclastic vasculitis 4) Hypocomplementemic urticarial vasculitis

Question 61

MC vasculitis of childhood

Answer 61

HSP

Question 62

Characterized by leukocytoclastic vasculitis and IgA deposition in small vessels of the skin, joints, GIT, and kidneys

Answer 62

HSP

Question 63

Takayasu arteritis is characterized by inflammation of the vessel wall starting at what layer

Answer 63

Vasa vasorum

Question 64

Proposed classification criteria for pediatric Takayasu arteritis

Answer 64

Angiographic abnormalities of the aorta or its main branches AND at least one of the ff: 1) Decreased peripheral artery pulse and/or claudication of extremities 2) BBP diff between arms and legs of >10mmHg 3) bruits over the aorta and/or its major branches 4) Htn

Question 65

T/F Radiographic assessment is essential to establish large vessel arterial involvement

Answer 65

T

Question 66

Gold standard to establish large vessel arterial involvement in Takayasu arteritis

Answer 66

Conventional arteriography of the aorta and its major branches