Systemic Vasculitis

Question 1

characteristics of vasculitis

Answer 1

 Inflammation of the blood vessel walls
 Pathology defined by leukocyte-mediated injury of
vessel walls
 Classified by whether
leukocytes damage
predominantly small,
medium, or large vessels

Question 2

outline the classifications of vasculitis

Answer 2

large vessel vasculitis: takayasu arteritis, giant cell arteritis

medium vessel: polyarteritis Nodosa, kawasaki disease

Small vessel (immune complex): cryglobulinemic vasculitis, igA vasculitis, hypocomplementemic urticarial vasulitis, (LESS FREQUENT)

Small vessel (ANCA-Associated): microscopic polangitis, granulomatosis (wegeners), eosinophilic granulomatosis with polyangitis (MORE COMMON)

Question 3

note vessel types in different types of vasculitis

Question 4

when to suspect vasculitis (clinical features)

Answer 4

• skin lesions (palpable purpura)
• ischemic vascular changes (gangrene, claudication, raynauds, livedo)
• glomerulonephritis
• mononeuritis multiplex
• foot or wrist drops
• myalgia, arthralgia, arthritis
• abdominal or testical pain.

Question 5

recognizing vasculitis usually happens in an ___ setting

Answer 5

emergent setting

Question 6

3 types of anca-associated vasculitis

Answer 6

1. granulomatosis with polyangitis (GPA) formerly known as wegeners granulomatosis
2. micropscopic polyangitis (MPA)
3. eosinophilic granulomatosis with polyangitis (EGPA)

Question 7

what type of anca-associated vasculitis is more common in caucasians?

Answer 7

 In Northern European populations, GPA is more common

 GPA is most common in Caucasian patients of European descent

 In Southern European populations, MPA is more common.

 AAV affects both sexes equally.3  AAV occurs in patients of all ages (range 9–78 years).3

Question 8

T/F in AAV, immunofluorescence typically shows deposition of immune complexes

Answer 8

false. Immunofluorescence typically shows no deposition of immune complexes (pauci-immune)

Question 9

organ pathology mechanism during AAV

Answer 9

AAV causes necrotizing vasculitis which can obstruct vessels .vascular injury leads to ischemia, hemorrhage, granulomatous inflmamation and loss of function of the organ.

Question 10

which organ is most ocmmonly involved in AAV

Answer 10

lungs and kidenys.

Question 11

when should AAV be suspected

Answer 11

AAV should be suspected in patients presenting with multisystemic symptoms not caused by infections or alignancy such as:

1. renal failure (renal failure glomerulonephirits)
2. skin rashes
3. pulmonary infiltrates
4. neurologial manifestations.

Question 12

clinical indications for ANCA test

Answer 12

Question 13

what type of AAV would this person be struggling with?

Answer 13

MPA: more frequently associated with alveolitis and pulmonary hemorrhage than GPA

Question 14

what type of AAV would this person be struggling with?

Answer 14

GPA. GPA is more frequently associated with pulmonary nodules than is MPA

Question 15

what does this slide depict?

Answer 15

glomerulonephritis. pathologically, this is indistinguishable in GPA, MPA, EGPA and renal-limited disease

in the acute phase, the glomerulonephritis is characterized by segmental fibrinoid necrosis and creascent formation

Question 16

Fill out this table for GPA, MPA, and EGPA

Answer 16

Question 17

manifestations of AAV in upper airways, joints, eyes ekin and nuerological symptoms

Answer 17

Question 18

2 phases of treatment for AAV

Answer 18

1. induction: aggressive treatment ot put into remission
2. maintenance: not as toxic drugs to prevent relapse. AV is very prone to relapse if maintenance therapy is stopped

Question 19

T/F untreated GPA (Wegeners) is fatal

Answer 19

true. natural course of severe disease has mean survival of 5 months. Corticosteroids alone have been shown to prolong median survival by 7.5 months only

Question 20

main induction therapy for AAV

Answer 20

corticosteroids, often in combination with methotrexate or cyclophosphamine. (in severe cases, methylprednisone IV)

Question 22

maintenance therapies for AAV

Answer 22

Azathioprine, retuximab, methotrexiate, mycophenolate mofetil and low dose prednisone-

think about MAINRITSAN: MAINtenance of remission using RITuximab for System ANca-associated vasculitides. – retux is less likely to have relpases in comparison with azothiaprine

Question 23

case:

 72 F, Chronic headache for 2 years  January 2013:
 Bilateral temporal headache
 Temporal area tenderness and swelling  May 2013:
 Jaw claudication for 2 weeks
 May 26: R eye blurry vision and metamorphopsia ( wavy lines)  ROS: fatigue, weight loss and anorexia

Answer 23

extreme case of giant cell arteritis

Question 25

a person with chronic and severe headaches presents with this: what is it and how would you treat it?

Answer 25

this is giant cell arteritis. vessel lumen completely occluded. inflammation of layers of the vessels.

• treatment with solumedrol and prednisone

Question 26

key neurological dysfunction as a result of GCA

Answer 26

stroke– would see infarct due to ischemic conditions and lack of blood flow/severe stenosis of vertebral arteries.

Question 27

clinical features of GCA

Answer 27

• new headache
• jaw claudication
• PMR
• partial or complete loss of vision
• arm claudication
• temporal artery prominence, tenderness and thickening wiht decreased or diminished pulses
• bruits
• aneurysm/aortic regurgitation

Question 28

GCA treatment

Answer 28

Question 29

what should prednisone be paired with so that tapering is easier during GCA

Answer 29

tocilizumab/biolgics. studies show that this pairing resulted in less flairs seen after one year

Question 30

how to diagnose takayasu’s arteritis

Answer 30

classification criteria for takayasus:

• 40 years or less
• claudication of the extremities
• decreased brachial artery pressure
• blood pressure difference of more than 10 mmhg
• bruit over sublavian arteries or the aorta
• narrowing of large arteries that is not the result of arteriosclerosis, fibromuscular dysplasia
• need to fulfil 3/6 criteria.

Question 31

treatment of takayasu arteritis

Answer 31

prednisone for 1 month then taper to maintenance dose. 60% get into remission, it usually takes 22 months and relapse is common

Question 32

what to use to treat takayasu arteritis relapse? what to use during refractory stage?

Answer 32

if relase: methotrexatae, azathioprine, cellcept, cyclophosphamide

when refractory: Anti-TNF

case reports of rituximab also working

Question 33

in summary:
small vessel vasculitis should be considered in patients presenting with diffuse alveolar hemorrhage, pulmonary renal syndrome, multiorgan failure and mononeuritis multiplex

large vessel vasculitis should be considered in patients presenting with aortic aneurysm and stroke: high CRP

• rheumatology should be consulted early when a rheumatic auto-immune disease is suspected to prevent delayed diagnosis and irreversable damage.