Vasculitis and Alveolar Hemorrhage

Question 1

3 ANCA associated vasculitises

Answer 1

MPA - Microscopic polyangiitis

GPA - Granulomatosis with polyangiitis (Wegener’s)

EGPA - Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Question 2

4 Immune complex associated vasculitises

Answer 2

Anti-GBM (Goodpastures)

IgA-vasculitis (Henoch-Shoenlein)

Cryoglobulinemic vasculitis

Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)

Question 3

Most common type of vasculitis

Answer 3

Giant cell arteritis

Rarely effects lungs but may have cough and nodules

Question 4

3 “NO’s” of polyarteritis Nodosa

Answer 4

No ANCA

No glomerulonephritis

No pulmonary capillaritis

Question 5

2 large to medium vessel vasculitises that can cause pulmonary artery aneurysms

Answer 5

Takayasu (young female)

Behcet’s disease

Question 6

What percentage of GPA or MPA will have develop diffuse alveolar hemorrhage?

Answer 6

25%

Question 7

Which ANCA version is more likely to relapse

Answer 7

C-ANCA/PR3-ANCA

Question 8

C-ANCA is associated with PR3 or MPO

Answer 8

PR3

Question 9

Is there a correlation of the level of ANCA positivity and the severity of disease?

Answer 9

NO

Question 10

Answer 10

Saddle Nose deformity

Necrotizing granulomatous inflammation of GPA

Question 11

Answer 11

Strawberry gums

Necrotizing granulomatous inflammation of GPA

Question 12

Remission induction regimen in limited GPA

Answer 12

MTX and GCS

Question 13

Trial that compared rtixumab and cyclophosphamide

Answer 13

RAVE trial

NEJM 2010

Question 14

Remission induction regimen for SEVERE GPA or MPA

Answer 14

GCS and cyclophosphamide or rituximab

Question 15

Subset of severe patients with MPA that will benefit from cyclophosphamide OVER rituximab in remission induction

Answer 15

MPO+ patients with severe renal disease

Question 16

Remission maintenance therapy for GPA or MPA following cyclophosphamide

Answer 16

Methotrexate or azithioprine

Cellcept can be used but not as good

Question 17

Remission maintenance therapy for GPA or MPA following rituximab

Answer 17

Rtiuximab

Question 18

Mainstay of therapy in churg strauss

Answer 18

glucocorticoids

Question 19

Steroid sparing agents in churg strauss

Answer 19

MTX, AZA, Anti-IL5

Question 20

Situations where CYC is required for severe churg strauss

Answer 20

Heart, CNS, mononeuritis multiplex, severe renal involvement

Question 21

Mepolizumab dosing difference in Asthma vs Churg Strauss

Answer 21

Asthma = 100 mg q 4 wks

Churg Strauss = 300 mg q 4 wks

Question 22

Biologic Asthma Meds (name, brand, Mechanism, dosing)

Answer 22

Question 23

Percentage of hemosiderin-laden macrophages that suggests DAH

Answer 23

> 20%