ANCA Vasculitis Part 4 EGPA

Question 1

Eosinophilic granulomatosis with polyangiitis EGPA

Answer 1

c-ANCA which is PR3 - 10%

p-ANCA which is MPO - 60%

Negative ANCA in about 30%

Question 2

Eosinophilic granulomatosis with polyangiitis EGPA

Answer 2

Age of onset is 5th to 7th decade of life with a slight male predominance. More common in white than black.

Question 3

Eosinophilic granulomatosis with polyangiitis EGPA

Answer 3

In the lungs you see asthma, eosinophil rich and granulomatous inflammation.

Question 4

Eosinophilic granulomatosis with polyangiitis EGPA

Answer 4

In the kidneys there is less frequent kidney involvement. Kidney disease is less severe than MPA or GPA.

Question 5

Eosinophilic granulomatosis with polyangiitis EGPA

Answer 5

In the gastrointestinal 50% of patients with MPA, GPA, EGPA have abdominal pain, bloody stool with mesenteric ischemia or intestinal infarction or perforation, pancreatitis, or hepatitis.

Question 6

Eosinophilic granulomatosis with polyangiitis EGPA

Answer 6

In the skin you will see skin nodules due to dermal or subcutaneous arteritis and necrotizing granulomatous infiltration.

Question 7

Eosinophilic granulomatosis with polyangiitis EGPA

Answer 7

Neurologically you see peripheral neuropathy, usually mononeuritis multiplex pattern. Up to 70% in the EGPA and a 50% GPA and 30% MPA.

Question 8

Eosinophilic granulomatosis with polyangiitis EGPA

Answer 8

Central nervous system involvement is less common, may present as vasculitis within the meninges.

Question 9

Eosinophilic granulomatosis with polyangiitis EGPA

Answer 9

Other organ involvements you see eosinophilia: systemic/infiltration.

50% cardiac involvement