ANCA Vasculitis Part 3 GPA

Question 1

Granulomatosis with polyangiitis GPA

Answer 1

c-ANCA which is PR3 - 80 to 90%

p-ANCA which is MPO - 10 to 20%

Negative ANCA in about 5 to 10%

Question 2

Granulomatosis with polyangiitis GPA

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

Granulomatosis with polyangiitis GPA

Answer 3

In the lungs you see necrotizing granulomatous lesions. In the upper respiratory tract you see involvement which is more common than MPA. This may include sinusitis, rhinitis, or otitis media, ocular inflammation, bone destruction leading to saddle-nose deformity.

Question 4

Granulomatosis with polyangiitis GPA

Answer 4

In the kidneys you often see rapidly progressive disease but can be indolent acute or chronic nephritis.

Question 5

Granulomatosis with polyangiitis GPA

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

Granulomatosis with polyangiitis GPA

Answer 6

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

Question 7

Granulomatosis with polyangiitis GPA

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

Granulomatosis with polyangiitis GPA

Answer 8

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

Question 9

Granulomatosis with polyangiitis GPA

Answer 9

Other organ involvements include 20% cardiac involvement