Small-vessel vasculitis: ANCA-associated vasculitis and non-ANCA-associated vasculitis

Question 1

What type of necrosis does ANCA-associated vasculitis result in?

Answer 1

Fibrinoid necrosis

Question 2

Why does ANCA-associated vasculitis result in circulating autoantibodies?

Answer 2

Presence of Anti-Neutrophil Cytoplasmic Antibodies (ANCAs)

Question 3

What are the 2 main types of ANCA-associated vasculitis?

Answer 3

Microscopic polyangiitis

Granulomatosis with polyangiitis

Question 4

Which 2 medications are both used to treat active ANCA-associated vasculitis?

Answer 4

High-dose glucocorticoids

IV cyclophosphamide

Question 5

Which medications are both used to manage ANCA-associated vasculitis in remission?

Answer 5

Glucocorticoids with an immunosuppressant such as azathioprine, methotrexate, MMF, rituximab

Question 6

Why do ANCA-associated vasculitis remission patients need to be regularly monitored?

Answer 6

High risk of relapse that can ultimately lead to renal failure

Question 7

Is microscopic polyangiitis a type of granulomatous or non-granulomatous vasculitis?

Answer 7

Non-granulomatous

Question 8

Is microscopic polyangiitis a type of necrotising or non-necrotising vasculitis?

Answer 8

Necrotising

Question 9

Which vessels are commonly affected by microscopic polyangiitis?

Answer 9

Capillaries
Venules
Arterioles

Question 10

Are all ages equally affected by microscopic polyangiitis?

Answer 10

Typically occurs in older adults but children can also be affected

Question 11

What is the characteristic renal symptom of microscopic polyangiitis?

Answer 11

Rapid, necrotising glomerulonephritis

Question 12

What is the characteristic pulmonary symptom of microscopic polyangiitis, and what complication does this cause?

Answer 12

Pulmonary capillaritis (alveolar wall inflammation) which presents as alveolar haemorrhage

Question 13

What is the uncommon pulmonary symptom of microscopic polyangiitis?

Answer 13

Pleural effusions

Question 14

Is neuropathy common in microscopic polyangiitis?

Answer 14

No, only occurs in 15% cases

Question 15

Can microscopic polyangiitis cause skin symptoms?

Answer 15

Yes

Question 16

Can microscopic polyangiitis cause heart symptoms?

Answer 16

Yes

Question 17

Can microscopic polyangiitis cause GI symptoms?

Answer 17

Yes

Question 18

Which type of antibodies are microscopic polyangiitis cases mostly positive for?

Answer 18

Myeloperoxidase antibody (MPO)

Question 19

What is the characteristic finding from a biopsy, in microscopic polyangiitis?

Answer 19

Segmental fibrinoid necrosis with non-granulomatous inflammation

Question 20

Describe the ESR and CRP levels in microscopic polyangiitis?

Answer 20

Elevated

Question 21

Describe the WCC in microscopic polyangiitis?

Answer 21

Elevated

Question 22

What is the typical result of the ANCA test in microscopic polyangiitis?

Answer 22

ANCA test is positive and shows presence of MPO-ANCA/P-ANCA (autoantibodies that target myeloperoxidase)

Question 23

Which 2 radiological investigations are done in ANCA-associated vasculitis?

Answer 23

CT or MRI of chest

Question 24

Is granulomatosis with polyangiitis a type of necrotising or non-necrotising vasculitis?

Answer 24

Necrotising

Question 25

In which 3 places are capillaries, arterioles and venules characteristically inflamed in granulomatosis with polyangiitis?

Answer 25

Respiratory tract (ears, nose, sinuses, throat) Lungs Kidneys

Question 26

Is granulomatosis with polyangiitis equally common in men and women?

Answer 26

No More common in men

Question 27

Does granulomatosis with polyangiitis affect all ages equally?

Answer 27

Most common in middle-aged people

Question 28

What common symptom presents due to ear involvement in granulomatosis with polyangiitis?

Answer 28

Hearing problems/deafness

Question 29

What common symptom presents due to sinus involvement in granulomatosis with polyangiitis?

Answer 29

Sinusitis

Question 30

What 2 common symptoms presents due to nose involvement in granulomatosis with polyangiitis?

Answer 30

Pus-like drainage with crusting from nose Stuffy nose

Question 31

What systemic symptom commonly occurs in granulomatosis with polyangiitis?

Answer 31

Fever

Question 32

What common symptom occurs due to kidney involvement in granulomatosis with polyangiitis, and give 3 signs?

Answer 32

Glomerulonephritis Signs include: Shortness of breath, fatigue, haematuria (if severe)

Question 33

Describe the ESR and CRP levels in granulomatosis with polyangiitis?

Answer 33

Elevated

Question 34

Describe the WCC in granulomatosis with polyangiitis?

Answer 34

Elevated

Question 35

What is the typical result of the ANCA test in granulomatosis with polyangiitis?

Answer 35

ANCA test is positive and shows presence of PR3-ANCA/C-ANCAA (target proteinase 3)

Question 36

What is eosinophilic granulomatosis with polyangiitis/Churg-Strass syndrome?

Answer 36

Necrotising vasculitis associated with peripheral eosinophilia

Question 37

How is Churg-Strass syndrome different from granulomatosis with polyangiitis?

Answer 37

Granulomatosis with polyangiitis doesn't involve peripheral eosinophilia Churg-Strass syndrome (eosinophilic granulomatosis with polyangiitis) involves peripheral eosinophilia

Question 38

What phase occurs before the onset of Churg-Strass syndrome?

Answer 38

Prodromal/allergic phase Can last years

Question 39

Give 2 ways in which the allergic phase of Churg-Strass syndrome affects the nose?

Answer 39

Allergic rhinitis (irritated nose due to allergy) Nasal polyposis

Question 40

What 3 respiratory conditions are symptoms of Churg-Strass syndrome?

Answer 40

Late-onset asthma Pleural effusions Pulmonary infiltrates (substances denser than air enter lungs)

Question 41

What skin lesions are commonly caused by Churg-Strass syndrome?

Answer 41

Palpable purpura

Question 42

Why does Churg-Strass syndrome also cause GI and renal impairment?

Answer 42

Causes mesenteric vasculitis

Question 43

What is the main finding of blood tests and biopsies, in Churg-Strass syndrome?

Answer 43

Eosinophilia

Question 44

When Churg-Strass is ANCA-associated, which antibody is it most commonly associated with?

Answer 44

MPO/P-ANCA

Question 45

Which 2 drugs are both used to treat active Churg-Strass syndrome?

Answer 45

high-dose glucocorticoids and cyclophosphamide

Question 46

Which 2 drugs are both used to maintain Churg-Strass syndrome in remission?

Answer 46

low-dose glucocorticoids and azathioprine, methotrexate or MMF