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Clinical lectures > Small-vessel vasculitis: ANCA-associated vasculitis and non-ANCA-associated vasculitis > Flashcards

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

1
Q

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

A

Fibrinoid necrosis

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2
Q

Why does ANCA-associated vasculitis result in circulating autoantibodies?

A

Presence of Anti-Neutrophil Cytoplasmic Antibodies (ANCAs)

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3
Q

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

A

Microscopic polyangiitis

Granulomatosis with polyangiitis

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4
Q

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

A

High-dose glucocorticoids

IV cyclophosphamide

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5
Q

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

A

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

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6
Q

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

A

High risk of relapse that can ultimately lead to renal failure

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7
Q

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

A

Non-granulomatous

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8
Q

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

A

Necrotising

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9
Q

Which vessels are commonly affected by microscopic polyangiitis?

A

Capillaries
Venules
Arterioles

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10
Q

Are all ages equally affected by microscopic polyangiitis?

A

Typically occurs in older adults but children can also be affected

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11
Q

What is the characteristic renal symptom of microscopic polyangiitis?

A

Rapid, necrotising glomerulonephritis

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12
Q

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

A

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

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13
Q

What is the uncommon pulmonary symptom of microscopic polyangiitis?

A

Pleural effusions

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14
Q

Is neuropathy common in microscopic polyangiitis?

A

No, only occurs in 15% cases

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15
Q

Can microscopic polyangiitis cause skin symptoms?

A

Yes

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16
Q

Can microscopic polyangiitis cause heart symptoms?

A

Yes

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17
Q

Can microscopic polyangiitis cause GI symptoms?

A

Yes

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18
Q

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

A

Myeloperoxidase antibody (MPO)

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19
Q

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

A

Segmental fibrinoid necrosis with non-granulomatous inflammation

20
Q

Describe the ESR and CRP levels in microscopic polyangiitis?

A

Elevated

21
Q

Describe the WCC in microscopic polyangiitis?

A

Elevated

22
Q

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

A

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

23
Q

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

A

CT or MRI of chest

24
Q

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

A

Necrotising

25
Q

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

A

Respiratory tract (ears, nose, sinuses, throat)

Lungs

Kidneys

26
Q

Is granulomatosis with polyangiitis equally common in men and women?

A

No

More common in men

27
Q

Does granulomatosis with polyangiitis affect all ages equally?

A

Most common in middle-aged people

28
Q

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

A

Hearing problems/deafness

29
Q

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

A

Sinusitis

30
Q

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

A

Pus-like drainage with crusting from nose

Stuffy nose

31
Q

What systemic symptom commonly occurs in granulomatosis with polyangiitis?

A

Fever

32
Q

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

A

Glomerulonephritis

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

33
Q

Describe the ESR and CRP levels in granulomatosis with polyangiitis?

A

Elevated

34
Q

Describe the WCC in granulomatosis with polyangiitis?

A

Elevated

35
Q

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

A

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

36
Q

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

A

Necrotising vasculitis associated with peripheral eosinophilia

37
Q

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

A

Granulomatosis with polyangiitis doesn’t involve peripheral eosinophilia

Churg-Strass syndrome (eosinophilic granulomatosis with polyangiitis) involves peripheral eosinophilia

38
Q

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

A

Prodromal/allergic phase

Can last years

39
Q

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

A

Allergic rhinitis (irritated nose due to allergy)

Nasal polyposis

40
Q

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

A

Late-onset asthma

Pleural effusions

Pulmonary infiltrates (substances denser than air enter lungs)

41
Q

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

A

Palpable purpura

42
Q

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

A

Causes mesenteric vasculitis

43
Q

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

A

Eosinophilia

44
Q

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

A

MPO/P-ANCA

45
Q

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

A

high-dose glucocorticoids and cyclophosphamide

46
Q

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

A

low-dose glucocorticoids and azathioprine, methotrexate or MMF

Clinical lectures (29 decks)

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