ANCA-associated Small Vessel Vasculitis

Question 1

Small vessels vasculitis are split into 2 things. What are they?

Answer 1

1. ANCA-associated Small Vessel Vasculitis
2. Non- ANCA-associated Small Vessel Vasculitis

Question 2

Non- ANCA-associated Small Vessel Vasculitis is split into what?

Answer 2

1. IgA dominant immune deposit
   yes = henoch-schnlein purpura

no = 2. serum cryoglobulin?

yes= cryoglobulinemia
no = other

Question 3

What is ANCA-associated small vessel vasculitis split into?

Answer 3

Granuloma present?
no = Microscopic polyangiitis

Question 4

What is the other name for Eosinophilic granulomatosis with polyangiitis?

Answer 4

Churg-strauss syndrome

Question 5

If granulomas are present in ANCA-associated Small Vessel Vasculitis what is it split into?

Answer 5

Asthma and eosinophilia?
yes = Eosinophilic granulomatosis with polyangiitis (EPGA)

no = Granulomatosis with polyangiitis (GPA)

Question 6

What is the other name for Granulomatosis with polyangiitis?

Answer 6

Wenger’s granulomatosis

Question 7

What ANCA-associated small vessel vasculitis autoantibody is present?

Answer 7

ANCA
IgG

Question 8

What is the primary characteristic of the vasculitis seen in Microscopic Polyangiitis (MPA)?

Answer 8

MPA is a necrotising vasculitis of small vessels with few immune deposits, typically affecting the lungs, kidneys, and skin

Question 9

Which organ involvement is very common in Microscopic Polyangiitis?

Answer 9

Necrotising glomerulonephritis

Question 9

What autoantibody is significant for Microscopic Polyangiitis?

Answer 9

pANCA

Question 10

How can Microscopic Polyangiitis be differentiated from Granulomatosis with Polyangiitis (GPA)?

Answer 10

MPA does not affect the nasopharynx and typically involves only the
(1) kidneys and lungs

while GPA affects the
(1) upper respiratory tract, including the nasopharynx

Question 11

What type of inflammation is seen in Eosinophilic Granulomatosis with Polyangiitis (EGPA)?

Answer 11

Eosinophilic granulomatous inflammation affects small and medium-sized vessels, most commonly
(1) respiratory tract
(2) skin

Question 11

What is the main cause of Eosinophilic Granulomatosis with Polyangiitis (EGPA)?

Answer 11

EGPA is secondary to asthma, often due to an increase in eosinophils in the body

Question 12

What is the main clinical feature that differentiates EGPA from Granulomatosis with Polyangiitis (GPA)?

Answer 12

1. Late-onset asthma
2. High eosinophil count
3. ANCA specificity

Question 13

Which antibodies are associated with Eosinophilic Granulomatosis with Polyangiitis (EGPA)?

Answer 13

Anti-MPO
Anti-PR3

Question 14

What is the typical age group for Granulomatosis with Polyangiitis (GPA)?

Answer 14

35-55 years of age, with a slightly higher incidence in middle-aged males

Question 15

What are the key respiratory symptoms in Granulomatosis with Polyangiitis (GPA)?

Answer 15

(1) bloody cough
(2) haemoptysis
(3) pulmonary infiltrates
(4) diffuse alveolar haemorrhage
(5) cavitating nodules on chest X-ray (CXR)

Question 15

What are some common clinical features of Granulomatosis with Polyangiitis (GPA) related to the nasopharynx?

Answer 15

(1) sinusitis
(2) nasal crushing
(3) epistaxis
(4) bloody mucus from mouth ulcers
(5) sensorineural deafness
(6) otitis media
(7) a ‘saddle nose’

Question 16

Which antibodies are associated with Granulomatosis with Polyangiitis (GPA)?

Answer 16

cANCA
Anti-PR3

Question 17

Which blood tests are typically raised in inflammatory vasculitis?

Answer 17

pANCA = MPO
cANCA = PR3

Question 18

What confirms the presence of vasculitis?

Answer 18

biopsy

Question 19

What is the treatment for early/localised vasculitis?

Answer 19

Steroids + methotrexate

Question 20

What is the treatment for generalised/systemic vasculitis?

Answer 20

IV steroids + cyclophosphamide

Question 21

What is the treatment for refractory vasculitis?

Answer 21

IV immunoglobulin + rituximab

Question 22

Perinuclear anti-nuclear cytoplasmic antibody (pANCA) - positive This suggests what?

Answer 22

Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis (EGPA)

Question 23

Cytoplasmic anti-nuclear cytoplasmic antibody (cANCA) - positive This suggests what?

Answer 23

Granulomatosis with polyangiitis (GPA) (Wegener’s)

Question 24

A 58-year-old man who was recently referred to rheumatology is undergoing further investigations due to his two-month history of fatigue, weight loss, joint pain, skin ulcers and haemoptysis. He is found to have new renal impairment and a renal biopsy confirms a small vessel vasculitis. The patient is subsequently diagnosed with microscopic polyangiitis . What investigation finding is most associated with this condition?

Answer 24

p-ANCA positive

Question 25

Which organ systems are primarily affected in GPA?

Answer 25

Upper and lower respiratory tract, kidneys, eyes, and skin

Question 26

What is the characteristic nasal deformity seen in GPA?

Answer 26

Saddle nose deformity

Question 27

What type of ANCA is most commonly associated with GPA?

Answer 27

cANCA (anti-PR3)

Question 28

Which antibody is most commonly associated with EGPA?

Answer 28

Anti-MPO and anti-PR3

Question 29

What is the most common ANCA type associated with MPA?

Answer 29

pANCA (anti-MPO)

Question 30

What blood markers are typically raised in ANCA-associated vasculitis?

Answer 30

ESR, PV, CRP

Question 31

A 45-year-old patient presents with bloody nasal discharge, joint pain, and rapidly progressive glomerulonephritis; given the most likely diagnosis, what autoantibody is most likely to be positive?

Answer 31

c-ANCA (PR3-ANCA)

Question 32

What are the key respiratory features of Granulomatosis with Polyangiitis (GPA)?

Answer 32

Upper respiratory symptoms = sinusitis, nasal crusting, nasal discharge Lower respiratory symptoms = shortness of breath, haemoptysis, chest pain

Question 33

Which antibody is classically associated with eosinophilic granulomatosis with polyangiitis?

Answer 33

p-ANCA

Question 34

A 36-year-old gentleman presents to a Rheumatology Clinic for a review. Over the last year, his health declined significantly. He has a history of asthma and later rhinorrhoea and nasal obstruction, which required surgical polyp removal. Subsequently, it was noted that his eosinophil count was 14% and he complained of fevers and malaise. Finally, he was seen in a neurology clinic and was diagnosed with peripheral neuropathy. Given the likely diagnosis, what is most likely to be elevated in this patient?

Answer 34

Eosinophilic Granulomatosis with Polyangiitis = therefore p-ANCA raised

Question 35

A 45-year-old man is referred by his GP to rheumatology due to late-onset asthma, persistent sinusitis and eosinophilia. He goes on to have further blood tests which show that he is positive for perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA), and a tissue biopsy, the results of which are also in line with the suspected diagnosis. Which renal biopsy finding is most expected?

Answer 35

Granuloma

Question 36

A 40-year-old man presents to the General Practice with shortness of breath and wheeze particularly noticeable on running for the bus in the morning. He is also complaining of a persistent blocked nose and paraesthesia in both feet. What findings are most likely to support the diagnosis?

Answer 36

MPO P-ANCA positive antibodies

Question 37

A 40-year-old man presents to the GP with fatigue and unintentional weight loss. He reports recurrent sinusitis and rashes of unknown cause. His only medical history consists of asthma which was diagnosed last year. His blood shows very high eosinophil levels. The GP suspects eosinophilic granulomatosis with polyangiitis (Churg-Strauss). What is the most appropriate initial treatment option?

Answer 37

Corticosteroids

Question 38

pANCA associated conditions are what

Answer 38

pee, poo, UC, Primary Sclerosing Cholangitis