Small-vessel vasculitis: ANCA-associated vasculitis and non-ANCA-associated vasculitis Flashcards
What type of necrosis does ANCA-associated vasculitis result in?
Fibrinoid necrosis
Why does ANCA-associated vasculitis result in circulating autoantibodies?
Presence of Anti-Neutrophil Cytoplasmic Antibodies (ANCAs)
What are the 2 main types of ANCA-associated vasculitis?
Microscopic polyangiitis
Granulomatosis with polyangiitis
Which 2 medications are both used to treat active ANCA-associated vasculitis?
High-dose glucocorticoids
IV cyclophosphamide
Which medications are both used to manage ANCA-associated vasculitis in remission?
Glucocorticoids with an immunosuppressant such as azathioprine, methotrexate, MMF, rituximab
Why do ANCA-associated vasculitis remission patients need to be regularly monitored?
High risk of relapse that can ultimately lead to renal failure
Is microscopic polyangiitis a type of granulomatous or non-granulomatous vasculitis?
Non-granulomatous
Is microscopic polyangiitis a type of necrotising or non-necrotising vasculitis?
Necrotising
Which vessels are commonly affected by microscopic polyangiitis?
Capillaries
Venules
Arterioles
Are all ages equally affected by microscopic polyangiitis?
Typically occurs in older adults but children can also be affected
What is the characteristic renal symptom of microscopic polyangiitis?
Rapid, necrotising glomerulonephritis
What is the characteristic pulmonary symptom of microscopic polyangiitis, and what complication does this cause?
Pulmonary capillaritis (alveolar wall inflammation) which presents as alveolar haemorrhage
What is the uncommon pulmonary symptom of microscopic polyangiitis?
Pleural effusions
Is neuropathy common in microscopic polyangiitis?
No, only occurs in 15% cases
Can microscopic polyangiitis cause skin symptoms?
Yes
Can microscopic polyangiitis cause heart symptoms?
Yes
Can microscopic polyangiitis cause GI symptoms?
Yes
Which type of antibodies are microscopic polyangiitis cases mostly positive for?
Myeloperoxidase antibody (MPO)
What is the characteristic finding from a biopsy, in microscopic polyangiitis?
Segmental fibrinoid necrosis with non-granulomatous inflammation
Describe the ESR and CRP levels in microscopic polyangiitis?
Elevated
Describe the WCC in microscopic polyangiitis?
Elevated
What is the typical result of the ANCA test in microscopic polyangiitis?
ANCA test is positive and shows presence of MPO-ANCA/P-ANCA (autoantibodies that target myeloperoxidase)
Which 2 radiological investigations are done in ANCA-associated vasculitis?
CT or MRI of chest
Is granulomatosis with polyangiitis a type of necrotising or non-necrotising vasculitis?
Necrotising
In which 3 places are capillaries, arterioles and venules characteristically inflamed in granulomatosis with polyangiitis?
Respiratory tract (ears, nose, sinuses, throat)
Lungs
Kidneys
Is granulomatosis with polyangiitis equally common in men and women?
No
More common in men
Does granulomatosis with polyangiitis affect all ages equally?
Most common in middle-aged people
What common symptom presents due to ear involvement in granulomatosis with polyangiitis?
Hearing problems/deafness
What common symptom presents due to sinus involvement in granulomatosis with polyangiitis?
Sinusitis
What 2 common symptoms presents due to nose involvement in granulomatosis with polyangiitis?
Pus-like drainage with crusting from nose
Stuffy nose
What systemic symptom commonly occurs in granulomatosis with polyangiitis?
Fever
What common symptom occurs due to kidney involvement in granulomatosis with polyangiitis, and give 3 signs?
Glomerulonephritis
Signs include: Shortness of breath, fatigue, haematuria (if severe)
Describe the ESR and CRP levels in granulomatosis with polyangiitis?
Elevated
Describe the WCC in granulomatosis with polyangiitis?
Elevated
What is the typical result of the ANCA test in granulomatosis with polyangiitis?
ANCA test is positive and shows presence of PR3-ANCA/C-ANCAA (target proteinase 3)
What is eosinophilic granulomatosis with polyangiitis/Churg-Strass syndrome?
Necrotising vasculitis associated with peripheral eosinophilia
How is Churg-Strass syndrome different from granulomatosis with polyangiitis?
Granulomatosis with polyangiitis doesn’t involve peripheral eosinophilia
Churg-Strass syndrome (eosinophilic granulomatosis with polyangiitis) involves peripheral eosinophilia
What phase occurs before the onset of Churg-Strass syndrome?
Prodromal/allergic phase
Can last years
Give 2 ways in which the allergic phase of Churg-Strass syndrome affects the nose?
Allergic rhinitis (irritated nose due to allergy)
Nasal polyposis
What 3 respiratory conditions are symptoms of Churg-Strass syndrome?
Late-onset asthma
Pleural effusions
Pulmonary infiltrates (substances denser than air enter lungs)
What skin lesions are commonly caused by Churg-Strass syndrome?
Palpable purpura
Why does Churg-Strass syndrome also cause GI and renal impairment?
Causes mesenteric vasculitis
What is the main finding of blood tests and biopsies, in Churg-Strass syndrome?
Eosinophilia
When Churg-Strass is ANCA-associated, which antibody is it most commonly associated with?
MPO/P-ANCA
Which 2 drugs are both used to treat active Churg-Strass syndrome?
high-dose glucocorticoids and cyclophosphamide
Which 2 drugs are both used to maintain Churg-Strass syndrome in remission?
low-dose glucocorticoids and azathioprine, methotrexate or MMF
