Wegener's granulomatosis (aka granulomatosis with polyangitis) Flashcards
1
Q
Why does it have 2 names?
A
Wegener was a Nazi!!!
2
Q
What is it?
A
A primary small vessel vasculitis (ANCA) associated
Multisystem disorder (affecting vasculature of all organs)
3
Q
where does it have a predilection for?
A
upper RT, lungs and kidneys
4
Q
pathophysiology
A
necrotising, granulomatous vasculitis of arterioles, capillaries and post capillary venules
5
Q
clinical presentation
A
Upper airway disease: nasal obstruction, ulcers, “saddle nose” deformity
Pulmonary involvement (cough, haemopysis or pleuritis)
Renal disease –> rapidly progressive glomerulonephritis w crescent formation, proteinuria or hematuria
6
Q
Diagnostic tests
A
exclude infection and malignancy ESR/CRP c-ANCA +ve (only association) Urinalysis Biopsy
7
Q
Treatment
A
steroids + immunosuppression
high dose steroids if severe
if non-end organ threatening: moderate doses
?plasma exchange
