Wegener's granulomatosis

Question 1

what is the definition of wegener’s granulomatosis?

Answer 1

Granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) is a systemic vasculitis that typically involves small and medium vessels

Question 2

what are the risk factors for wegeners granulomatosis?

Answer 2

genetic predisposition
infection
environmental exposures

Question 3

what are the key presentations for wegeners granulomatosis?

Answer 3

upper and lower respiratory tract involvement
renal involvement
constitutional features

Question 4

what are the signs of wegeners granulomatosis?

Answer 4

ocular manifestations
cutaneous manifestations
musculoskeletal manifestations
neurological manifestations

Question 5

what are the first line and gold standard investigations for wegeners granulomatosis?

Answer 5

urinalysis and microscopy
CT chest
anti-neutrophil cytoplasmic antibody (ANCA)
FBC and differential

Question 6

what are the differential diagnoses for wegeners granulomatosis?

Answer 6

Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome)
Microscopic polyangiitis (MPA)
Classic polyarteritis nodosa (cPAN)

Question 7

how is wegeners granulomatosis managed?

Answer 7

Induction of remission was traditionally achieved using cyclophosphamide and high-dose corticosteroids. Methotrexate may effectively substitute for cyclophosphamide in granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) that is not immediately life- or organ-threatening. Remission can be maintained using low-dose corticosteroids and either azathioprine or methotrexate. Rituximab has demonstrated efficacy and safety as an alternative to cyclophosphamide for induction of remission in severe disease, particularly in the setting of disease relapse. However, the optimal timing and dosing for remission maintenance has not been clearly defined, and questions remain regarding the long-term safety profile of rituximab in this population

Question 8

how is wegeners granulomatosis monitored?

Answer 8

Anti-neutrophil cytoplasmic antibody (ANCA) testing may help with diagnosis, but is not reliable for monitoring disease activity

Question 9

what is the prognosis for wegeners granulomatosis?

Answer 9

With appropriate therapy, patients generally experience prolonged survival with a chronic relapsing course. Most patients develop disease- and/or treatment-related morbidity during the course of their disease