A previously fit and well
31-year-old male patient requiring ventilation for severe respiratory failure
characterised by haemoptysis and hypoxia was found to have diffuse pulmonary
haemorrhage. He also tested positive for haematuria and proteinuria. His tests for c-ANCAs
(classical antineutrophil cytoplasmic antibodies) were positive, confirming the
diagnosis of Wegener’s granulomatosis. Once the diagnosis has been made, which one
of the following would be the PREFERRED drug therapy?
a) Cyclophosphamide i.v. 1
g/day
b) Methylprednisolone i.v.
30 mg/day
c) Ciclosporin i.v. 200
mg/day
d) Azathioprine i.v. 250
mg/day
e) Methotrexate i.v. 7.5
mg/week
Answer: a
Explanation
Even though both high dose
intravenous steroids and cyclophosphamide are used in the acute management of
organ-threatening Wegener’s granulomatosis, the answer to this question is cyclophosphamide as it is usually the specific definitive
treatment for this condition and the
dose of methylprednisolone is too low. Ciclosporin has been used in the management of
patients with Wegener’s granulomatosis who have not been successfully treated
with cyclophosphamide and steroids. Azathioprine and methotrexate are both used
in the longer term management of Wegener’s granulomatosis, once the patient is in
remission, as they are less toxic.
No comments:
Post a Comment