Sudden death and Wegener's granulomatosis of the pituitary

Involvement of brain parenchyma or meninges in ANCA-associated small-vessel vasculitis such as Wegener's granulomatosis (WG) is not uncommon. In contr ast, involvement of the pituitary is exceedingly rare with only a few cases reported so far. The diagnosis is usually made on the basis of imaging tec hniques and abnormal pituitary function tests in the setting of active syst emic vasculitis. However, histology-proven involvement of the pituitary by WG has not been reported so far. We report a case of WG with histology-prov en granulomatous necrotizing inflammation of the pituitary and hypothalamo- pituitary stalk, disclosed at autopsy after the patient had died suddenly a nd unexpectedly in his sleep. Tn a setting of histology-proven WG, these fi ndings were regarded as a pituitary manifestation of the disorder. A distin ct cause of death could not be found, hence we speculate that hypothalamo-p ituitary inflammation due to WG may have caused the sudden death in this pa tient.