Fatal inflammatory hypophysitis.

Abstract:

:A young female patient presented as an acute medical emergency with hypoglycaemia. Investigations revealed panhypopituitarism and an inflammatory pituitary mass. An antibody screen was negative for anti-neutrophil cytoplasmic antibodies with cytoplasmic distribution (cANCA). Pituitary histology showed lymphocytic infiltration and a few Langerhan's cells. The pituitary mass rapidly expanded to involve the optic nerves and led to bilateral blindness. Later, the patient developed diarrhoea, a vasculitis rash, scleritis, and proteinuria. In subsequent investigations cANCA became positive. The patient responded to steroids and cyclophosphamide treatment and remained in partial remission for six months before dying of severe sepsis. This is the first description of Wegener's granulomatosis presenting with acute anterior pituitary failure in the absence of other organ involvement and negative serology.

journal_name

Pituitary

journal_title

Pituitary

authors

McIntyre EA,Perros P

doi

10.1007/s11102-007-0016-z

subject

Has Abstract

pub_date

2007-01-01 00:00:00

pages

107-11

issue

1

eissn

1386-341X

issn

1573-7403

journal_volume

10

pub_type

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