Summary

Wegener’s granulomatosis (WG) is a systemic disease with a complex genetic background. It is characterised by inflammation of the small blood vessels leading to damage in any number of organs. The common features include granulomatous inflammation of the respiratory tract and kidneys. Most patients have measurable autoantibodies against neutrophil proteinase-3 (Antineutrophil Cytoplasmic Antibody, ANCA). Pituitary involvement is a rare complication of this disease and, when it occurs, diabetes insipidus is the most common manifestation. We describe a 38-year-old female with known long-term WG who presented with partial hypopituitarism, severe malnutrition and ANCA negative status, with a favourable response to steroid pulse therapy.