Wegener granulomatosis is an antineutrophil cytoplasmic antibody (cANCA)-associated, multi-system, necrotizing granulomatous vasculitis. Inflammation of nasal or oral mucosa, lung and kidney involvements are typical in the course of the disease. It was first described by Friedrich Wegener in 1939, and has an incidence rate of 1 in 100 000 people, often observed in individuals aged between 25 and 50 years, but uncommon in children and adolescent. In rare cases, pituitary involvement may occur and cause panhypopituitarism. Pituitary involvement is very rare especially in pediatric population. This is a case report of an adolescent patient who presented with panhypopituitarism symptoms and was later diagnosed with WG. Sixteen years-old female patient complained fever, headache, purulent nasal discharge and severe muscle and joint pain. Additionally she had polyuria and polydipsia. Investigations revealed pituitary mass and panhypopituitarism. The MR imaging showed a high-signal-intensity mass, which had a height of 1519 mm, suspicious necrotic hemorrhagic regions, thickening of the pituitary stalk, hypothalamic involvement, loss of the hyperintensity of the neurohypophysis. Positivity of c-ANCA and renal biopsy result compatible with Wegeners granulomatosis confirmed the diagnosis. The patient was started on conventional treatment, which leads to the shrinking of the granuloma in the pituitary gland. A relapse did not occur over the one-year follow-up period, and the patient was still under hormone replacement therapy. WG should be considered in pediatric patients with panhypopituitarism symptoms when suspected of a granulomatous disease due to the involvement of other organs. Necrotic granulomatous lesions in MR imaging of the pituitary and ANCA positivity serve as guides in WG diagnosis.
27 Apr - 01 May 2013
European Society of Endocrinology