Diabetes insipidus and anterior pituitary insufficiency as presenting features of Wegener's granulomatosis

Abstract

Central diabetes insipidus (DI) is a rare complication of Wegener's granulomatosis (WG), which usually presents after pulmonary or kidney involvement. Anterior pituitary dysfunction secondary to WG has been extremely rare, documented in only three cases. We report a case of a 47-year-old postmenopausal woman who was diagnosed with hypopituitarism in November 1999 and started on vasopressin, thyroxine, and hydrocortisone. She sought treatment at the Mayo Clinic in February 2000 with a purpuric rash, fever, cough, shortness of breath, and blood in the sputum. Computed tomography of the chest showed a 6-cm irregular mass in the right lower lobe, and a biopsy of the mass showed marked reactive atypia and necrosis. Positive C-antineutrophil cytoplasmic antibodies (ANCA) and skin biopsy of a purpuric lesion showing leukocytoclastic vasculitis confirmed the diagnosis of WG. Hormonal studies showed low gonadotropins, thyroid-stimulating hormone (TSH), and prolactin. Magnetic resonance imaging (MRI) of the head showed cystic enlargement of the pituitary gland that did not enhance with gadolinium. Two months into the treatment with cyclophosphamide and prednisone, she had persistent pituitary dysfunction, despite the normal appearance of the pituitary gland on repeat MRI. We conclude that WG should be included in the differential diagnosis of DI and anterior pituitary dysfunction in the proper clinical setting. Early diagnosis and treatment may be crucial in preventing pituitary gland destruction and long-term endocrine sequelae. We suggest screening for anterior pituitary failure in the presence of the WG-associated DI.