Multiple endocrine abnormalities

Abstract

In September, 2008, a 48-year-old woman was admitted because of nausea, vomiting, and malaise. She had a history of maxillary sinusitis and otitis media. Physical examination was normal. Laboratory tests showed increased concentrations of infl ammatory markers (CRP 51 mg/L, ESR 90 mm/h), hypernatraemia (150 mmol/L), and hypercalcaemia (2·97 mmol/L, PTH 1·3 pmol/L). Central diabetes insipidus was confi rmed by high plasma osmolality and undetectable ADH. All other hormonal axes were tested and showed hypogonadotropic hypogonadism (LH <0·1 IU/L, FSH 1·2 IU/L), mild hyperprolactinaemia (1·36 U/L), and, surprisingly, primary hyperthyroidism (thyrotropin 0·006 mU/L, free thyroxine 24·2 pmol/L). MRI of her brain showed a diff use enlarged pituitary gland with a swollen stalk.Because chest radiography and CT showed multiple pulmonary infi ltrates, we suspected a granulomatous disease. Tuberculin skin test was negative, ACE concentrations were normal, but antineutrophil cytoplas matic antibodies (ANCA) were positive (c-ANCA 1:128, PR3-ANCA). Fluoro-D-Glucose FDG)- (PET-CT showed increased FDG uptake in the pituitary gland, left nostril, and left lung, and a focal active lesion in the left lobe of the thyroid gland (fi gure). Nasal biopsy showed active, chronic ulcerative infl ammation. Fine-needle aspiration of the thyroid lesion showed infl ammation with giant cells. A diagnosis of granulomatosis with polyangiitis (Wegener’s granulo-matosis) with pulmonary, nasal, pituitary, and probable thyroidal involvement was made. We treated our patient with 1000 mg methylprednisolone intravenously (3 days), followed by oral prednisolone (60 mg, tapering dose), and oral cyclophosphamide (2 mg/kg per day). Central diabetes insipidus was treated with oral desmopressin. There was no clinical need for oestrogen supplemention. Thyroid dysfunction and the hyper-calcaemia resolved soon after initiation of treatment. When last seen in May, 2011, she was in a good clinical condition without relapse.Pituitary insuffi ciency due to hypophysitis as the fi rst presentation of granulomatosis with polyangiitis is rare and therefore not the fi rst diagnosis that came to mind with our patient. The presence of pituitary-stalk thickening is the most striking radiological feature of an infl ammatory process involving the pituitary gland.