Department of Nephrology and Transplantation and Department of Radiology, Royal Free Hospital, London, UK
A 57-year-old woman presented with tiredness, thirst, polyuria, and a bitemporal hemianopia. Magnetic resonance imaging (MRI) showed a pituitary mass, which was removed surgically. The mass recurred 10 months later (Figure 1) and a second resection was performed. Histology showed granulomatous inflammation. The patient received cyclophosphamide 50 mg daily and dexamethasone 4 mg t.d.s. for 3 weeks. A repeat MRI showed reduction in the size of the mass (Figure 2
). Six months later the patient was readmitted with lethargy, fever, vasculitic rash, new deafness, and sinusitis. She had a high C-reactive protein, positive antineutrophil cytoplasmic antibodies (having been negative before) with PR3 titre of 4.7 (normal range 02), neutrophilia, thrombocytosis, and impaired renal function (creatinine 102 µmol/l, creatinine clearance 60 ml/min, and urinary protein 0.7 g/24 h). A diagnosis of Wegener's granulomatosis was made and oral cyclophosphamide was started. The patient became progressively short of breath and hypoxic. Her haemoglobin decreased to 5 g/dl and a chest X-ray was consistent with pulmonary haemorrhage (Figure 3
). She was ventilated in the intensive care unit and received plasma exchange.
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Wegener's granulomatosis is a systemic necrotizing vasculitis that usually affects the respiratory tract and the kidneys. Associated neurological manifestations have been described in 2254% of cases and most commonly involve the peripheral nerves [1]. Involvement of the CNS has been documented in up to 12% of cases. Pituitary involvement has been described in only a small proportion and has predominantly affected posterior pituitary, invariably in association with other systemic manifestation of the disease [2,3]. Systemic vasculitis is a rare cause of granulomatous pituitary destruction and can be controlled with aggressive immunosuppression.
Notes
Correspondence and offprint requests to: R. Tappouni, Department of Nephrology and Transplantation, Royal Free Hospital, Pond Street, London, UK.
References