Abstract
BackgroundGranulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis) is a multisystem vasculitis of small- to medium-sized blood vessels. Cranial involvement can result in cranial nerve palsies and, rarely, pituitary infiltration.Case presentationWe describe the case of a 32 year-old woman with limited but severe GPA manifesting as progressive cranial nerve palsies and pituitary dysfunction. Our patient initially presented with localised ENT involvement, but despite treatment with methotrexate, she deteriorated. Granulomatous inflammatory tissue around the skull base resulted in cavernous sinus syndrome, facial nerve palsy, palsies of cranial nerves IX-XII (Collet-Sicard syndrome), and the rare complication of cranial diabetes insipidus due to pituitary infiltration. The glossopharyngeal, vagus and accessory nerve palsies resulted in severe dysphagia and she required nasogastric tube feeding. Her neurological deficits substantially improved with treatment including high dose corticosteroid, cyclophosphamide and rituximab.ConclusionsThis case emphasises that serious morbidity can arise from localised cranial Wegener’s granulomatosis in the absence of systemic disease. In such cases intensive induction immunosuppression is required. Analysis of previously reported cases of pituitary involvement in GPA reveals that this rare complication predominantly affects female patients.
Highlights
Granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis) is a multisystem vasculitis of small- to medium-sized blood vessels
This case emphasises that serious morbidity can arise from localised cranial Wegener’s granulomatosis in the absence of systemic disease
Analysis of previously reported cases of pituitary involvement in GPA reveals that this rare complication predominantly affects female patients
Summary
Pituitary involvement is a rare complication of GPA It disproportionately affects female patients and tends to occur early in the disease course. Organ- or even life-threatening disease can occur in the absence of pulmonary or renal involvement. In this case a good outcome was achieved with high-dose steroid and dual treatment with cyclophosphamide and rituximab with minimal adverse events. In cases of severe neurological compromise due to GPA where a rapid therapeutic effect is required, such combination therapy may be a useful strategy. Such cases should be managed by a multi-disciplinary team in a tertiary referral centre
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