Endocrine Abstracts

Introduction: Wegener’s granulomatosis is a necrotizing vasculitis that can affect the central nervous system. Pituitary involvement is exceptional, but may be underestimated. Pituitary involvement in Wegener’s disease is rare, and is mainly expressed as diabetes insipidus.

Clinical case: A 33-year-old man was admitted to hospital in 2009 with bilateral optic neuropathy, altered general condition revealing Wegener’s disease with rhinosinus involvement, pachymeningitis and pulmonary involvement. The patient was treated with high-dose corticosteroids and monthly boli of Endoxan®, with stabilization of his disease. In 2011, he presented with polyuro-polydypsia. The diagnosis, of central diabetes insipidus, was confirmed by a water restriction test with desmopressin injection. Pituitary MRI showed a disappearance of the usual hypersignal of the post-pituitary on T1-weighted sequences. There was no anteropituitary deficit or hyperprolactinemia. The patient was started on methotrexate 30 mg/week and high-dose corticosteroids, methotrexate and desmopressin. Progression was marked by persistent symptomatology requiring maintenance of desmopressin.

Conclusion: Diabetes insipidus often regresses with general treatment of Wegener’s disease. Nevertheless, desmopressin substitution is often necessary, at least initially. Occasionally, pituitary involvement persists despite effective treatment of Wegener’s granulomatosis, suggesting glandular destruction.