Panhypopituitarism due to Wegener's granulomatosis

Abstract

SUMMARYWegener's granulomatosis (WG) is a multi-system necrotizing granulomatous vasculitis which classically affects the upper respiratory tract, lungs and kidneys. Pituitary participation has been described in 24 patients in the literature to date. The aim of this article is to report a case of pituitary involvement in WG, and to present a literature review on this association. We present a female patient with WG who evolved with central diabetes insipidus (CDI), panhypopituitarism, and mild hyperprolactinemia. MRI showed an infiltrative pattern. Pituitary involvement has been reported in around 1% of patients with WG, mostly in women. It is represented by CDI and hypopituitarism. MRI generally shows pituitary enlargement, stalk thickening and loss of hyperintensity of the neurohypophysis. Permanent endocrine therapy is generally needed. WG should be considered in cases of CDI and hypopituitarism, essentially if a vasculitis is suspected and more common sellar disorders have been ruled out. Arq Bras Endocrinol Metab. 2011;55(7):481-5 SUMáRio A granulomatose de Wegener (GW) é uma vasculite necrotizante multissistêmica que afeta classicamente o trato respiratório superior, pulmões e rins. O envolvimento da hipófise foi descrita em 24 pacientes na literatura, até hoje. O objetivo deste artigo é relatar um caso de GW com envolvimento pituitário, e apresentar uma revisão da literatura sobre esta associação. Apresentamos uma paciente com GW que evoluiu para diabetes insipidus central (DIC), panhipopituitarismo e leve hiperprolactinemia. A RM mostrou um padrão infiltrativo. O envolvimento da hipó-fise foi relatado em cerca de 1% dos pacientes cm GW, na sua maioria mulheres. A desordem é representada por DIC e hipopituitarismo. A RM geralmente mostra o aumento da hipófise, aumento da espessura da haste, e perda da hiperintensidade da neurohipofise. Normalmente, é necessária terapia endócrina permanente. A GW é geralmente considerada nos casos de DIC e hipopituitarismo, essencialmente se há suspeita de vasculite e quando desordens selares mais comuns foram descartadas. Arq Bras Endocrinol Metab. 2011;55(7):481-5

Pituitary Dysfunction in Granulomatosis With Polyangiitis: The Mayo Clinic Experience

“…As is the case in our series, isolated anterior pituitary dysfunction is less common and is almost always accompanied by DI (12,15,16). Isolated anterior pituitary dysfunction was noted in one of our patients (12.5%) and has been previously reported in about 13% of the patients with pituitary dysfunction due to GPA (7).…”

Successful treatment with rituximab in relapse of granulomatosis with polyagiitis presenting with diabetes insipidus

“…These are direct involvement of vessels of the pituitary, dissemination of granulomas from nearby anatomic areas such as the sinus or orbits, and the formation of new granulomas in the pituitary due to direct involvement of the gland. 1,3 The most frequent manifestation is central diabetes insipidus with or without combined anterior hypopituitarism. 3 Most frequent MRI finding is pituitary enlargement, thickening of the pituitary stalk and loss of classic hyperintensity of the neurohypophysis on T1 images.…”

“…1,3 The most frequent manifestation is central diabetes insipidus with or without combined anterior hypopituitarism. 3 Most frequent MRI finding is pituitary enlargement, thickening of the pituitary stalk and loss of classic hyperintensity of the neurohypophysis on T1 images. 2,3 Sellar mass or cyst has been described.…”

“…3 Both stalk thickening and loss of the hyperintensity of the neurohypophysis generally remain unaltered in spite of adequate general response to therapy. 3 The majority of cases sustain permanent damage to the pituitary gland even with remission of the underlying granulomatous disease. 2,3 In most of the reported cases cyclophosphamide based induction therapy was used.…”

“…3 The majority of cases sustain permanent damage to the pituitary gland even with remission of the underlying granulomatous disease. 2,3 In most of the reported cases cyclophosphamide based induction therapy was used. 2 Biological agents are now being increasingly used in refractory cases.…”