Severe localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review

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.

Hypophysis Involvement in Granulomatosis With Polyangiitis

“…Generally, patients with pituitary involvement are younger, and symptoms of pituitary disease precede other systemic features . Immunosuppressive therapy is needed to control disease activity in GPA, and glucocorticoids along with rituximab, CYC, or methotrexate have been used to treat pituitary involvement . In our patient, early treatment with high‐dose steroids and pulse CYC resulted in a good outcome after 8 months, although permanent desmopressin treatment was required as a consequence of surgery.…”

“…Involvement of the pituitary gland in GPA is uncommon, with an estimated prevalence of nearly 1% . Generally, patients with pituitary involvement are younger, and symptoms of pituitary disease precede other systemic features . Immunosuppressive therapy is needed to control disease activity in GPA, and glucocorticoids along with rituximab, CYC, or methotrexate have been used to treat pituitary involvement .…”

Atypical Manifestations of Granulomatosis with Polyangiitis: The Diagnostic Challenge for Pulmonologists

“…Regarding pituitary involvement in GPA, recent search for the cases indicated in the literature revealed 58 published reports. This rare complication more frequently affected females (69%) than males [88]. Moreover, pituitary involvement has predominantly concerned posterior gland and is usually associated with other organ involvement [88,89].…”

“…Moreover, pituitary involvement has predominantly concerned posterior gland and is usually associated with other organ involvement [88,89]. Numerous patients (43-73%) with pituitary manifestation of GPA have concomitant lung lesions [88,89]. Cranial diabetes insipidus was the most common endocrine abnormality found in 81% of cases [88].…”

“…This rare complication more frequently affected females (69%) than males [88]. Moreover, pituitary involvement has predominantly concerned posterior gland and is usually associated with other organ involvement [88,89]. Numerous patients (43-73%) with pituitary manifestation of GPA have concomitant lung lesions [88,89].…”