Rare presentation of Wegener’s granulomatosis in the pituitary gland: Case report and literature review

Abstract

HighlightsPituitary involvement is a rare recognized complication of Wegener’s granulomatosis.Central diabetes insipidus and varying hypopituitarism is a common presentation.Radiological abnormalities occur in greater than 90% of cases.Surgery is indicated to confirm diagnosis or when compressive symptoms develop.Long-term prognosis of patients with pituitary WG is unknown.

Granulomatosis with polyangiitis: potentially lethal gingival lesions presenting to the dentist

“…GPA or Wegener granulomatosis is a rare autoimmune multisystemic disease of unknown cause affecting 1–9:100 000 people in Europe,4–9 and 500 men and women are diagnosed each year within the UK 10. It is often associated with necrotising granulomas commonly affecting the upper and lower respiratory tracts, vasculitis of small and medium blood vessels, and glomerulonephritis 9 11–13. In its later stages, it has been known to affect the oral cavity, eyes, nose and very rarely the central nervous system and pituitary gland 12.…”

“…It is often associated with necrotising granulomas commonly affecting the upper and lower respiratory tracts, vasculitis of small and medium blood vessels, and glomerulonephritis 9 11–13. In its later stages, it has been known to affect the oral cavity, eyes, nose and very rarely the central nervous system and pituitary gland 12. It can present as a limited disease affecting only one area, however, the disseminated version can rapidly progress leading to life threatening multisystem organ failure 4–9.…”

Granulomatosis with Polyangiitis Manifesting as a Symptomatic Sellar Mass in a Young Woman

“…Pituitary lesions are exceedingly uncommon; in a study of 819 patients with GPA, only 9 (1.1%) had pituitary involvement [3]. We identified 57 reported cases of GPA with pituitary involvement in the literature to date, with diabetes insipidus presenting often and panhypopituitarism presenting as a less common complication [3][4][5][6][7][8]. Here we describe the case of a young woman with GPA who presented with symptoms initially suggestive of meningitis, but on further evaluation had hypopituitarism and was found to have an enlarging pituitary mass caused by GPA.…”

Pituitary hypophysitis in granulomatosis with polyangiitis (GPA): a case series

“…Arginine Vasopressin deficiency (AVPD) due to the infiltration of the posterior pituitary appears to be the most frequent hormone deficiency (67-78%) followed by secondary hypothyroidism (50-71%) and ACTH deficiency (11-12.5%) [3][4][5]. Proposed mechanisms include vasculitis of central nervous system blood vessels and granuloma formation within the pituitary or invasion from nearby anatomic areas such as the paranasal sinuses or orbits [6]. Current treatment algorithms for systemic GPA recommend remission induction with highdose glucocorticoids together with cyclophosphamide or rituximab (RTX) followed by a less immunosuppressive maintenance therapy of RTX and/or conventional steroidsparing agents [7].…”