title: Refractory pituitary granulomatosis with polyangiitis (Wegener's) treated with rituximab.
abstract: OBJECTIVE
  Granulomatosis with polyangiitis (GPA), also known as Wegener's granulomatosis, is an autoimmune disease characterized by inflammation of blood vessels most often seen in the upper respiratory tract, lungs, kidneys, and skin. Central nervous system (CNS) involvement of GPA is rare, particularly in the pituitary, and can be difficult to treat.
  
  
  METHODS
  Case report.
  
  
  RESULTS
  We present a 30-year-old woman with pituitary and ocular GPA, whose unusually recalcitrant disease led to the development of pan-hypopituitarism and near-total vision loss. After failing multiple systemic immunosuppressants, she was ultimately treated with the novel immunomodulatory agent rituximab together with pulse corticosteroids, which achieved a gratifying response.
  
  
  CONCLUSIONS
  Pituitary and optic chiasm involvement is a rare complication of GPA. We believe this case illustrates the complexity of management of pituitary GPA and provides insight into the potential utility of the biologic agent rituximab in this disease.
authors.affiliation: Section of Endocrinology and Metabolism, Yale University School of Medicine, New Haven, CT 06519, USA. jing.hughes@yale.edu
authors.forename: Jing
authors.initials: J
authors.lastname: Hughes
authors.forename: Garni
authors.initials: G
authors.lastname: Barkhoudarian
authors.forename: Pedro
authors.initials: P
authors.lastname: Ciarlini
authors.forename: Edward R
authors.initials: ER
authors.lastname: Laws
authors.forename: Elinor
authors.initials: E
authors.lastname: Mody
authors.forename: Silvio E
authors.initials: SE
authors.lastname: Inzucchi
authors.forename: Whitney W
authors.initials: WW
authors.lastname: Woodmansee
mesh_terms: D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000188:drug therapy* / Q000601:surgery; D006801:Humans; D007018:Hypopituitarism / Q000150:complications / Q000188:drug therapy* / Q000601:surgery; D007155:Immunologic Factors / Q000627:therapeutic use*; D010902:Pituitary Gland / Q000601:surgery*; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab