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[IgG4 related disease presenting as panhypopituitarism and perimacular scotoma].

Authors
  • Sosa, Gabriela A1
  • Fainstein-Day, Patricia2
  • Christiansen, Silvia3
  • Ajler, Pablo4
  • Yampolsky, Claudio4
  • 1 Servicio de Endocrinología, Hospital Provincial de Unquillo, Córdoba, Argentina. , (Argentina)
  • 2 Servicio de Endocrinología, Metabolismo y Medicina Nuclear, Hospital Italiano de Buenos Aires. Argentina. E-mail: [email protected] , (Argentina)
  • 3 Servicio de Patología, Hospital Italiano de Buenos Aires, Argentina. , (Argentina)
  • 4 Servicio de Neurocirugía, Hospital Italiano de Buenos Aires, Argentina. , (Argentina)
Type
Published Article
Journal
Medicina
Publication Date
Jan 01, 2018
Volume
78
Issue
3
Pages
194–196
Identifiers
PMID: 29940546
Source
Medline
Keywords
Language
Spanish
License
Unknown

Abstract

IgG4-related disease (IgG4-RD) is a recently described systemic entity of unknown origin. It predominantly affects older men and has distinctive histopathologic features as storiform fibrosis, obliterative phlebitis, dense lymphoplasmacytic infiltrate with immunostaining for IgG4, and it may be associated with elevated serum levels of IgG4. Although any organ can be affected, pituitary gland is rarely involved. We describe the case of a 36-year-old man who presented with headaches, impaired vision, panhypopituitarism with diabetes insipidus and an infiltrative lesion mainly of infundibulum and pituitary. We arrived at diagnosis of IgG4-RD by pituitary biopsy. A successful response to treatment with immunosuppressive doses of corticosteroids was achieved.

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