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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Case of the Week

Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada

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April 13, 2023
  • Description
  • Legends
  • Histopathology
  • Diagnosis
  • Brain Teaser
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IgG4-Related Hypophysitis

Background:

  • IgG4-related hypophysitis is a pituitary manifestation of IgG4-related disease, a disease characterized by tumor-like involvement of multiple organs with IgG4-positive plasma cells.

Clinical Presentation:

  • The patient typically presents with hypopituitarism (diabetes insipidus and/or anterior pituitary hormone deficiencies).
  • The other main presenting symptoms are due to local mass effect, including vision changes and headaches.

Key Diagnostic Features:

  • MRI demonstrates loss of the normal posterior pituitary T1 bright spot, a sellar mass, and thickening and enhancement of the infundibulum.
  • Imaging is required to assess for systemic manifestations of IgG4-related disease outside the brain; this includes the orbits, paranasal sinuses, salivary glands, thyroid, lungs, pancreas, and kidneys.
  • Although serum IgG4 levels are often elevated, more definitive diagnosis requires histopathologic analysis of the pituitary or other affected organ, which demonstrates lymphoplasmacytic infiltration of predominantly IgG4-positive plasma cells (proposed cutoffs include IgG4+/IgG+ plasma ratio of >40%, however, this varies by organ).

Differential Diagnoses:

  • Lymphocytic hypophysitis: Seen in the pregnancy/early postpartum period
  • Infectious granulomatous hypophysitis, such as tuberculosis: Presence of systemic illness
  • Granulomatosis with polyangiitis: Associated findings in the sinuses (erosion of the nasal septum/turbinates) and lungs (nodules or consolidation with or without cavitation).
  • Neurosarcoidosis: Seen in the presence of other typical CNS findings (pachymeningeal or leptomeningeal involvement) and chest findings (hilar adenopathy)
  • Langerhans cell histiocytosis: Classically associated lytic calvarial lesions with beveled edges
  • Germinoma: May be multifocal and involve the pineal region
  • Lymphoma: Can be primary or secondary; when the pituitary is involved, there is typically an aggressive appearance with potential involvement of the skull base, cavernous sinus, and cranial nerves
  • Metastasis: Typically seen in adults with known primary tumor

Treatment:

  • IgG4-related hypophysitis typically demonstrates rapid response to corticosteroid therapy.
  • Treatment also entails managing pituitary dysfunction with hormone replacement.

Suggested Reading

  1. Decker L, Crawford A, Lorenzo G, et al. IgG4-related hypophysitis: case report and literature review. Cureus 2016;8:e907
  2. Hamilton B, Salzman K, Osborn A. Anatomic and pathologic spectrum of pituitary infundibulum lesions. AJR Am J Roentgenol 2007;188:W223-32
  3. Toyoda K, Oba H, Kutomi K, et al. MR imaging of IgG4-related disease in the head and neck and brain. AJNR Am J Neuroradiol 2012;33:2136-39

Current Issue

American Journal of Neuroradiology: 46 (7)
American Journal of Neuroradiology
Vol. 46, Issue 7
1 Jul 2025
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