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. 2022 Aug;164(8):2095-2103.
doi: 10.1007/s00701-022-05231-9. Epub 2022 May 7.

IgG4-related hypophysitis: a retrospective cohort study

R Bhargava  1 , Z Hussein  2   3 , N L Dorward  4 , J P Grieve  4 , Z Jaunmuktane  5 , H J Marcus  4 , I Proctor  5 , S E Baldeweg  2   3
Affiliations

IgG4-related hypophysitis: a retrospective cohort study

R Bhargava et al. Acta Neurochir (Wien). 2022 Aug.

Abstract

Purpose: IgG4-related hypophysitis (IgG4-RH) is a rare chronic inflammatory condition of the pituitary gland. This study reports the presentation, management and outcomes for patients with histologically proven IgG4-related hypophysitis.

Methods: A prospectively maintained electronic database was searched over a 14-year period from 1 January 2007 to 31 December 2020 at a single academic centre to identify all patients with a histological diagnosis of IgG4-RH. A retrospective case note review from electronic health records was conducted for each case to extract data on their presentation, management and outcomes.

Results: A total of 8 patients (5 male) with a median age of 51 years were identified. The most common presenting symptoms were headache (4/8; 50%), fatigue (3/8; 37.5%) and visual impairment (2/8; 25%). Three patients were initially treated with high-dose steroids aiming for reduction of the pituitary mass. However, ultimately all patients underwent transsphenoidal surgery. Post-operative changes included radiological reduction in pituitary mass in all patients that had imaging (7/7; 100%), improvement in vision (1/2; 50%), residual thick pituitary stalk (5/7; 71.4%), persistent anterior hypopituitarism (4/8; 50%) and panhypopopituitarism including diabetes insipidus (3/8; 37.5%).

Conclusions: IgG4-RH is an increasingly recognised entity presenting with a variety of symptoms and signs. Clinical presentation is similar to other forms of hypophysitis. It is therefore important to consider IgG4-RH as a differential and to have a low threshold for pituitary biopsy, the diagnostic gold standard. The diagnosis of IgG4-RH will guide decisions for additional workup for IgG4-related disease, multi-disciplinary team involvement and follow-up.

Keywords: Hypophysitis; Hypopituitarism; IgG4; Pituitary biopsy.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Post-contrast T1-weighted pituitary magnetic resonance (MR) images in one patient with IgG4-RH. A Pre-treatment sagittal section scan showing a pituitary adenoma 15 × 7 × 8 mm (height × width × length) with sellar extension (white arrow). Pituitary stalk is thick and bulges into infundibular recess, posterior bright spot of neurohypophysis is lost. B Post-treatment coronal section scan at 5 months after treatment with oral glucocorticoids over 4 weeks did not show any reduction in mass and visual loss persisted. C Post-operative sagittal section scan at 1 year following pituitary biopsy shows reduction in pituitary mass and residual thick pituitary stalk (white arrow)
Fig. 2
Fig. 2
Algorithm depicting management of IgG4-related hypophysitis (IgG4-RH). MDT multi-disciplinary team meeting
See this image and copyright information in PMC

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