Balancing Disease Control and Metabolic Harm: A Case of IgG4-Related Hypophysitis
https://doi.org/10.15605/jafes.041.S1
- Author:
Asma’ Mohd Nazlee
1
;
Dorothy Maria Anthony Bernard
1
;
Siti Sanaa Wan Azman
1
;
Siew Hui Foo
1
Author Information
1. Hospital Selayang
- Publication Type:Journal Article
- MeSH:
Autoimmune Hypophysitis
- From:
Journal of the ASEAN Federation of Endocrine Societies
2026;41(S1):83-
- CountryPhilippines
- Language:English
-
Abstract:
Introduction:Immunoglobulin G4-related hypophysitis (IgG4-RH) is a
rare fibro-inflammatory disorder affecting the pituitary
gland. Glucocorticoids remain the first-line therapy, but
their use may be complicated in patients with significant
metabolic comorbidities. We report a case of suspected
IgG4-RH presenting with hyperosmolar hyperglycemic
state (HHS), highlighting the challenges of balancing
disease control against glucocorticoid metabolic toxicity
adverse effects.
Case:A 32-year-old female with obesity and newly diagnosed
diabetes mellitus was admitted with HHS. Prior to
admission, she reported weight fluctuations, episodic
headaches, progressive visual disturbance, and secondary
amenorrhea. Following resolution of HHS, persistent
polyuria of 10–16 L/day prompted further evaluation and led
to a diagnosis of arginine vasopressin deficiency. Anterior
pituitary hormonal work-up revealed hypogonadotropic
hypogonadism. Pituitary magnetic resonance imaging
demonstrated infundibular thickening measuring 0.5 cm,
with concomitant marked bilateral parotid enlargement.
Serum IgG4 was elevated at 2.26 g/L (0.63–2.01), raising
strong suspicion for IgG4-RH with systemic involvement.
Histopathological confirmation from the parotid gland
biopsy was consistent with sialadenosis.
She was commenced on sublingual desmopressin and
cyclical sex hormone replacement therapy. Given the
provisional diagnosis of IgG4-RH, oral prednisolone 40
mg daily was initiated as a reduced induction regimen.
However, treatment was poorly tolerated, with rapid
weight gain from 93 to 100 kg and worsening glycemic
control. Prednisolone was therefore tapered rapidly
to 10 mg daily. Repeat imaging demonstrated interval
improvement in infundibular thickening, but no functional
endocrine recovery was observed.
Conclusion:This case illustrates the therapeutic challenge of managing
IgG4-RH in the setting of pre-existing metabolic syndrome.
Although glucocorticoids are effective for induction,
their metabolic adverse effects may significantly restrict treatment tolerability. Early consideration of steroidsparing agents, such as rituximab or azathioprine, may
be important to achieve remission while minimizing
glucocorticoid-related adverse effects.
- Full text:2026080414071466036EP_A115.pdf
