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.
Autoimmune Hypophysitis

Idiopathic granulomatous hypophysitis (IGH), a rare disease, requires differentiation from more common mass lesions of the sella such as pituitary adenoma, craniopharyngioma, Rathke's cleft cyst, or pituitary tuberculoma. IGH usually presents with an insidious onset of visual defects and headaches. On the other hand, rapid onset of neurologic and visual symptoms in an IGH patient is exceptionally rare. Here, we present a biopsy-proven case of IGH with rapid onset and satisfactory outcome after high dose steroid treatment.
Adult ; Autoimmune Hypophysitis ; Craniopharyngioma ; Endoscopy ; Glucocorticoids ; Hand ; Headache ; Humans ; Pituitary Gland ; Pituitary Neoplasms ; Rare Diseases ; Sella Turcica ; Tuberculoma

Granulomatous hypophysitis is an extremely rare condition, with no established definitive treatment. An elderly Asian woman was diagnosed to have recurrent granulomatous hypophysitis 5 years after transsphenoidal surgery. No other intervention was done post-operatively. Since another surgery was not advisable due to the high probability of recurrence, she was started on a trial of oral glucocorticoids. After 3 months of steroid therapy, complete resolution of symptoms and sellar mass were achieved.
Autoimmune Hypophysitis ; Glucocorticoids

Primary hypoparathyroidism is caused by a group of heterogeneous conditions in which hypocalcemia and hyperphosphatemia occur as a result of deficient parathyroid hormone (PTH) secretion. The most common cause is surgical excision and damage to the parathyroid gland(s). Nonetheless, autoimmune endocrine disorder of primary hypothyroidism has been well-described in polyglandular autoimmune syndromes (PAS).1 Its association with pituitary lesion may be autoimmune lymphocytic hypophysitis as the cause for pituitary disorder. In this report, we encountered a patient with primary hypoparathyroidism who had a non-functioning pituitary tumour. It was confirmed as pituitary adenoma rather than lymphocytic hypophysitis from the histopathological examination. To our knowledge, this is the first reported case of non-functioning pituitary macroadenoma and primary hypoparathyroidism.
Hypopituitarism ; Autoimmune Hypophysitis ; DiGeorge Syndrome