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Diagnosis and Treatment > Medication

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H Joshi Department of Endocrinology, Peterborough City Hospital, Peterborough, UK

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M Hikmat Department of Endocrinology, Peterborough City Hospital, Peterborough, UK

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A P Devadass Department of Histopathology, Addenbrookes Hospital, Cambridge, UK

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S O Oyibo Department of Endocrinology, Peterborough City Hospital, Peterborough, UK

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S V Sagi Department of Endocrinology, Peterborough City Hospital, Peterborough, UK

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Summary

IgG4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory condition which can affect various organs including the pituitary gland. The true annual incidence of this condition remains widely unknown. In addition, it is unclear whether IgG4 antibodies are causative or the end result of a trigger. With no specific biomarkers available, the diagnosis of IgG4-related hypophysitis remains a challenge. Additionally, there is a wide differential diagnosis. We report a case of biopsy-proven IgG4-related hypophysitis in a young man with type 2 diabetes mellitus.

Learning points:

  • IgG4-related hypophysitis is part of a spectrum of IgG4-related diseases.

  • Clinical manifestations result from anterior pituitary hormone deficiencies with or without diabetes insipidus, which can be temporary or permanent.

  • A combination of clinical, radiological, serological and histological evidence with careful interpretation is required to make the diagnosis.

  • Tissue biopsy remains the gold standard investigation.

  • Disease monitoring and long-term management of this condition is a challenge as relapses occur frequently.

Taxonomy:
Clinical Overview, Gland/Organ, Pituitary, Topic, Pituitary, Hormone, Cortisol, FSH, IGF1, LH, Prolactin, Testosterone, Thyroxine (T4), Triiodothyronine (T3), TSH, Condition/ Syndrome, Diabetes mellitus type 2, Hypophysitis, Hypopituitarism, IgG4–related systemic disease, Patient Demographics, Age, Adolescent/young adult, Gender, Male, Ethnicity, Asian - other, Country of Treatment, United Kingdom, Diagnosis and Treatment, Signs and Symptoms, Diabetes mellitus type 2, Headache, Hypopituitarism, Sixth nerve palsy, Vomiting, Weight loss, Investigation, BMI, Cortisol (9am), CT scan, FSH, FT3, FT4, Haemoglobin A1c, IGF1, Immunohistochemistry, Immunoglobulin G4, LH, MRI, Pituitary function, Prolactin, Testosterone, TSH, Weight, Medication, Glucocorticoids, Hydrocortisone, Levothyroxine, Metformin, Methylprednisolone, Testosterone, Vitamin D, Publication Details, Case Report Type, Insight into disease pathogenesis or mechanism of therapy
DOI:
https://doi.org/10.1530/EDM-18-0137
Volume/Issue:
Volume 2019: Issue 1
Open access