Anterior hypopituitarism secondary to biopsy-proven IgG4-related hypophysitis in a young man

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.

Medienart:

E-Artikel

Erscheinungsjahr:

2019

Erschienen:

2019

Enthalten in:

Zur Gesamtaufnahme - volume:2019

Enthalten in:

Endocrinology, diabetes & metabolism case reports - 2019(2019) vom: 03. Apr.

Sprache:

Englisch

Beteiligte Personen:

Joshi, H [VerfasserIn]
Hikmat, M [VerfasserIn]
Devadass, A P [VerfasserIn]
Oyibo, S O [VerfasserIn]
Sagi, S V [VerfasserIn]

Links:

Volltext

Themen:

2019
Adolescent/young adult
April
Asian - other
BMI
CT scan
Cortisol
Cortisol (9am)
Diabetes mellitus type 2
FSH
FT3
FT4
Glucocorticoids
Haemoglobin A1c
Headache
Hydrocortisone
Hypophysitis
Hypopituitarism
IGF1
IgG4–related systemic disease
Immunoglobulin G4
Immunohistochemistry
Insight into disease pathogenesis or mechanism of therapy
Journal Article
LH
Levothyroxine
MRI
Male
Metformin
Methylprednisolone
Pituitary
Pituitary function
Prolactin
Sixth nerve palsy
TSH
Testosterone
Thyroxine (T4)
Triiodothyronine (T3)
United Kingdom
Vitamin D
Vomiting
Weight
Weight loss

Anmerkungen:

Date Revised 27.02.2024

published: Print-Electronic

Citation Status Publisher

doi:

10.1530/EDM-18-0137

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM295652373