IgG4-related hypophysitis in adolescence
-
Ved Bhushan Arya
,
Sarra Abu El-Gasim
Abstract
Objectives
IgG4-related hypophysitis is a novel clinical disease entity, which is typically seen in the sixth decade of life and is typically complicated by hypopituitarism. We describe an adolescent female with IgG4-related hypophysitis with normal pituitary function and summarize the relevant literature.
Case presentation
A 11.8-year-old girl presented with headache and left VI cranial nerve palsy. MRI brain identified an enlarged pituitary gland. Endocrine investigations revealed normal pituitary function. She underwent a transsphenoidal biopsy of the pituitary gland, and histological examination confirmed the diagnosis of IgG4-related hypophysitis. Serum IgG4 concentrations were normal and no evidence of other organ involvement was found. Although the patient tested strongly positive for TB on an interferon gamma release assay, pituitary biopsy was negative for granuloma formation and acid-fast bacilli (Ziehl-Neelson staining). IgG4-related hypophysitis was treated with oral prednisolone and mycophenolate-mofetil with a good response.
Conclusions
We describe to the best of our knowledge, the youngest patient in the published literature with IgG4-related hypophysitis presenting without pituitary insufficiency. A literature review identified only five cases of IgG4-related hypophysitis in adolescence. Serum IgG4 concentrations were normal in all, except one of the adolescent patients reported so far, and appear unhelpful in diagnosis in this age group.
Acknowledgments
Nil.
Research funding: Nil.
Author contributions: All authors accept responsibility for the entire content of this submitted manuscript and approve submission. VBA conceptualised the idea of manuscript, collected the data and wrote the manuscript. SAE collected the data and reviewed the manuscript. BZ, DD’C, TH and SJBA were involved in patient management and critically reviewed the manuscript. SD, RK and CRB conceptualised the idea of manuscript and critically reviewed the manuscript. ZR and AK were involved in histological examination of the pituitary biopsy, prepared the figures for the manuscript and critically reviewed the manuscript. VBA is the guarantor of this work.
Competing interests: The authors have no conflicts of interest to declare.
Informed consent: Written informed consent was obtained from the parents of the patient for publication of this case report and any accompanying images.
References
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- Frontmatter
- Review Article
- Inborn errors of immunity and metabolic disorders: current understanding, diagnosis, and treatment approaches
- Original Articles
- Evaluation of hydration status of children with obesity—a pilot study
- Relationship between insulin-like growth factor-1, insulin resistance and metabolic profile with pre-obesity and obesity in children
- Evaluation of Hemoglobin A1c before and after initiation of continuous glucose monitoring in children with type 1 diabetes mellitus
- Karyotype is associated with timing of ovarian failure in women with Turner syndrome
- The role of makorin ring finger protein-3, kisspeptin, and neurokinin B in the physiology of minipuberty
- Brain MRIs may be of low value in most children diagnosed with isolated growth hormone deficiency
- Significant improvement in bone mineral density in pediatric celiac disease: even at six months with gluten-free diet
- Population-based waist circumference reference values in Japanese children (0–6 years): comparisons with Dutch, Swedish and Turkish preschool children
- Extended growth charts for Indian children
- Rib fractures in infancy, case-series and register case-control study from Sweden
- Gonadotropin releasing hormone analogue therapy in girls with idiopathic precocious puberty/early-fast puberty: dynamics in adiposity indices, eating habits and quality of life
- Case Reports
- Transient benign hyperphosphatasemia due to COVID-19: the first case report
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- Graves’ disease in a five-month-old boy with an unusual treatment course
- Oral sodium phenylbutyrate for hyperammonemia associated with congenital portosystemic shunt: a case report
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