IgG4-related hypophysitis in patients with autoimmune pancreatitis
IgG4-related disease involves various organs including the pituitary and pancreas. The prevalence of IgG4-related hypophysitis is relatively rare compared with IgG4-related pancreatitis (autoimmune pancreatitis). Although several cases demonstrating both autoimmune pancreatitis and hypophysitis have been reported, the prevalence of IgG4-related hypophysitis in patients with autoimmune pancreatitis remains unknown. This study aimed at screening for IgG4-related hypophysitis to accurately determine its prevalence in patients with autoimmune pancreatitis.
In this cohort study, we screened IgG4-related hypophysitis via pituitary magnetic resonance imaging (MRI) and endocrinological examination in 27 patients who were undergoing follow-up for autoimmune pancreatitis at Kobe University Hospital between 2014 and 2018.
Among 27 patients with autoimmune pancreatitis, 5 patients exhibited morphological abnormalities in the pituitary (18.5%). Among them, one patient (3.7%) met the criteria for hypophysitis with an enlarged pituitary and stalk concomitant with hypopituitarism. After glucocorticoid treatment, the enlarged pituitary shrank and became empty sella during the clinical course. Four patients (14.8%) revealed empty sella without obvious pituitary dysfunction. Four of 5 patients with morphological pituitary abnormalities showed multiple organ involvement in addition to pancreatic and pituitary involvement. Accordingly, multiple organ involvement was more prevalent in patients with morphological pituitary abnormalities (80%) compared to those without (48%).
Although a large-scale study is necessary to validate these results, these data suggest that the prevalence of hypophysitis in patients with autoimmune pancreatitis may be underestimated. Based on our findings, we recommend screening for hypophysitis, especially in patients with multiple organ involvement.
KeywordsHypophysitis IgG4-related hypophysitis Autoimmune pancreatitis IgG4-related pancreatitis
We thank K. Imura and C. Ogata for their excellent assistance.
KK, HB, and GI performed analysis and interpretation of data and wrote the draft of the article. HS, AM, AS, TK, YS, and YK contributed to the preparation of the patient cohort. HF, HN, KY, RM, KS, and WO contributed to the assembly of data. YT contributed to the study supervision and critical revision of the article for important intellectual content.
This work was supported by Japan Society for the Promotion of Science (JSPS) (KAKENHI, Grant numbers 23659477 and 26670459 [Y Takahashi], 23591354, 15K09431, and 18K08514 [G Iguchi], and 17K16165 [H Bando]), the Foundation for Growth Science (G Iguchi), the Japan Agency for Medical Research and Development (AMED) (17bm0804012h0001) (Y Takahashi), the Uehara Memorial Foundation (Y Takahashi), and the Naito Foundation (Y Takahashi).
Compliance with ethical standards
Conflict of interest
The authors declare no conflict of interest associated with this research.
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