Autoimmune hypophysitis associated with Crohn’s disease: a rare case presentation

Autoimmune hypophysitis, a rare inflammatory condition of the pituitary gland, is exceptionally uncommon when coexisting with Crohn’s disease. This report illustrates the diagnostic challenge posed by such a rare association, revealed through an unexpected symptom - seizure - highlighting the need for increased clinical vigilance and providing further evidence of shared dysimmune mechanisms underlying this uncommon condition. We report the case of a 16-year-old female with active Crohn’s disease, treated with azathioprine for two years, who presented with an acute, afebrile seizure. Initial non-contrast CT scan of the brain was unremarkable. However, during hospitalization, she experienced a second seizure, this time associated with severe hypoglycemia, hypotension, and new-onset headache. Neurological examination remained unremarkable, but hormonal analysis revealed hypopituitarism, specifically deficiencies in the corticotropic, somatotropic, and gonadotropic axes. A subsequent gadolinium-enhanced brain MRI showed hypertrophy of the anterior pituitary, diffuse homogeneous contrast enhancement, and thickening of the pituitary stalk. After excluding other potential diagnoses, autoimmune hypophysitis was confirmed. The patient was treated with hydrocortisone replacement therapy for corticotropic insufficiency and infliximab, optimizing her Crohn’s disease management. This therapeutic approach resulted in a favorable outcome. At 25 months follow-up, she remained stable, with only isolated corticotropic insufficiency requiring continued hydrocortisone. This report illustrates the diagnostic challenge of such a rare disease association, revealed by an unexpected symptom: seizure. It highlights the systemic nature of autoimmune diseases and their overlapping pathophysiology. Early recognition of hypophysitis in patients with Crohn’s disease presenting with atypical neurological or growth-related symptoms is crucial for prompt treatment, which can prevent life-threatening complications and significantly improve patient outcomes.