Rationale: Immunoglobulin G4 (IgG4)-related hypophysitis is a type of IgG4-related disease (IgG4-RD), which is characterized by plasma cells infiltration in the pituitary causing functional changes and (or) space-occupying effect in the pituitary. IgG4-related hypophysitis is sensitive to hormone therapy in most patients, but recurrence is very likely.
Patient concerns: Here, we report a 57-year-old male patient with bilateral eye redness as the initial presentation. He later presented with pituitary hypofunction that involved multiple organs, including eyes, lacrimal gland, pituitary, lung, gall bladder, and intestine. There was an elevation of C-reactive protein and blood sedimentation, but the IgG and IgG4 levels of the serum and the cerebrospinal fluid did not increase obviously following irregular glucocorticoid therapy. Magnetic resonance imaging revealed enlarged pituitary and obviously thickened pituitary stalk. IgG4-related hypophysitis was confirmed by biopsy of the pituitary.
Diagnoses: The patient was diagnosis of IgG4-related hypophysitis with ophthalmopathy by pathological and molecular tests.
Interventions: The patient responded to methylprednisolone pulse therapy but relapsed during the maintenance therapy using small-dose hormones combined with azathioprine. Methylprednisolone pulse therapy was given for 3 days followed by rituximab injection for 4 weeks.
Outcomes: After use methylprednisolone pulse therapy with rituximab the patient achieved complete remission.
Lessons: Rituximab achieved good effect for recurrent IgG4-related hypophysitis after glucocorticoid therapy combined with immunosuppressant in this case. Moreover, comparative analysis was carried out with other reported cases of IgG4-related hypophysitis in terms of clinical features, treatment, and follow-up.