Deterioration of pituitary function without relapse after steroid therapy for IgG4-related hypophysitis

Abstract

Summary IgG4-related hypophysitis is an autoimmune hypophysitis associated with IgG4-related disease. Swelling of the pituitary gland is responsive to steroid therapy, but the prognosis of pituitary function after the treatment remains unclear. The present case implies that transiently improved pituitary function can re-worsen during long-term follow-up in IgG4-related hypophysitis. A 71-year-old male patient with IgG4-related hypophysitis visited a nearby hospital with malaise, anorexia, and polyuria. Pituitary dysfunction was suspected, so he was referred to our hospital for further examination. Imaging studies and laboratory data showed swelling of the pituitary gland and panhypopituitarism, which dramatically improved following steroid therapy. There was no evidence of relapsing IgG4-related disease during prednisolone tapering. Pituitary function was examined after 4 years under treatment with low-dose prednisolone; surprisingly, anterior pituitary function had worsened again. Our case suggests a need for continuous monitoring of pituitary function after steroid therapy for IgG4-related hypophysitis. This report illustrates the natural course of pituitary function in IgG4-related hypophysitis and may be informative when considering the introduction of steroid therapy. Learning points Steroid therapy is an effective first-line therapy for pituitary dysfunction and pituitary swelling in IgG4-related hypophysitis. Pituitary function can worsen again during follow-up, despite transient improvement after steroid therapy in IgG4-related hypophysitis. Continuous monitoring of pituitary function is necessary for IgG4-related hypophysitis, regardless of disease activity.

A case of IgG4-related hypophysitis maintained remission of diabetes insipidus for over 3 months after completion of steroid treatment

“…Immunosuppressive drugs such as rituximab have been used in cases where steroids were ineffective or caused side effects ( 5 ). Tapering of steroids or immunosuppressive drugs for treatment of IgG4-RH requires careful consideration because of the possibility of reoccurrence of the disease or reenlargement of the pituitary ( 9 ).…”

Occlusion of the cavernosal portion of the internal carotid artery, secondary to IgG4 hypophysitis

“…IgG4-RH has been shown to relapse during steroid tapering, but in patients without relapse, the prognosis of pituitary function after treatment with this drug has not been clarified. 1 In a study published in 2017, it is evidenced that glucocorticoid therapy can improve anterior pituitary function, but does not have an effect on posterior pituitary function and does not improve DI. 21 Our patient has improved since the start of Rituximab.…”

“…Organs typically affected include the pancreas, lacrimal glands, salivary gland, thyroid, lungs, skin, lymph nodes, kidneys, aorta, and retroperitoneum; the pituitary gland can also be affected. 1,2 The incidence of IgG4-RD was estimated at 0.28 to 1.08/100,000 patients in Japan, 3 however a substantial underestimation of the true prevalence of the disease is possible. The increasing recognition of this entity and the development of worldwide accepted criteria for IgG4-RD will allow for more precise epidemiological studies.…”

Glucocorticoid impact therapy for recurrent IgG4-related disease with diabetes insipidus as the main manifestation: A case report and literature review

“…We used "hypophysitis," "IgG4-related hypophysitis," "diabetes insipidus" as keywords and searched on PubMed database. The review literature has been available for 115 cases of IgG4-RH before 2021, [1] so the search period was from January 1, 2021 to December 1, 2022, and 15 case reports were detected, [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] including 12 with complete clinical information and a total of 24 patients [2][3][4][5][6][7][9][10][11][12][13][14] (Table 1).…”