Clinical course of pituitary function and image in IgG4-related hypophysitis

Abstract

A 76-year-old man had a hypopituitarism including adrenal insufficiency, hypogonadism and hypothyroidism. Based on various findings including the swelling of the pituitary gland, increase of serum IgG4 level and abundant IgG4-positive plasma cell infiltration in immunostaining of the pituitary gland, we diagnosed this subject as IgG4-related hypophysitis. In general, a high-dose glucocorticoid treatment is effective for IgG4-related disease. His clinical symptom, laboratory data and adrenal insufficiency were almost improved without any therapy. The serum IgG4 level was decreased and pituitary size was normalized with hydrocortisone as physiological replacement. This case report provides the possibility that IgG4 level is decreased spontaneously or with physiological dose of glucocorticoid therapy.Learning points:We performed the pituitary gland biopsy and histochemical examination glucocorticoid therapy in a subject with IgG4-related hypophysitis.This case report provides the possibility that IgG4 level is decreased spontaneously or with a physiological dose of glucocorticoid therapy. We reported the clinical course of IgG4-related hypophysitis without a high-dose glucocorticoid treatment, although there were a few reports about the retrospective examination.Although the patient had still higher IgG4 level compared to normal range, his clinical symptom disappeared and his laboratory data were improved.We should keep in mind the possibility of IgG4-related hypophysitis when we examine one of the uncertain causes of a hypopituitarism including adrenal insufficiency, hypogonadism and hypothyroidism.

A case series of atypical features of patients with biopsy-proven isolated IgG4-related hypophysitis and normal serum IgG4 levels

“…The findings of our 3 cases of isolated IgG4-related hypophysitis with no other systemic lesions is somewhat unusual; however, these observations are not totally unique as there have been previous reports, albeit in small numbers, in Japanese patients [6,13,30,36]. In all of these reported cases, the patients were diagnosed by a pituitary biopsy without any other suspected lesions.…”

“…While there is no consensus for the optimum dose, type, and duration of glucocorticoid therapy for this disease, prednisolone at a dose of 0.6 mg/kg/day has been used and continued for 1 to 2 months, with the objective of tapering the dose at a rate of 5 mg/week. In fact, recent studies have reported using low physiological hydrocortisone doses in effectively decreasing serum IgG4 levels and pituitary size [30,31]. The physiological hydrocortisone doses in these reports are comparable to the doses used in our patients, with the exception of the pharmacological doses that we intentionally used to preemptively cover for potential adrenal crisis on the day of and the day after surgery.…”

“…Glucocorticoids are the first-line treatment for IgG4-related hypophysitis because the disease responds well to this therapy, with concomitant reductions in serum IgG4 levels [10,13,30,31] that can range from days to 3 months [10,11,28,32]. While there is no consensus for the optimum dose, type, and duration of glucocorticoid therapy for this disease, prednisolone at a dose of 0.6 mg/kg/day has been used and continued for 1 to 2 months, with the objective of tapering the dose at a rate of 5 mg/week.…”

A case report of IgG4-related disease: an insidious path to the diagnosis through kidney, heart and brain

“…Goto et al reported a case of IgG4-related ophthalmic disease misdiagnosed as intraocular tumor [10]. IgG4-RD could also occur as hypophysitis.…”

“…IgG4-RD presents features appearing heterogeneously associated and it is not known how exactly the different organs are implicated [1,6,10,11,[20][21][22].…”