ImmunoglobulIn (Ig) G4-related systemic disease is a recently characterized disorder. The bestknown manifestation of this disease is pancreatitis [1], but other systemic involvements have also been described, including those in salivary glands [2], lung [3], liver, bile duct, gall bladder [4,5], kidney [6] and retroperitoneum [5,7]. Significantly, they all share similar features, i.e., a high serum level of IgG4 and an infiltration of IgG4-positive plasma cells as determined using histopathological specimens of the organs involved. To our knowledge, only six cases of IgG4-related hypophysitis have been reported in the literature (English language) thus far, and none of these reports has described the long-term clinical course as an IgG4-related systemic disease. In our current case Abstract. A 70-year old man with a 14 year history of Sjögren syndrome, interstitial pneumonia, and autoimmune hepatitis (AIH) was admitted to our hospital due to hyponatremia with a one month history of fatigue, thirst, and nausea. Laboratory tests on admission revealed that this patient had a central adrenal insufficiency. Pituitary magnetic resonance imaging (MRI) further showed swelling of the stalk and posterior lobe of his pituitary, suggesting infundibulo-hypophysitis. Based on his past history of autoimmune disease, his serum IgG4 levels were measured and found to be remarkably high (924 mg/ dL). Previous biopsy specimens from his liver, lung, and parotid gland were immunostained for IgG4, which revealed a marked infiltration of IgG4-positive plasma cells. As a result of our tests, we made a diagnosis of IgG4-related systemic disease. Interestingly, a subsequent MRI scan at three weeks after the patient commenced glucocorticoid replacement therapy for adrenal insufficiency showed that the swelling of his pituitary stalk was reduced. This finding suggested that IgG4-related hypophysitis may improve either as a result of a supplemental dose of glucocorticoid or possibly spontaneously. Although six cases of IgG4-related hypophysitis have been reported in the scientific literature published in English, our current case is the first in which IgG4-related hypophysitis likely occurred as a result of a long-term history of IgG4-related systemic disease. We report this case herein and review the relevant literature.Key words: IgG4, Hypophysitis, Adrenal insufficiency study, we describe a patient who was diagnosed with hypophysitis 14 years after the onset of hepatitis, interstitial pneumonia, and sialadenitis, which turned out pathologically to be an IgG4-related disease. Case ReportA 70-year-old man who had been followed up in our hospital for diabetes mellitus visited the emergency room and was admitted in March 2009 with severe hyponatremia (Na 115 mEq/L) involving a one month history of fatigue, thirst, and nausea. Fourteen years earlier, he had been diagnosed with Sjögren syndrome, lymphoid interstitial pneumonia, and AIH, and had been treated with 30 mg per day of prednisolone. This steroid regimen had been gradually reduced nine...
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