Session: SUN 163-194-Pituitary Disorders & Case Reports
Poster Board SUN-163
To detect IgG4-related hypophysitis, we screened 44 patients, those with pituitary tumors had been excluded, who presented with hypopituitarism (PH)/ central diabetes insipidus (DI). IgG4-related hypophysitis was diagnosed according to the following criteria; (1) serum IgG4 concentration³a135mg/dl, and/or (2) co-existing other organ involvement observed in IgG4-related disease and/or (3) infiltration of a substantial number of IgG4-positive plasma cells in the obtained tissues.
Totally, 7 cases (3 males and 4 females) were diagnosed as IgG4-related hypophysitis. The mean age at the time of onset of hypophysitis was 61.8±8.8 years. The mean serum IgG4 concentration was 191.1±78.3 mg/dL. 4, 2, and 1 cases showed PH+DI, DI, PH, respectively. These pituitary dysfunctions did not improve after corticosteroid administration. 6 cases showed swelling of pituitary and/or pituitary stalk, and 1 case showed empty sella. 4 cases demonstrated an involvement of other organs including Mikulicz's disease, autoimmune pancreatitis, interstitial pneumonitis, orbital psuedotumor, and hypertrophic pachymeningitis. 2 cases developed hypophysitis during the tapering of glucocorticoid therapy for the other organ lesions.
We performed a histopathological analysis of the pituitary in 3 cases and of the other involved organs in 3 cases and confirmed the infiltrate of IgG4-positive plasma cells in these lesions. Intriguingly, we found a unique ‘storiform fibrosis’ in the HE staining of the pituitary tissue, which has been reported in IgG4-related pancreatitis (e.g.(3)) but not in hypophysitis previously in all 3 cases.
In conclusion, IgG4-related hypophysitis manifests various organ involvement and clinical course. The ‘storiform fibrosis’ in the pituitary pathology may be a useful marker for the diagnosis.
Nothing to Disclose: HB, GI, HF, MT, MY, KS, HN, MT, EK, YT
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