68 22 Proceeding Clinical features and diagnosis of lymphocytic infundibulo-hypophysitis IgG4 12 / 7/ IgG4 3/7 IgG

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1 68 22 Proceeding Clinical features and diagnosis of lymphocytic infundibulo-hypophysitis IgG4 12 /7/ IgG4 3/7 IgG IgG4 IgG4 IgG4 IgG4 MR 1 Tolosa-Hunt IgG4 2 IgG MRI 12 /7/ IgG4 5 cases cases MRI IgG4 7 3 IgG IgG IgG4 Vol. 88 Suppl. Aug 2012

2 22 Proceeding 69 case 2 case 3 case 4 case 5 case 6 case 7 case 9 case 10 case 11 case 12 1 MRI T1 30mg 15mg 10mg 2case 4 Vol. 88 Suppl. Aug 2012

3 70 22 Proceeding IgG4 IgG IgG IgG4 4 IgG4 5 IgG4 Tolosa-Hunt 2 1n=12 Case 1 50 F* 2 52 F 3 70 M ** 23 ** 10 RPF 10 IgG4 mg/dl Pred 40mg 47, HC 5mg Pred 20mg 4 72 M* 181 Pred 60mg 5 78 F* ** 9 RPF 143 HC 15mg 6 26 M* M Sarcoidosis** 92 Pred 10mg 8 54 F* Tolosa-Hunt HC 15mg 9 60 M Pred 7.5mg M HC 20mg F 23 HC 15mg F HC 15mg * ** PRF Pred HC 2 Vol. 88 Suppl. Aug 2012

4 22 Proceeding 71 IgG4 IgG4 2 IgG4 2 Leporati 6 IgG4 IgG4 10 /HPF / MRI IgG4 140mg/dl IgG mg/kg/day 5 IgG4 IgG4 IgG4 ACTH IgG4 7 76kDa Rabphilin-3A IgG4 IgG4 IgG4 IgG , pp Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4- RD), Mod Rheumatol 2012; 22: ; 64(1): Shimatsu A, Oki Y, Fujisawa I, et al. Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: an emerging clinical entity. Endocr J 2009; 56(9): IgG4 Annual Review , pp Leporati P, Landek-Salgado MA, Lupi I, et al. IgG4- related hypophysitis: a new addition to the hypophysitis spectrum. J Clin Endocrinol Metab 2011; 96(7): , pp Vol. 88 Suppl. Aug 2012

5 72 22 Proceeding 6 Lymphocytic hypophysitis: LH IgG LH TSS TSS MRI IgG 4 IgG4 TSS LH IgG 6 4 IgG4 TSS LH TSS 1 Leporati P, Landek-Salgado MA, Lupi I, Chiovato L, Caturegli P. IgG4-related hypophysitis: a new addition to the hypophysitis spectrum. J Clin Endocrinol Metab Jul; 96(7): Epub 2011 May Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. A novel clinical entity, IgG4- related disease (IgG4RD): general concept and details. Mod Rheumatol Feb; 22(1): Epub 2011 Sep 1. 3 Shao-bo Su, Da-jian Zhang, Shu-yuan Yue and Jianning Zhang. Primary granulomatous hypophysitis: a case report and literature review. Endocr J. 2011; 58(6): Epub 2011 Apr 23. Vol. 88 Suppl. Aug 2012

6 22 Proceeding SLE, TTP MRI PRL PRL PRL PRL IgG4 PRL Vol. 88 Suppl. Aug 2012

7 74 22 Proceeding Vol. 88 Suppl. Aug 2012

8 22 Proceeding 75 IgG Table 1IgG IgG4 1 2 MRI 1 MRI Fig. 1 IgG mg/dL 2 924mg/dL mg/dL 1 IgG4 IgG MRI Table Table 1 Table 1. Case summary No. age/sex diagnosis pituitary function (chronic phase) 1 52/F IgG4-related hypophysitis hypopituitarism 2 72/M IgG4-related hypophysitis hypopituitarism 3 28/F adenohypophysitis during pregnancy normal 4 78/M adenohypophysitis hypopituitarism 5 51/F panhypophysitis normal 6 64/M panhypophysitis DI, hypopituitarism 7 84/M panhypophysitis DI 8 16/F hypophysitis due to Rathke s cleft cyst DI 9 66/M hypophysitis due to Rathke s cleft cyst DI, hypopituitarism 10 43/M hypophysitis due to Rathke s cleft cyst DI, hypopituitarism 11 64/M hypophysitis due to Rathke s cleft cyst DI 12 61/F hypophysitis due to Rathke s cleft cyst DI, hypopituitarism 13 21/F hypophysitis due to Rathke s cleft cyst DI 14 11/M hypophysitis due to Rathke s cleft cyst DI, hypopituitarism 15 44/F hypophysitis due to Rathke s cleft cyst DI, hypopituitarism DI: diabetes insipidus Vol. 88 Suppl. Aug 2012

9 76 22 Proceeding germ cell tumor germinoma 3 5 germ cell tumor germ cell tumor Fig. 1 MR imaging of the pituitary in case 1. T1-weighted gadolinium enhanced imaging shows a mass lesion in the pituitary stalk and gland. IgG4 germ cell tumor IgG4 1 IgG4 IgG4 Shimatsu 22 IgG4 review 11 IgG IgG4 IgG4 IgG4 2 1 IgG4 3 Caturegli 1 Shimatsu A, Oki Y, Fujisawa I, Sano T. Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: an emerging clinical entity. Endocrine journal 2009; 56: Caturegli P, Newschaffer C, Olivi A, Pomper MG, Burger PC, Rose NR. Autoimmune hypophysitis. Endocrine reviews 2005; 26: Fehn M, Bettendorf M, Ludecke DK, Sommer C, Saeger W. Lymphocytic hypophysitis masking a suprasellar germinoma in a 12-year-old girl a case report. Pituitary 1999; 1: Mikami-Terao Y, Akiyama M, Yanagisawa T, Takahashi-Fujigasaki J, Yokoi K, Fukuoka K, Sakuma M, Miyata I, Fujisawa K, Oi S, Eto Y. Lymphocytic hypophysitis with central diabetes insipidus and subsequent hypopituitarism masking a suprasellar germinoma in a 13-year-old girl. Child s nervous system 2006; 22: Endo T, Kumabe T, Ikeda H, Shirane R, Yoshimoto T. Neurohypophyseal germinoma histologically misidentified as granulomatous hypophysitis. Acta neurochirurgica 2002; 144: Vol. 88 Suppl. Aug 2012

10 22 Proceeding MRI The findings on Magnetic Resonance Imaging and Clinical Characteristics in 14 Patients with Hypophysitis MRI T mm 2 mm Mann-Whitney U P 0.05 CRH TRH LHRH GHRP2 Table 2 ACTH ACTH Table 1: Etiology of 14patients with hypophysitis (AN: adeno hypophysitis (N=6); INH: Infundibulo-neurohypophysitis (N=3); PH: panhypophysitis (N=5)) with or without hypertrophic dura. 14 AH 6 /3/3 INH 3 /1/2PH 5 /2/3AH INH PH BMI kg/m 2 MRI 8 / 3/5 5 /3/2 1 AH4 INH1 PH PH1 7 Table 1 Table 2: Pituitary hormone dysfunction in 14 patients with hypophysitis (AN: adenohypophysitis (N=6); INH: Infundibulo-neurohypophysitis (N=3); PH: panhypophysitis (N=5)) with or without hypertrophic dura. Vol. 88 Suppl. Aug 2012

11 78 22 Proceeding TIA Leung MRI MRI 2 MRI MRI 50 3 Caturegli 3 GHD ACTH ACTH GHD ACTH IgG Leung GK, Lopes MB, Thorner MO, Vance ML, Laws ER. Primary hypophysitis: a single-center experience in 16 cases. J Neurosurg 2004; 101: Nishioka H, Ito H, Haraoka J, Takahashi M, Shinmura F. Idiopathic hypertrophic cranial pachymeningitis of the cavernous sinus mimicking lymphocytic hypophysitis. Neurol Med Chir (Tokyo) 1998; 38: Caturegli P, Newschaffer C, Olivi A, Pomper MG, Burger PC, Rose NR. Autoimmune hypophysitis. Endocr Rev 2005; 26: Shimatsu A, Oki Y, Fujisawa I, Sano T. Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: an emerging clinical entity. Endocr J 2009; 56: ; 87: ; 85: Vol. 88 Suppl. Aug 2012

IgG4 2 IgG4 * ** Key Words : IgG4 related disease, IgG4 related pachymeningitis, IgG4 related leptomeningitis, IgG4 related hypophysitis, IgG4 related

IgG4 2 IgG4 * ** Key Words : IgG4 related disease, IgG4 related pachymeningitis, IgG4 related leptomeningitis, IgG4 related hypophysitis, IgG4 related 2 * ** Key Words : IgG4 related disease, IgG4 related pachymeningitis, IgG4 related leptomeningitis, IgG4 related hypophysitis, IgG4 related neuropathy 32 108 112 2015 IgG4 IgG4 related disease IgG4 RD

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