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BMC Endocrine Disorders

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Open Access 01-12-2015 | Case report

Clinical course of IgG4-related hypophysitis presenting with focal seizure and relapsing lymphocytic hypophysitis

Authors: Kanchana Ngaosuwan, Therdkiat Trongwongsa, Shanop Shuangshoti

Published in: BMC Endocrine Disorders | Issue 1/2015

Abstract

Background

This is the first case report of focal seizure as a manifestation of Immunoglobulin G4 (IgG4)-related hypophysitis. IgG4-related hypophysitis is a novel category of hypophysitis. The clinical presentations, imaging studies and initial pathology studies can mimic lymphocytic hypophysitis. Here we report additional clinical clues in differentiating these two conditions.

Case presentation

A 43-year-old Thai male presented with focal seizure, headache, and anterior pituitary hypofunction. His MRI study showed typical hypophysitis lesion with abnormal cerebral parenchymal signal intensity at right frontal lobe. The pituitary biopsied was obtained and the patient was initially diagnosed with lymphocytic hypophysitis. Following initial low-dose steroid therapy, his seizure and headache resolved but his anterior pituitary hormones remained deficient. However, during steroid tapering, he developed new onset acute visual loss. Upon rigorous pathologic review, his diagnosis of IgG4-related hypophysitis with suspected CNS involvement was established. He was subsequently treated with high-dose steroid and rapidly regained his sight.

Conclusion

This case report highlights the important distinguishing features of IgG4-related hypophysitis from lymphocytic hypophysitis. These include the relapsing clinical course of hypophysitis after steroid decrement and concomitant pachymeningitis particularly in middle-aged to elderly Asian male who presented with hypophysitis. With appropriate dosage of steroids, medical treatment is usually sufficient to control the disease and surgical interventions are usually not required.

Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38(10):982–4.CrossRefPubMed

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. 2011;96(7):1971–80.PubMedCentralCrossRefPubMed

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(9):1033–41.CrossRefPubMed

Nishioka H, Shibuya M, Haraoka J. Immunohistochemical study for IgG4-positive plasmacytes in pituitary inflammatory lesions. Endocr Pathol. 2010;21(4):236–41.CrossRefPubMed

Bando H, Iguchi G, Fukuoka H, Taniguchi M, Kawano S, Saitoh M, et al. A diagnostic pitfall in IgG4-related hypophysitis: infiltration of IgG4-positive cells in the pituitary of granulomatosis with polyangiitis. Pituitary. 2015.

Ohkubo Y, Sekido T, Takeshige K, Ishi H, Takei M, Nishio S, et al. Occurrence of IgG4-related hypophysitis lacking IgG4-bearing plasma cell infiltration during steroid therapy. Intern Med. 2014;53(7):753–7.CrossRefPubMed

Caturegli P, Newschaffer C, Olivi A, Pomper MG, Burger PC, Rose NR. Autoimmune hypophysitis. Endocr Rev. 2005;26(5):599–614.CrossRefPubMed

Rivera JA. Lymphocytic hypophysitis: disease spectrum and approach to diagnosis and therapy. Pituitary. 2006;9(1):35–45.CrossRefPubMed

van der Vliet HJ, Perenboom RM. Multiple pseudotumors in IgG4-associated multifocal systemic fibrosis. Ann Intern Med. 2004;141(11):896–7.CrossRefPubMed

10.

Tanabe T, Tsushima K, Yasuo M, Urushihata K, Hanaoka M, Koizumi T, et al. IgG4-associated multifocal systemic fibrosis complicating sclerosing sialadenitis, hypophysitis, and retroperitoneal fibrosis, but lacking pancreatic involvement. Intern Med. 2006;45(21):1243–7.CrossRefPubMed

11.

Taniguchi T, Hamasaki A, Okamoto M. A case of suspected lymphocytic hypophysitis and organizing pneumonia during maintenance therapy for autoimmune pancreatitis associated with autoimmune thrombocytopenia. Endocr J. 2006;53(4):563–6.CrossRefPubMed

12.

Yamamoto M, Takahashi H, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, et al. A case of Mikulicz’s disease (IgG4-related plasmacytic disease) complicated by autoimmune hypophysitis. Scand J Rheumatol. 2006;35(5):410–1.CrossRefPubMed

13.

Ralli S, Lin J, Farrell J. Autoimmune pancreatitis. N Engl J Med. 2007;356(15):1586. author reply 1587.CrossRefPubMed

14.

Wong S, Lam WY, Wong WK, Lee KC. Hypophysitis presented as inflammatory pseudotumor in immunoglobulin G4-related systemic disease. Hum Pathol. 2007;38(11):1720–3.CrossRefPubMed

15.

Isaka Y, Yoshioka K, Nishio M, Yamagami K, Konishi Y, Inoue T, et al. A case of IgG4-related multifocal fibrosclerosis complicated by central diabetes insipidus. Endocr J. 2008;55(4):723–8.CrossRefPubMed

16.

Tsuboi H, Inokuma S, Setoguchi K, Shuji S, Hagino N, Tanaka Y, et al. Inflammatory pseudotumors in multiple organs associated with elevated serum IgG4 level: recovery by only a small replacement dose of steroid. Intern Med. 2008;47(12):1139–42.CrossRefPubMed

17.

Osawa S, Ogawa Y, Watanabe M, Tominaga T. Hypophysitis presenting with atypical rapid deterioration: with special reference to immunoglobulin G4-related disease-case report. Neurol Med Chir (Tokyo). 2009;49(12):622–5.CrossRef

18.

Haraguchi A, Era A, Yasui J, Ando T, Ueki I, Horie I, et al. Putative IgG4-related pituitary disease with hypopituitarism and/or diabetes insipidus accompanied with elevated serum levels of IgG4. Endocr J. 2010;57(8):719–25.CrossRefPubMed

19.

Hori M, Makita N, Andoh T, Takiyama H, Yajima Y, Sakatani T, et al. Long-term clinical course of IgG4-related systemic disease accompanied by hypophysitis. Endocr J. 2010;57(6):485–92.CrossRefPubMed

20.

Patel SM, Szostek JH. IgG4-related systemic disease in a Native American man. Intern Med. 2011;50(8):931–4.CrossRefPubMed

21.

Nishina N, Kaneko Y, Kuwana M, Hanaoka H, Kameda H, Mikami S, et al. IgG4-related disease without overexpression of IgG4: pathogenesis implications. Case Rep Rheumatol. 2012. doi:10.1155/2012/754935.PubMedCentralPubMed

22.

Chiba K, Kamisawa T, Tabata T, Hara S, Kuruma S, Fujiwara T, et al. Clinical features of 10 patients with IgG4-related retroperitoneal fibrosis. Intern Med. 2013;52(14):1545–51.CrossRefPubMed

23.

Hattori Y, Tahara S, Ishii Y, Kitamura T, Inomoto C, Osamura RY, et al. A case of IgG4-related hypophysitis without pituitary insufficiency. J Clin Endocrinol Metab. 2013;98(5):1808–11.CrossRefPubMed

24.

Hsing MT, Hsu HT, Cheng CY, Chen CM. IgG4-related hypophysitis presenting as a pituitary adenoma with systemic disease. Asian J Surg. 2013;36(2):93–7.CrossRefPubMed

25.

Kanoke A, Ogawa Y, Watanabe M, Kumabe T, Tominaga T. Autoimmune hypophysitis presenting with intracranial multi-organ involvement: three case reports and review of the literature. BMC Res Notes. 2013;6:560.PubMedCentralCrossRefPubMed

26.

Bando H, Iguchi G, Fukuoka H, Taniguchi M, Yamamoto M, Matsumoto R, et al. The prevalence of IgG4-related hypophysitis in 170 consecutive patients with hypopituitarism and/or central diabetes insipidus and review of the literature. Eur J Endocrinol. 2014;170(2):161–72.CrossRefPubMed

27.

Caputo C, Bazargan A, McKelvie PA, Sutherland T, Su CS, Inder WJ. Hypophysitis due to IgG4-related disease responding to treatment with azathioprine: an alternative to corticosteroid therapy. Pituitary. 2014;17(3):251–6.CrossRefPubMed

28.

Iseda I, Hida K, Tone A, Tenta M, Shibata Y, Matsuo K, et al. Prednisolone markedly reduced serum IgG4 levels along with the improvement of pituitary mass and anterior pituitary function in a patient with IgG4-related infundibulo-hypophysitis. Endocr J. 2014;61(2):195–203.CrossRefPubMed

29.

Khong P, Enno A, Darwish B. Lymphoplasmacytic hypophysitis associated with immunoglobulin G4. J Clin Neurosci. 2014;21(2):342–4.CrossRefPubMed

30.

Sosa GA, Bell S, Christiansen SB, Pietrani M, Glerean M, Loto M. et al. Histologically confirmed isolated IgG4-related hypophysitis: two case reports in young women. Endocrinol Diabetes Metab Case Rep 2014; doi:10.1530/EDM-14-0062.

31.

Harano Y, Honda K, Akiyama Y, Kotajima L, Arioka H. A case of IgG4-related hypophysitis presented with hypopituitarism and diabetes insipidus. Clin Med Insights Case Rep. 2015;8:23–6.PubMedCentralCrossRefPubMed

32.

Joshi D, Jager R, Hurel S, Pereira SP, Johnson GJ, Chapman M, et al. Cerebral involvement in IgG4-related disease. Clin Med. 2015;15(2):130–4.CrossRefPubMed

33.

Nakasone Y, Oguchi K, Sato Y, Okubo Y, Yamauchi K, Aizawa T. Rapid conversion of autoimmune hypophysitis to an empty Sella with immediate lowering of the serum IgG4 level. Case report. Neuro Endocrinol Lett. 2015;36:112–4.PubMed

34.

Tauziede-Espariat A, Polivka M, Bouazza S, Decq P, Robert G, Laloi-Michelin M, et al. The prevalence of IgG4-positive plasma cells in hypophysitis: a possible relationship to IgG4-related disease. Clin Neuropathol. 2015;34(4):181–92.CrossRefPubMed

35.

Regev K, Nussbaum T, Cagnano E, Giladi N, Karni A. Central nervous system manifestation of IgG4-related disease. JAMA Neurol. 2014;71(6):767–70.CrossRefPubMed

36.

Li LF, Tse PY, Tsang FC, Lo RC, Lui WM, Leung GK. IgG4-related hypertrophic pachymeningitis at the falx cerebrii with brain parenchymal invasion: a case report. World Neurosurg. 2015. doi:10.1016/j.wneu.2015.03.035.

37.

Brooks BS, el Gammal T, Allison JD, Hoffman WH. Frequency and variation of the posterior pituitary bright signal on MR images. AJR Am J Roentgenol. 1989;153(5):1033–8.CrossRefPubMed

38.

Cote M, Salzman KL, Sorour M, Couldwell WT. Normal dimensions of the posterior pituitary bright spot on magnetic resonance imaging. J Neurosurg. 2014;120(2):357–62.CrossRefPubMed

Title: Clinical course of IgG4-related hypophysitis presenting with focal seizure and relapsing lymphocytic hypophysitis
Authors: Kanchana Ngaosuwan
Therdkiat Trongwongsa
Shanop Shuangshoti
Publication date: 01-12-2015
Publisher: BioMed Central
Published in: BMC Endocrine Disorders / Issue 1/2015
Electronic ISSN: 1472-6823
DOI: https://doi.org/10.1186/s12902-015-0062-x

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Clinical course of IgG4-related hypophysitis presenting with focal seizure and relapsing lymphocytic hypophysitis

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Conclusion

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