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DOI: 10.1055/s-0037-1604079
Lymphocytic Hypophysitis Successfully Treated with Stereotactic Radiosurgery: Case Report and Review of the Literature
Publication History
15 August 2016
28 December 2016
Publication Date:
25 July 2017 (online)
![](https://www.thieme-connect.de/media/jnlsa/201801/lookinside/thumbnails/10-1055-s-0037-1604079_161745cr-1.jpg)
Abstract
Lymphocytic hypophysitis (LH) is an autoimmune inflammatory infiltration of the pituitary gland, usually with a benign evolution. In rare circumstances the inflammatory process may extend beyond the pituitary and infiltrate the surrounding structures. We present a 42-year-old woman affected by an aggressive form of LH with extension to the cavernous sinus causing internal carotid artery occlusion and right sixth cranial nerve palsy. Prednisone therapy caused severe iatrogenic Cushing's syndrome, and the patient underwent transsphenoidal decompression. The histopathology report was consistent with LH. The patient was symptom free for a short period with reappearance of severe headache, diplopia, and hearing loss (middle ear inflammation) 3 months after surgery. Corticosteroids were reintroduced with the addition of azathioprine, but there was no regression of the pituitary mass. The patient was referred for stereotactic radiosurgery (SRS) using Gamma Knife (15 Gy to the margin). After 26 months, azathioprine was stopped, and the dose of prednisone was gradually tapered to 7.5 mg/day. Sellar magnetic resonance imaging showed regression of the pituitary mass. After follow-up for > 3 years after SRS, there was no clinical or radiologic evidence of the disease, but carotid arteries remained occluded. The patient developed secondary hypothyroidism and hypogonadism as consequences of SRS. An aggressive form of LH extending beyond the pituitary gland infiltrating surrounding structures is described. It was successfully treated with SRS after failure of transsphenoidal surgery and combined immunosuppressive therapy (prednisone, azathioprine). The review of the literature presents timely information concerning treatment with azathioprine and SRS of patients with an aggressive form of LH.
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References
- 1 Caturegli P, Newschaffer C, Olivi A, Pomper MG, Burger PC, Rose NR. Autoimmune hypophysitis. Endocr Rev 2005; 26 (05) 599-614
- 2 Laws ER, Vance Jr ML, Jane Jr JA. Hypophysitis. Pituitary 2006; 9 (04) 331-333
- 3 Caturegli P, Lupi I, Landek-Salgado M, Kimura H, Rose NR. Pituitary autoimmunity: 30 years later. Autoimmun Rev 2008; 7 (08) 631-637
- 4 Carmichael JD. Update on the diagnosis and management of hypophysitis. Curr Opin Endocrinol Diabetes Obes 2012; 19 (04) 314-321
- 5 Glezer A, Bronstein MD. Pituitary autoimmune disease: nuances in clinical presentation. Endocrine 2012; 42 (01) 74-79
- 6 Falorni A, Minarelli V, Bartoloni E, Alunno A, Gerli R. Diagnosis and classification of autoimmune hypophysitis. Autoimmun Rev 2014; 13 (4-5): 412-416
- 7 Takahashi Y. Autoimmune hypophysitis: new developments. Handb Clin Neurol 2014; 124: 417-422
- 8 Furuoka H. Hypophysitis. Endocrinol Metab Clin North Am 2015; 44 (01) 143-149
- 9 Caturegli P, Iwama S. From Japan with love: another tessera in the hypophysitis mosaic. J Clin Endocrinol Metab 2013; 98 (05) 1865-1868
- 10 Cheung CC, Ezzat S, Smyth HS, Asa SL. The spectrum and significance of primary hypophysitis. J Clin Endocrinol Metab 2001; 86 (03) 1048-1053
- 11 Faje A. Immunotherapy and hypophysitis: clinical presentation, treatment, and biologic insights. Pituitary 2016; 19 (01) 82-92
- 12 Shi J, Zhang JM, Wu Q, Chen G, Zhang H, Bo WL. Granulomatous hypophysitis: two case reports and literature review. J Zhejiang Univ Sci B 2009; 10 (07) 552-558
- 13 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 (03) 251-256
- 14 Gutenberg A, Hans V, Puchner MJ. , et al. Primary hypophysitis: clinical-pathological correlations. Eur J Endocrinol 2006; 155 (01) 101-107
- 15 Karaca Z, Kelestimur F. Pregnancy and other pituitary disorders (including GH deficiency). Best Pract Res Clin Endocrinol Metab 2011; 25 (06) 897-910
- 16 Sautner D, Saeger W, Lüdecke DK, Jansen V, Puchner MJ. Hypophysitis in surgical and autoptical specimens. Acta Neuropathol 1995; 90 (06) 637-644
- 17 Goudie RB, Pinkerton PH. Anterior hypophysitis and Hashimoto's disease in a young woman. J Pathol Bacteriol 1962; 83: 584-585
- 18 Pestell RG, Best JD, Alford FP. Lymphocytic hypophysitis. The clinical spectrum of the disorder and evidence for an autoimmune pathogenesis. Clin Endocrinol (Oxf) 1990; 33 (04) 457-466
- 19 Nishiki M, Murakami Y, Ozawa Y, Kato Y. Serum antibodies to human pituitary membrane antigens in patients with autoimmune lymphocytic hypophysitis and infundibuloneurohypophysitis. Clin Endocrinol (Oxf) 2001; 54 (03) 327-333
- 20 De Bellis A, Pane E, Bellastella G. , et al; Italian Autoimmune Hypophysitis Network Study. Detection of antipituitary and antihypothalamus antibodies to investigate the role of pituitary or hypothalamic autoimmunity in patients with selective idiopathic hypopituitarism. Clin Endocrinol (Oxf) 2011; 75 (03) 361-366
- 21 Lupi I, Manetti L, Raffaelli V. , et al. Pituitary autoimmunity is associated with hypopituitarism in patients with primary empty sella. J Endocrinol Invest 2011; 34 (08) e240-e244
- 22 Ward L, Paquette J, Seidman E. , et al. Severe autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy in an adolescent girl with a novel AIRE mutation: response to immunosuppressive therapy. J Clin Endocrinol Metab 1999; 84 (03) 844-852
- 23 Tubridy N, Saunders D, Thom M. , et al. Infundibulohypophysitis in a man presenting with diabetes insipidus and cavernous sinus involvement. J Neurol Neurosurg Psychiatry 2001; 71 (06) 798-801
- 24 Lecube A, Francisco G, Rodríguez D. , et al. Lymphocytic hypophysitis successfully treated with azathioprine: first case report. J Neurol Neurosurg Psychiatry 2003; 74 (11) 1581-1583
- 25 Curtò L, Torre ML, Cotta OR. , et al. Lymphocytic hypophysitis: differential diagnosis and effects of high-dose pulse steroids, followed by azathioprine, on the pituitary mass and endocrine abnormalities—report of a case and literature review. Sci World J 2010; 10: 126-134
- 26 Lu Z, Yang G, Ba J, Dou J, Mu Y, Lu J. Spontaneous pregnancy in a patient with a relapse of lymphocytic hypophysitis successfully treated with azathioprine and glucocorticoids. Endocrine 2011; 39 (03) 296-297
- 27 Papanastasiou L, Pappa T, Tsiavos V. , et al. Azathioprine as an alternative treatment in primary hypophysitis. Pituitary 2011; 14 (01) 16-22
- 28 Yang GQ, Lu ZH, Gu WJ. , et al. Recurrent autoimmune hypophysitis successfully treated with glucocorticoids plus azathioprine: a report of three cases. Endocr J 2011; 58 (08) 675-683
- 29 Schreckinger M, Francis T, Rajah G, Jagannathan J, Guthikonda M, Mittal S. Novel strategy to treat a case of recurrent lymphocytic hypophysitis using rituximab. J Neurosurg 2012; 116 (06) 1318-1323
- 30 Bianchi A, Mormando M, Doglietto F. , et al. Hypothalamitis: a diagnostic and therapeutic challenge. Pituitary 2014; 17 (03) 197-202
- 31 Selch MT, DeSalles AA, Kelly DF. , et al. Stereotactic radiotherapy for the treatment of lymphocytic hypophysitis. Report of two cases. J Neurosurg 2003; 99 (03) 591-596
- 32 Leung GK, Lopes MB, Thorner MO, Vance ML, Laws Jr ER. Primary hypophysitis: a single-center experience in 16 cases. J Neurosurg 2004; 101 (02) 262-271
- 33 Ray DK, Yen CP, Vance ML, Laws ER, Lopes B, Sheehan JP. Gamma knife surgery for lymphocytic hypophysitis. J Neurosurg 2010; 112 (01) 118-121
- 34 Kartal I, Yarman S, Tanakol R, Bilgic B. Lymphocytic panhypophysitis in a young man with involvement of the cavernous sinus and clivus. Pituitary 2007; 10 (01) 75-80
- 35 Gellner V, Kurschel S, Scarpatetti M, Mokry M. Lymphocytic hypophysitis in the pediatric population. Childs Nerv Syst 2008; 24 (07) 785-792
- 36 Yamagami K, Yoshioka K, Sakai H. , et al. Treatment of lymphocytic hypophysitis by high-dose methylprednisolone pulse therapy. Intern Med 2003; 42 (02) 168-173
- 37 Minniti G, Osti MF, Niyazi M. Target delineation and optimal radiosurgical dose for pituitary tumors. Radiat Oncol 2016; 11 (01) 135
- 38 Lanciano R, Fowble B, Sergott RC. , et al. The results of radiotherapy for orbital pseudotumor. Int J Radiat Oncol Biol Phys 1990; 18 (02) 407-411
- 39 Sheehan JP, Niranjan A, Sheehan JM. , et al. Stereotactic radiosurgery for pituitary adenomas: an intermediate review of its safety, efficacy, and role in the neurosurgical treatment armamentarium. J Neurosurg 2005; 102 (04) 678-691