Novel strategy to treat a case of recurrent lymphocytic hypophysitis using rituximab

Case report

Matthew Schreckinger M.D., Todd Francis M.D., Ph.D., Gary Rajah B.S., Jay Jagannathan M.D., Murali Guthikonda M.D., and Sandeep Mittal M.D., F.R.C.S.C.
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  • Department of Neurosurgery, Wayne State University, Detroit, Michigan
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Lymphocytic hypophysitis is an uncommon autoimmune condition that often results in significant morbidity. Although most cases resolve spontaneously or after a short course of steroids, rarely, refractory cases can cause persistent neurological deficits despite aggressive medical and surgical management.

A 41-year-old woman presented with progressive visual loss in the left eye and was found to have a sellar mass. She underwent transsphenoidal surgery because of lesion enlargement. Histopathology was consistent with adenohypophysitis with B-cell predominance. Despite steroid treatment, her neurological condition worsened and she experienced loss of vision in the right eye. Craniotomy with decompression of the right optic nerve was performed. Her condition improved initially, but she continued to have progressive visual compromise over the following months. She was therefore treated with rituximab, a monoclonal antibody against B cells. Her vision improved significantly within a few weeks. There was no clinical or radiographic exacerbation 2 years after starting immunotherapy.

Rituximab, an anti-CD20 antibody that specifically depletes B lymphocytes, can be an effective treatment strategy for patients with steroid-refractory, B cell–predominant lymphocytic hypophysitis.

Abbreviation used in this paper:LH = lymphocytic hypophysitis.

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Contributor Notes

Address correspondence to: Sandeep Mittal, M.D., F.R.C.S.C., Department of Neurosurgery, Wayne State University, 4160 John R Street, Suite 930, Detroit, Michigan 48201. email: smittal@med.wayne.edu.

Please include this information when citing this paper: published online March 23, 2012; DOI: 10.3171/2012.2.JNS111456.

  • 1

    Bitton RN, , Slavin M, , Decker RE, , Zito J, & Schneider BS: The course of lymphocytic hypophysitis. Surg Neurol 36:4043, 1991

  • 2

    Caturegli P, , Lupi I, , Landek-Salgado M, , Kimura H, & Rose NR: Pituitary autoimmunity: 30 years later. Autoimmun Rev 7:631637, 2008

  • 3

    Caturegli P, , Newschaffer C, , Olivi A, , Pomper MG, , Burger PC, & Rose NR: Autoimmune hypophysitis. Endocr Rev 26:599614, 2005

  • 4

    Cosman F, , Post KD, , Holub DA, & Wardlaw SL: Lymphocytic hypophysitis. Report of 3 new cases and review of the literature. Medicine (Baltimore) 68:240256, 1989

    • Search Google Scholar
    • Export Citation
  • 5

    Ezzat S, & Josse RG: Autoimmune hypophysitis. Trends Endocrinol Metab 8:7480, 1997

  • 6

    Goudie RB, & Pinkerton PH: Anterior hypophysitis and Hashimoto's disease in a young woman. J Pathol Bacteriol 83:584585, 1962

  • 7

    Gutenberg A, , Hans V, , Puchner MJ, , Kreutzer J, , Brück W, & Caturegli P, : Primary hypophysitis: clinical-pathological correlations. Eur J Endocrinol 155:101107, 2006

    • Search Google Scholar
    • Export Citation
  • 8

    Howlett TA, , Levy MJ, & Robertson IJ: How reliably can autoimmune hypophysitis be diagnosed without pituitary biopsy. Clin Endocrinol (Oxf) 73:1821, 2010

    • Search Google Scholar
    • Export Citation
  • 9

    Kageyama K, , Naraoka M, , Sakihara S, , Ikeda H, , Sano T, & Suda T: A progressive case of lymphocytic hypophysitis accompanied by paresis of the left abducens nerve. Intern Med 47:12451249, 2008

    • Search Google Scholar
    • Export Citation
  • 10

    Laws ER, , Vance ML Jr, & Jane JA Jr: Hypophysitis. Pituitary 9:331333, 2006

  • 11

    Lecube A, , Francisco G, , Rodríguez D, , Ortega A, , Codina A, & Hernández C, : Lymphocytic hypophysitis successfully treated with azathioprine: first case report. J Neurol Neurosurg Psychiatry 74:15811583, 2003

    • Search Google Scholar
    • Export Citation
  • 12

    Leung GK, , Lopes MB, , Thorner MO, , Vance ML, & Laws ER Jr: Primary hypophysitis: a single-center experience in 16 cases. J Neurosurg 101:262271, 2004

    • Search Google Scholar
    • Export Citation
  • 13

    Menon SK, , Sarathi V, , Bandgar TR, , Menon PS, , Goel N, & Shah NS: Autoimmune hypophysitis: a single centre experience. Singapore Med J 50:10801084, 2009

    • Search Google Scholar
    • Export Citation
  • 14

    Ng WH, , Gonzales M, & Kaye AH: Lymphocytic hypophysitis. J Clin Neurosci 10:409413, 2003

  • 15

    Nishioka H, , Ito H, , Miki T, & Akada K: A case of lymphocytic hypophysitis with massive fibrosis and the role of surgical intervention. Surg Neurol 42:7478, 1994

    • Search Google Scholar
    • Export Citation
  • 16

    Papanastasiou L, , Pappa T, , Tsiavos V, , Tseniklidi E, , Androulakis I, & Kontogeorgos G, : Azathioprine as an alternative treatment in primary hypophysitis. Pituitary 14:1622, 2011

    • Search Google Scholar
    • Export Citation
  • 17

    Pestell RG, , Best JD, & Alford FP: Lymphocytic hypophysitis. The clinical spectrum of the disorder and evidence for an autoimmune pathogenesis. Clin Endocrinol (Oxf) 33:457466, 1990

    • Search Google Scholar
    • Export Citation
  • 18

    Ray DK, , Yen CP, , Vance ML, , Laws ER, , Lopes B, & Sheehan JP: Gamma knife surgery for lymphocytic hypophysitis. Case report. J Neurosurg 112:118121, 2010

    • Search Google Scholar
    • Export Citation
  • 19

    Rivera JA: Lymphocytic hypophysitis: disease spectrum and approach to diagnosis and therapy. Pituitary 9:3545, 2006

  • 20

    Selch MT, , DeSalles AA, , Kelly DF, , Frighetto L, , Vinters HV, & Cabatan-Awang C, : Stereotactic radiotherapy for the treatment of lymphocytic hypophysitis. Report of two cases. J Neurosurg 99:591596, 2003

    • Search Google Scholar
    • Export Citation
  • 21

    Sinha D, , Sinha A, & Pirie AM: A case of recurrent lymphocytic hypophysitis in pregnancy. J Obstet Gynaecol 26:255256, 2006

  • 22

    Supler ML, & Mickle JP: Lymphocytic hypophysitis: report of a case in a man with cavernous sinus involvement. Surg Neurol 37:472476, 1992

    • Search Google Scholar
    • Export Citation
  • 23

    Tony HP, , Burmester G, , Schulze-Koops H, , Grunke M, , Henes J, & Kötter I, : Safety and clinical outcomes of rituximab therapy in patients with different autoimmune diseases: experience from a national registry (GRAID). Arthritis Res Ther 13:R75, 2011

    • Search Google Scholar
    • Export Citation
  • 24

    Tubridy N, , Saunders D, , Thom M, , Asa SL, , Powell M, & Plant GT, : Infundibulohypophysitis in a man presenting with diabetes insipidus and cavernous sinus involvement. J Neurol Neurosurg Psychiatry 71:798801, 2001

    • Search Google Scholar
    • Export Citation
  • 25

    Ward L, , Paquette J, , Seidman E, , Huot C, , Alvarez F, & Crock P, : Severe autoimmune polyendocrinopathy-candidiasisectodermal dystrophy in an adolescent girl with a novel AIRE mutation: response to immunosuppressive therapy. J Clin Endocrinol Metab 84:844852, 1999

    • Search Google Scholar
    • Export Citation
  • 26

    Yamagami K, , Yoshioka K, , Sakai H, , Fukumoto M, , Yamakita T, & Hosoi M, : Treatment of lymphocytic hypophysitis by high-dose methylprednisolone pulse therapy. Intern Med 42:168173, 2003

    • Search Google Scholar
    • Export Citation

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