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

Case report

Restricted access

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.

Article Information

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.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Coronal postcontrast T1-weighted MR image showing an enlarged, heterogeneously enhancing pituitary gland with mild mass effect on the optic chiasm and deviation of the pituitary stalk to the left (arrow).

  • View in gallery

    Magnetic resonance imaging studies obtained 3 months after initial presentation. Coronal (left) and sagittal (right) postcontrast T1-weighted MR images showing extension of the mass (2.1 × 1.2 × 1.3 cm) into the suprasellar cistern and left cavernous sinus. The infundibulum continues to be deviated to the left and is mildly thickened at its attachment to the gland. The optic chiasm is bowed superiorly and is mildly compressed on the left side. The mass appears to be part of the gland itself and is indistinguishable from the normal pituitary gland.

  • View in gallery

    Photomicrographs showing results of histopathological analysis. A: Nests of normal acinar cells surrounded by a dense infiltrate of inflammatory cells are seen on H & E stain. B: Immunostaining with CD3 reveals a minority of T cells. C: Immunostaining with CD20 confirms that the majority of the inflammatory infiltrate is composed of B lymphocytes. Original magnification × 40.

  • View in gallery

    Follow-up coronal postcontrast T1-weighted MR image obtained 18 months after the transsphenoidal biopsy, showing persistent enlargement of the pituitary gland, thickened infundibulum, and encasement of the left cavernous carotid artery (arrows).

  • View in gallery

    A: Coronal postcontrast T1-weighted MR image obtained 22 months after surgery, showing a mass extending into both cavernous sinuses (arrowheads) with compression of the optic chiasm (arrows). B: Axial T2-weighted MR image showing hyperintense signal in the left optic nerve (arrowheads). C: Axial T1-weighted MR image showing diffuse dural enhancement along the tuberculum sellae (arrows) encroaching on the optic nerves bilaterally at the level of the optic canal (arrowheads).

  • View in gallery

    Follow-up coronal MR image obtained 3 months after craniotomy, showing a larger mass with extension into the cavernous sinus bilaterally and superior displacement of the infundibulum and chiasm.

  • View in gallery

    Coronal MR image obtained 2 years after starting rituximab therapy, showing decreased size and enhancement of the sellar mass. The dural enhancement previously seen along the planum sphenoidale and optic nerve/chiasm is also decreased in intensity. There is no suprasellar extension of the gland.

References

  • 1

    Bitton RNSlavin MDecker REZito JSchneider BS: The course of lymphocytic hypophysitis. Surg Neurol 36:40431991

  • 2

    Caturegli PLupi ILandek-Salgado MKimura HRose NR: Pituitary autoimmunity: 30 years later. Autoimmun Rev 7:6316372008

  • 3

    Caturegli PNewschaffer COlivi APomper MGBurger PCRose NR: Autoimmune hypophysitis. Endocr Rev 26:5996142005

  • 4

    Cosman FPost KDHolub DAWardlaw SL: Lymphocytic hypophysitis. Report of 3 new cases and review of the literature. Medicine (Baltimore) 68:2402561989

    • Search Google Scholar
    • Export Citation
  • 5

    Ezzat SJosse RG: Autoimmune hypophysitis. Trends Endocrinol Metab 8:74801997

  • 6

    Goudie RBPinkerton PH: Anterior hypophysitis and Hashimoto's disease in a young woman. J Pathol Bacteriol 83:5845851962

  • 7

    Gutenberg AHans VPuchner MJKreutzer JBrück WCaturegli P: Primary hypophysitis: clinical-pathological correlations. Eur J Endocrinol 155:1011072006

    • Search Google Scholar
    • Export Citation
  • 8

    Howlett TALevy MJRobertson IJ: How reliably can autoimmune hypophysitis be diagnosed without pituitary biopsy. Clin Endocrinol (Oxf) 73:18212010

    • Search Google Scholar
    • Export Citation
  • 9

    Kageyama KNaraoka MSakihara SIkeda HSano TSuda T: A progressive case of lymphocytic hypophysitis accompanied by paresis of the left abducens nerve. Intern Med 47:124512492008

    • Search Google Scholar
    • Export Citation
  • 10

    Laws ERVance ML JrJane JA Jr: Hypophysitis. Pituitary 9:3313332006

  • 11

    Lecube AFrancisco GRodríguez DOrtega ACodina AHernández C: Lymphocytic hypophysitis successfully treated with azathioprine: first case report. J Neurol Neurosurg Psychiatry 74:158115832003

    • Search Google Scholar
    • Export Citation
  • 12

    Leung GKLopes MBThorner MOVance MLLaws ER Jr: Primary hypophysitis: a single-center experience in 16 cases. J Neurosurg 101:2622712004

    • Search Google Scholar
    • Export Citation
  • 13

    Menon SKSarathi VBandgar TRMenon PSGoel NShah NS: Autoimmune hypophysitis: a single centre experience. Singapore Med J 50:108010842009

    • Search Google Scholar
    • Export Citation
  • 14

    Ng WHGonzales MKaye AH: Lymphocytic hypophysitis. J Clin Neurosci 10:4094132003

  • 15

    Nishioka HIto HMiki TAkada K: A case of lymphocytic hypophysitis with massive fibrosis and the role of surgical intervention. Surg Neurol 42:74781994

    • Search Google Scholar
    • Export Citation
  • 16

    Papanastasiou LPappa TTsiavos VTseniklidi EAndroulakis IKontogeorgos G: Azathioprine as an alternative treatment in primary hypophysitis. Pituitary 14:16222011

    • Search Google Scholar
    • Export Citation
  • 17

    Pestell RGBest JDAlford FP: Lymphocytic hypophysitis. The clinical spectrum of the disorder and evidence for an autoimmune pathogenesis. Clin Endocrinol (Oxf) 33:4574661990

    • Search Google Scholar
    • Export Citation
  • 18

    Ray DKYen CPVance MLLaws ERLopes BSheehan JP: Gamma knife surgery for lymphocytic hypophysitis. Case report. J Neurosurg 112:1181212010

    • Search Google Scholar
    • Export Citation
  • 19

    Rivera JA: Lymphocytic hypophysitis: disease spectrum and approach to diagnosis and therapy. Pituitary 9:35452006

  • 20

    Selch MTDeSalles AAKelly DFFrighetto LVinters HVCabatan-Awang C: Stereotactic radiotherapy for the treatment of lymphocytic hypophysitis. Report of two cases. J Neurosurg 99:5915962003

    • Search Google Scholar
    • Export Citation
  • 21

    Sinha DSinha APirie AM: A case of recurrent lymphocytic hypophysitis in pregnancy. J Obstet Gynaecol 26:2552562006

  • 22

    Supler MLMickle JP: Lymphocytic hypophysitis: report of a case in a man with cavernous sinus involvement. Surg Neurol 37:4724761992

    • Search Google Scholar
    • Export Citation
  • 23

    Tony HPBurmester GSchulze-Koops HGrunke MHenes JKötter I: Safety and clinical outcomes of rituximab therapy in patients with different autoimmune diseases: experience from a national registry (GRAID). Arthritis Res Ther 13:R752011

    • Search Google Scholar
    • Export Citation
  • 24

    Tubridy NSaunders DThom MAsa SLPowell MPlant GT: Infundibulohypophysitis in a man presenting with diabetes insipidus and cavernous sinus involvement. J Neurol Neurosurg Psychiatry 71:7988012001

    • Search Google Scholar
    • Export Citation
  • 25

    Ward LPaquette JSeidman EHuot CAlvarez FCrock P: Severe autoimmune polyendocrinopathy-candidiasisectodermal dystrophy in an adolescent girl with a novel AIRE mutation: response to immunosuppressive therapy. J Clin Endocrinol Metab 84:8448521999

    • Search Google Scholar
    • Export Citation
  • 26

    Yamagami KYoshioka KSakai HFukumoto MYamakita THosoi M: Treatment of lymphocytic hypophysitis by high-dose methylprednisolone pulse therapy. Intern Med 42:1681732003

    • Search Google Scholar
    • Export Citation

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 174 174 12
Full Text Views 160 160 0
PDF Downloads 148 148 0
EPUB Downloads 0 0 0

PubMed

Google Scholar