Summary
The most common form of primary hypophysitis is lymphocytic hypophysitis (LH), which is a rare inflammatory disorder of the pituitary that may present both as a sellar mass extending into the suprasellar space and as hypopituitarism. It is often confused radiographically with pituitary tumors. It usually presents in the peripartum period and may be associated with autoimmune diseases. The clinical context is usually quite important in raising the clinical suspicion of this disorder. This chapter discusses the epidemiology, pathogenesis, clinical presentation, imaging, differential diagnosis, and management of LH.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Rivera JA. Lymphocytic hypophysitis: disease spectrum and approach to diagnosis and therapy. Pituitary 2006;9(1):35–45.
Caturegli P, Newschaffer C, Olivi A, Pomper MG, Burger PC, Rose NR. Autoimmune hypophysitis. Endocr Rev 2005;26(5):599–614.
Gutenberg A, Hans V, Puchner MJ, et al. Primary hypophysitis: clinical-pathological correlations. Eur J Endocrinol 2006;155(1):101–7.
Imura H, Nakao K, Shimatsu A, et al. Lymphocytic infundibuloneurohypophysitis as a cause of central diabetes insipidus. N Engl J Med 1993;329(10):683–9.
Goudie RB, Pinkerton PH. Anterior hypophysitis and Hashimoto’s disease in a young woman. J Pathol Bacteriol 1962;83:584–5.
Hashimoto K, Takao T, Makino S. Lymphocytic adenohypophysitis and lymphocytic infundibuloneurohypophysitis. Endocr J 1997;44(1):1–10.
Crock PA. Cytosolic autoantigens in lymphocytic hypophysitis. J Clin Endocrinol Metab 1998;83(2):609–18.
Tanaka S, Tatsumi KI, Takano T, et al. Anti-alpha-enolase antibodies in pituitary disease. Endocr J 2003;50(6):697–702.
O’Dwyer DT, Clifton V, Hall A, Smith R, Robinson PJ, Crock PA. Pituitary autoantibodies in lymphocytic hypophysitis target both gamma- and alpha-Enolase – a link with pregnancy? Arch Physiol Biochem 2002;110(1–2):94–8.
Goswami R, Kochupillai N, Crock PA, Jaleel A, Gupta N. Pituitary autoimmunity in patients with Sheehan’s syndrome. J Clin Endocrinol Metab 2002;87(9):4137–41.
Bensing S, Hulting AL, Hoog A, Ericson K, Kampe O. Lymphocytic hypophysitis: report of two biopsy-proven cases and one suspected case with pituitary autoantibodies. J Endocrinol Invest 2007;30(2):153–62.
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(3):327–33.
De Bellis A, Colao A, Bizzarro A, et al. Longitudinal study of vasopressin-cell antibodies and of hypothalamic-pituitary region on magnetic resonance imaging in patients with autoimmune and idiopathic complete central diabetes insipidus. J Clin Endocrinol Metab 2002;87(8):3825–9.
Maghnie M, Ghirardello S, De Bellis A, et al. Idiopathic central diabetes insipidus in children and young adults is commonly associated with vasopressin-cell antibodies and markers of autoimmunity. Clin Endocrinol (Oxf) 2006;65(4):470–8.
Bellastella A, Bizzarro A, Coronella C, Bellastella G, Sinisi AA, De Bellis A. Lymphocytic hypophysitis: a rare or underestimated disease? Eur J Endocrinol 2003;149(5):363–76.
Flanagan DE, Ibrahim AE, Ellison DW, Armitage M, Gawne-Cain M, Lees PD. Inflammatory hypophysitis – the spectrum of disease. Acta Neurochir (Wien) 2002;144(1):47–56.
Thodou E, Asa SL, Kontogeorgos G, Kovacs K, Horvath E, Ezzat S. Clinical case seminar: lymphocytic hypophysitis: clinicopathological findings. J Clin Endocrinol Metab 1995;80(8):2302–11.
Beressi N, Beressi JP, Cohen R, Modigliani E. Lymphocytic hypophysitis. A review of 145 cases. Ann Med Interne (Paris) 1999;150(4):327–41.
Jensen MD, Handwerger BS, Scheithauer BW, Carpenter PC, Mirakian R, Banks PM. Lymphocytic hypophysitis with isolated corticotropin deficiency. Ann Intern Med 1986;105(2):200–3.
Kristof RA, Van Roost D, Klingmuller D, Springer W, Schramm J. Lymphocytic hypophysitis: non-invasive diagnosis and treatment by high dose methylprednisolone pulse therapy? J Neurol Neurosurg Psychiatry 1999;67(3):398–402.
Matta MP, Kany M, Delisle MB, Lagarrigue J, Caron PH. A relapsing remitting lymphocytic hypophysitis. Pituitary 2002;5(1):37–44.
Perez-Nunez A, Miranda P, Arrese I, Gonzalez P, Ramos A, Lobato RD. Lymphocytic hypophysitis with cystic MRI appearance. Acta Neurochir (Wien) 2005;147(12):1297–300.
Tamiya A, Saeki N, Kubota M, Oheda T, Yamaura A. Unusual MRI findings in lymphocytic hypophysitis with central diabetes insipidus. Neuroradiology 1999;41(12):899–900.
Ozbey N, Sencer A, Tanyolac S, et al. An intrasellar germinoma with normal cerebrospinal fluid beta-HCG concentrations misdiagnosed as hypophysitis. Hormones (Athens) 2006;5(1):67–71.
Shin JH, Lee HK, Choi CG, et al. MR imaging of central diabetes insipidus: a pictorial essay. Korean J Radiol 2001;2(4):222–30.
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(6):798–801.
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(1):75–80.
De Bellis A, Bizzarro A, Bellastella A. Pituitary antibodies and lymphocytic hypophysitis. Best Pract Res Clin Endocrinol Metab 2005;19(1):67–84.
Keda YM, Krjukova IV, Ilovaiskaia IA, et al. Antibodies to pituitary surface antigens during various pituitary disease states. J Endocrinol 2002;175(2):417–23.
Kobayashi T, Yabe S, Kikuchi T, Kanda T, Kobayashi I. Presence of anti-pituitary antibodies and GAD antibodies in NIDDM and IDDM. Diabetes Care 1997;20(5):864–6.
Komatsu M, Kondo T, Yamauchi K, et al. Antipituitary antibodies in patients with the primary empty sella syndrome. J Clin Endocrinol Metab 1988;67(4):633–8.
Mau M, Phillips TM, Ratner RE. Presence of anti-pituitary hormone antibodies in patients with empty sella syndrome and pituitary tumours. Clin Endocrinol (Oxf) 1993;38(5):495–500.
Scherbaum WA, Schrell U, Gluck M, Fahlbusch R, Pfeiffer EF. Autoantibodies to pituitary corticotropin-producing cells: possible marker for unfavourable outcome after pituitary microsurgery for Cushing’s disease. Lancet 1987;1(8547):1394–8.
Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 25–1995. A 44-year-old woman with headache, blurred vision, and an intrasellar mass. N Engl J Med 1995;333(7):441–7.
Cemeroglu AP, Blaivas M, Muraszko KM, Robertson PL, Vazquez DM. Lymphocytic hypophysitis presenting with diabetes insipidus in a 14-year-old girl: case report and review of the literature. Eur J Pediatr 1997;156(9):684–8.
Selch MT, DeSalles AA, Kelly DF, et al. Stereotactic radiotherapy for the treatment of lymphocytic hypophysitis. Report of two cases. J Neurosurg 2003;99(3):591–6.
Leung GK, Lopes MB, Thorner MO, Vance ML, Laws ER, Jr. Primary hypophysitis: a single-center experience in 16 cases. J Neurosurg 2004;101(2):262–71.
Lecube A, Francisco G, Rodriguez D, et al. Lymphocytic hypophysitis successfully treated with azathioprine: first case report. J Neurol Neurosurg Psychiatry 2003;74(11):1581–3.
Donadieu J, Rolon MA, Thomas C, et al. Endocrine involvement in pediatric-onset Langerhans’ cell histiocytosis: a population-based study. J Pediatr 2004;144(3):344–50.
Goyal M, Kucharczyk W, Keystone E. Granulomatous hypophysitis due to Wegener’s granulomatosis. AJNR Am J Neuroradiol 2000;21(8):1466–9.
Newman LS, Rose CS, Maier LA. Sarcoidosis. N Engl J Med 1997;336(17):1224–34.
Porter N, Beynon HL, Randeva HS. Endocrine and reproductive manifestations of sarcoidosis. QJM 2003;96(8):553–61.
Lower EE, Broderick JP, Brott TG, Baughman RP. Diagnosis and management of neurological sarcoidosis. Arch Intern Med 1997;157(16):1864–8.
Sharma OP. Effectiveness of chloroquine and hydroxychloroquine in treating selected patients with sarcoidosis with neurological involvement. Arch Neurol 1998;55(9):1248–54.
Carter JD, Valeriano J, Vasey FB, Bognar B. Refractory neurosarcoidosis: a dramatic response to infliximab. Am J Med 2004;117(4):277–9.
Prosch H, Grois N, Prayer D, et al. Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis. Pediatr Blood Cancer 2004;43(5):594–9.
Makras P, Samara C, Antoniou M, et al. Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH). Neuroradiology 2006;48(1):37–44.
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2008 Humana Press, Totowa, NJ
About this chapter
Cite this chapter
Ulmer, S., Byrne, T.N. (2008). Lymphocytic Hypophysitis and Inflammatory Disease of the Pituitary. In: Swearingen, B., Biller, B.M. (eds) Diagnosis and Management of Pituitary Disorders. Contemporary Endocrinology. Humana Press. https://doi.org/10.1007/978-1-59745-264-9_18
Download citation
DOI: https://doi.org/10.1007/978-1-59745-264-9_18
Publisher Name: Humana Press
Print ISBN: 978-1-58829-922-2
Online ISBN: 978-1-59745-264-9
eBook Packages: MedicineMedicine (R0)