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Hypophysitis Pituitary DOI 10.1007/s11102-006-0415-6 Hypophysitis Edward R. Laws · Mary Lee Vance · John A. Jane Jr. Published online: 31 October 2006 C Springer Science + Business Media, LLC 2006 Abstract Hypophysitis is an uncommon inflammatory con- The condition may affect both sexes and may occur in dition that may affect the pituitary gland and stalk. Patients adults of any age. often present with varying degrees of hypopituitarism. The There are two basic varieties of hypophysitis [4, 6]. The diagnosis is often made presumptively based on clinical his- first of these is lymphocytic hypophysitis, which is usually tory and biochemical data but may also be supported by mag- associated with pregnancy and autoimmune phenomena. The netic resonance imaging. Therapy is generally supportive in second is granulomatous hypophysitis, which may be asso- nature but may require surgery for pathological diagnosis ciated with sarcoid or other granulomatous processes. and treatment of mass effect. There is a significant differential diagnosis with regard to different forms of hypophysitis, and one must consider Keywords Hypophysitis . Pituitary . Hypopituitarism . the possibility of sarcoid, tuberculosis, histiocytosis, fungal Transsphenoidal surgery infection, abscess-bacterial or fungal, germinoma and other infiltrative neoplasms in the region, and the Tolosa-Hunt Syndrome. In some cases the imaging characteristics of hy- Demographic and clinical findings pophysitis are identical to those of hyperplasia of the pituitary (e.g. TSH hyperplasia secondary to hypothyroidism). Hypophysitis is an uncommon condition and represents a fairly broad spectrum of inflammatory lesions that can affect the pituitary gland and the pituitary stalk [2, 4, 6, 12]. The Diagnosis clinical presentation ordinarily includes headache, nausea and vomiting, hypopituitarism, and diabetes insipidus which Biochemical diagnosis usually is episodic, and these symptoms can respond variably to symptomatic therapy. In most patients, hypophysitis presents with hypopituitarism, Hypophysitis is often associated with pregnancy, but can and therefore a thorough analysis of the pituitary hormone be associated with other forms of autoimmune disease [1, 5, function and its response to stress is essential in the ini- 13]. In many cases hypophysitis may not obviously be asso- tial characterization of the disease. Diabetes insipidus, when ciated with other disorders [7, 10]. Often the clinical course present, should be documented by appropriate measurement involves relapses and this is particularly true of the active in- of serum and urine osmolality and electrolytes. flammatory aspects of lymphocytic hypophysitis, which may With regard to the nuances of differential diagnosis, one respond temporarily to corticosteroid-based therapy [11]. must also consider measuring markers of other types of hy- pophysitis, which include ACE levels, the hematological pro- file including the differential count, the CBC, CRP and ESR. E. R. Laws () · M. L. Vance · J. A. Jane Jr. University of Virginia, School of Medicine, Appropriate laboratory tests and cultures for infectious Charlottesville, VA 22908, USA disease, particularly tuberculosis and fungus, need to be con- e-mail: [email protected] sidered as well. Springer Pituitary Pituitary imaging with significant and intractable headache [8]. Some of these are candidates for initial surgical therapy with the goal of The characteristic imaging features of hypophysitis include a removing as much of the sellar mass as possible. A definitive homogeneous enlargement of the pituitary with a peak in the diagnosis can also be made on the basis of tissue obtained at diaphragm giving it a pear-shaped appearance on the coronal surgery. Other indications for surgical management are lack images [9]. A typical feature is enlargement of the pituitary of response to medical therapy or clinical and MRI relapse stalk with extension of the thickened stalk up toward the occurring after cessation of medical therapy. The outcome hypothalamus. In granulomatous hypophysitis in particular, of surgical treatment is usually satisfactory, however, there there may be extension of the disease into the cavernous have been some cases of relapse even after surgery, and these sinuses which can be detected on MRI studies. MRI with represent difficult management problems. contrast and dedicated pituitary sequences is the best way of diagnosing the anatomic extent of the disease. Radiotherapy/radiosurgery Secondary effects of disease Radiotherapy has been utilized in a limited number of pa- tients with refractory hypophysitis. We have had satisfac- In hypophysitis, involvement of the pituitary stalk and hy- tory experience with these cases using Gamma Knife radio- pothalamus often causes diabetes insipidus, which may be surgery, and a low dose of radiation is often effective. a presenting complaint. Involvement of the stalk may also produce secondary hypopituitarism which can also occur as Treatment algorithms a result of inflammatory destruction of the normal anterior pituitary gland itself. The process is normally destructive to It is difficult to generalize with regard to treatment algo- both the pituitary gland the pituitary stalk, leading to the rithms for hypophysitis. The treatment sequence really de- death of ADH secreting cells in the hypothalamus. For these pends upon the results of the diagnostic studies as well as reasons, patients rarely recover from the diabetes insipidus upon the tissue diagnosis. Many patients who present with that is associated with hypophysitis, and often do not recover the classical picture of lymphocytic hypophysitis can suc- from the hypopituitarism. cessfully be treated with medication. If they do not respond A recognized feature of granulomatous hypophysitis is to medical therapy, or if they develop a relapse in their clin- extension into the cavernous sinus that can produce vascular ical course, or if they have mass effect with visual loss, then insufficiency and even occlusion of the cavernous segment surgery can be successful management. In other patients who of the internal carotid artery. This, of course, can lead to present in an atypical fashion, some can also be treated medi- ischemia and stroke. cally, but often will require surgical management. In patients who represent failures of medical and surgical management, Treatment low dose radiosurgery or the use of Methotrexate can be considered. Medical therapy Criteria for disease control The diagnosis of hypophysitis can often be made presump- tively because of the clinical setting, particularly in associa- Control of disease in this category of pituitary pathology tion with pregnancy or in the presence of autoimmune dis- would include relief of symptoms, primarily headache and ease. If there is a clinical and imaging picture sufficient for visual loss and the manifestations of inflammatory disease the diagnosis of hypophysitis, then a preliminary period of which include vascular compromise, and constrictive in- medical management is desirable and is usually done with volvement of vessels and nerves in the cavernous sinus. As high dose corticosteroid medication [3]. Pituitary replace- mentioned, hypopituitarism must be characterized and cor- ment for other pituitary hormones can also be instituted and rected, along with diabetes insipidus. diabetes insipidus should be treated effectively with dDAVP. Medical therapy in refractory cases may also include the ju- dicious use of Methotrexate, which has been helpful in some Follow-up difficult cases. These patients, treated for one or another form of hypophysi- Surgical therapy tis, require periodic imaging, which should be done at least once a year for the first three or four years and then Many patients with mass effect and visual loss in association periodically thereafter depending upon the course of the with the hypopituitarism and diabetes insipidus will present disease. Springer Pituitary Obviously the clinical course must be carefully followed. 6. Gutenberg A, Buslei R, Fahlbusch R, Buchfelder M, Bruck Some patients require permanent pituitary replacement ther- W (2005) Immunopathology of primary hypophysitis: im- apy, which should be carefully monitored using pituitary plications for pathogenesis. Am J Surg Pathol 29(3):329– 338 hormonal analysis every six months. Patients must also be 7. Lee JH, Laws ER Jr, Guthrie BL, et al (1994) Lymphocytic monitored for recurrence or evolution of the disease process. hypophysitis: occurrence in two men. Neurosurgery 34:159–162 8. Leung GKA, Lopes MB, Thorner MO, Vance ML, Laws ER Jr (2004) Primary hypophysitis—a single center experience of 16 References cases. J Neurosurg (in press) 9. Levine SN, Benzel EC, Fowler MR, Shroyer JV III, Mirfakhraee M (1988) Lymphocytic adenohypophysitis: clinical, radiological, 1. Asa SL, Bilbao JM, Kovacs K, et al (1981) Lymphocytic and magnetic resonance imaging characterization. Neurosurgery hypophysitis of pregnancy resulting in hypopituitarism: a distant 22:937–941 clinicopathologic entity. Ann Intern Med 95:166–171 10. Ng WH, Gonzales M, Kaye AH (2003) Lymphocytic hypophysitis. 2. Caturegli P,Newschaffer C, Olivi A, Pomper MG, Burger PC, Rose J Clin Neurosci 10(4):409–413 NR (2005) Autoimmune hypophysitis. Endocr Rev 26(5):599–614 11. Reusch JE, Kleinschmidt-DeMasters BK, Lillehei KO, et al 3. Cheung CC, Ezzat S, Smyth HS, et al (2001) The spectrum (1992) Preoperative diagnosis of lymphocytic hypophysitis and significance of primary hypophysitis. J Clin Endocr Metab (adenohypophysitis) unresponsive to short course dexamethasone: 86:1048–1053 case report. Neurosurgery 30:268–272 4. Folkerth RD, Price DL Jr, Schwartz M, Black PM, de 12. Sautner D, Saeger W, Ludecke DK, Jansen V, Puchner MJ Girolami U (1998) Xanthomatous hypophysitis. Am J Surg (1995) Hypophysitis in surgical and autoptical specimens. Acta Pathol 22(6):736–741 Neuropathol 90:637–644 5. Goudie RB, Pinkerton PH (1962) Anterior hypophysitis and 13. Thodou E, Asa SL, Kontogeorgos G, et al (1995) Clinical case Hashimoto’s disease in a young woman. J Pathol Bacter seminar: lymphocytic hypophysitis: clinicopathological findings. 83:584–585 J Clin Endocr Metab 80:2302–2311 Springer.
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