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|Year : 2018 | Volume
| Issue : 2 | Page : 405-406
Diagnosis and management of lymphocytic hypophysitis: A synopsis on current perspective
Anil Nanda, Amey R Savardekar, Devi Prasad Patra
Department of Neurosurgery, Louisiana State University Health Sciences Center, Shreveport, LA, USA
|Date of Web Publication||15-Mar-2018|
Dr. Anil Nanda
Department of Neurosurgery, Louisiana State University Health Sciences Center-Shreveport, 1501 Kings Highway, PO Box 33932, Shreveport, LA 71130-3932
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Nanda A, Savardekar AR, Patra DP. Diagnosis and management of lymphocytic hypophysitis: A synopsis on current perspective. Neurol India 2018;66:405-6
Hypophysitis, an autoimmune phenomenon, is characterized by inflammation and cellular infiltration of the pituitary gland. It can be either primary, when no other inflammatory causes are identified, or secondary to adjacent lesions or systemic diseases., Lymphocytic hypophysitis (LYH) or auto-immune hypophysitis (AH) is the most common histopathological subtype of primary hypophysitis and has very important diagnostic and therapeutic implications, considering that it is usually mistaken for tumors that may often require surgical management.,,
LYH or AH is typified by diffuse polyclonal lymphocytic infiltration with predominance of T cells (particularly [cluster of differentiation] CD4) and eventual destruction of the pituitary tissue manifested by various degrees of pituitary dysfunction. Histopathology is required for a definitive diagnosis of LYH; however, majority of the cases can be medically managed (with high dose steroids or rarely, with other immuno-suppressive therapies). With better understanding of the natural history of LYH and improvement in imaging technique and characterization of its specific MRI features, the diagnostic yield is much higher even in the absence of tissue diagnosis., Thus, diagnosing and treating LYH based on clinical, biochemical and radiological criteria, potentially avoids surgical intervention along with the associated risks.
Over the last decade, there has been a paradigm shift in the management strategy for LYH from surgical to non-surgical treatment.,, As the controversy over the optimal treatment strategy has subsided in favor of steroid pulse therapy or watchful observation, the discussion has migrated towards improving the diagnostic yield for LYH., We wish to emphasize the importance of clinical and radiological parameters in establishing the diagnosis in the absence of a histopathological confirmation.
Gutenberg et al., noted that approximately 40% of patients with LYH were diagnosed as pituitary macroadenomas on radiology and underwent unnecessary surgery. As there was no single radiological sign to accurately diagnose patients of LYH, they developed a scoring system that summarized numerous MR (magnetic resonance) imaging signs to increase the probability of diagnosing LYH pre-surgically. The criteria in their scoring system are as follows (the scores are in the bracket): age </=30 years (-1), presence of relation to pregnancy (-4), pituitary volume >6cm 3 (+2), medium or high gadolinium enhancement (-1), heterogenous gadolinium enhancement (+1), asymmetric sellar enlargement (+3), loss of posterior pituitary bright spot (-2), enlarged stalk size (-5), and presence of sphenoid mucosal thickening (+2). When these factors were considered for an individual patient, the cumulative score of greater than 1 suggested a diagnosis of adenoma, whereas a score of less than or equal to 0 suggested a diagnosis of AH, with a sensitivity of 92%, a specificity of 99%, a positive predictive value of 97%, and a negative predictive value of 97%. The Gutenberg score has resulted in a significant improvement in the diagnostic yield for autoimmune hypophysitis compared with the results obtained from the assessment of the individual parameters., Still, the differential diagnosis by MRI between a LYH and other pituitary masses is problematic and hence, a high degree of clinical suspicion and individualized patient care is advised, especially in patients with disproportionate affection of the anterior pituitary function vis-à-vis the magnitude of changes on MRI, in peri-partum patients and in patients with autoimmune disorders.
Kristof et al., reported the results of the first prospective trial of high dose methylprednisolone pulse therapy (HDMPT) in nine patients of LYH. Anterior pituitary function improved in four of the nine patients, diabetes insipidus ceased or improved in all four concerned patients, and MRI findings improved in seven patients. Lupi et al., in their systematic review of literature, identified 30 articles, reporting on 44 cases of autoimmune hypophysitis treated with glucocorticoids and/or azathioprine. Combining all the cases, medical therapy showed pituitary mass reduction in 84%, anterior pituitary function improvement in 45%, and posterior pituitary function improvement in 41% cases. Khare et al., in a well-documented study, presented the findings of 24 patients with autoimmune hypophysitis. In their cohort, 5 patients underwent surgical decompression through the transphenoidal route, 15 patients were managed conservatively with watchful observation and 4 patients were treated with steroid pulse therapy. There are several salient findings from this study. The symptoms of expanding sellar mass resolved immediately after surgery in the 'surgical decompression' group, while they resolved within one week in the 'steroid pulse therapy' group and within a median period of 1 month in the 'watchful observation' group. On serial imaging, in all 19 patients who did not undergo surgical decompression, the pituitary mass regressed within a median duration of 1.5 years. The pituitary axis recovery (complete or partial) at the last follow-up was seen in 20% (1/5) patients in the 'surgical decompression' group, 33% (5/15) patients in the 'watchful observation' group and 100% (4/4) patients in the 'steroid pulse therapy' group. The evidence from their study, which is backed by previous studies by Kristof et al., and Lupi et al., suggests that the recovery of hypopituitarism is better with steroid pulse therapy than that achieved without it.,, The natural history of LYH/AH (as seen by regression of the sellar lesion over time, in all 15 watchfully observed patients) depicted in their study, showed that surgical intervention (along with its accompanying complication rate of ~ 10%) was not warranted in LYH, unless cranial nerve dysfunction (vision loss or ophthalmoplegia) occurred at presentation or along its course.
The article by Panigrahi et al., provides an important insight into the entity of LYH for neurosurgeons, who are invariably consulted when the imaging finding of a sellar mass is discovered. The role of surgery has been gradually diminishing and recent literature on the subject has indeed shown better endocrinological outcome with steroid therapy as against surgery.,,, Complications of surgery, like postoperative meningitis and rhinorrhea requiring operative revision for LYH have been described in 0-10% of surgically treated LYH patients., Considering the complications, surgical intervention should be limited to cases with severe and/or deteriorating compressive signs or in cases with inconclusive findings of LYH in whom treatment would be based on histopathological examination., Non-operative management is the treatment of choice; however, this does not undermine the role of neurosurgical intervention for this disease., Panigrahi et al., have eloquently demonstrated the judicious consideration of surgery. In my personal series of over 500 transsphenoidal cases, I have encountered this entity on only 4 occasions. Retrospectively, one wishes that one must never encounter this entity surgically.
In a pituitary lesion without prior histopathological diagnosis, with unequivocal imaging findings, and with visual symptoms/ophthalmoplegia due to compression of the cranial nerves, surgery may be warranted. The optimal surgical strategy involves only partial resection or decompression of the mass (to relieve pressure over the surrounding structures) via a transsphenoidal approach, rather than an attempt at complete resection because surgery rarely improves the endocrine dysfunction.
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