The Diagnosis and Management of Lymphocytic Hypophysitis

Sherry L Iuliano; Edward R Laws

Disclosures

Expert Rev Endocrinol Metab. 2011;6(6):777-783. 

In This Article

Management Strategies

Medical Therapy

Medical treatment is initially based upon the use of corticosteroids to treat the inflammatory process. Ordinarily, this is given in the form of high-dose prednisone or equivalent steroids (hydrocortisone or dexamethasone), and tapered depending upon the extent and duration of the response. If there is a strong suspicion of lymphocytic hypophysitis and there is no visual loss, then corticosteroid therapy is usually given as first-line management. It is important to measure IgG4 levels prior to initiating steroid therapy.

As noted, a thorough pretreatment assessment of pituitary function is essential, because most patients develop one or more pituitary hormone deficiencies that can be replaced with appropriate hormone medications.

In the presence of diabetes insipidus, desmopressin (DDAVP) should be administered and the patient should be closely monitored for fluid intake and output, and particularly for serum sodium levels.

It has been reported that more than 72% of patients with lymphocytic hypophysitis will require life-long hormone replacement therapy.[9]

Medications such as methotrexate, azothiaprine and cyclosporine may be considered in patients who are resistant to corticosteroids or develop relapses after several courses of treatment.[4,6,10,12,13]

Surgical Therapy

Indications for a surgical procedure include lack of response to a vigorous trial of medical treatment for intractable symptoms. These may include headache, persistent diabetes insipidus and hypopituitarism. Patients presenting with intractable daily headache and mass effect with visual loss may consider the option of a trans-sphenoidal operation for the resection of part or all of the mass. This management strategy will also provide the necessary tissue for a definitive pathological diagnosis.[1,6,7] The surgical goal when the diagnosis is apparent from intraoperative pathological assessment is to debulk the mass in order to relieve the pressure that is responsible for headache, compression of remaining normal pituitary gland, and relief of pressure on the optic chiasm. Total hypophysectomy should not be the goal when the diagnosis is confirmed or highly suspected. In our practice, excision of one third to half of the mass usually suffices. Modern endoscopic trans-sphenoidal surgery is relatively safe and highly effective. Every effort is made to preserve normal uninvolved pituitary tissue.

Complications of surgery are uncommon; however, recurrence of symptoms and signs can develop, requiring further therapy.

Radiosurgery

Radiosurgery has been effectively employed in selected patients who have failed medical treatment or suffer from repeated recurrence of lymphocytic hypophysitis. Experience with this modality is limited, but success has been reported with the use of Gamma Knife radiosurgery (15 Gy prescription dose), and stereotactic radiotherapy in intractable cases.[14,15]

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