The diagnosis and management of lymphocytic hypophysitis

Abstract

Although a relatively rare phenomenon, lymphocytic hypophysitis can represent a difficult diagnostic and therapeutic challenge. This condition was initially thought to be an autoimmune phenomenon occurring primarily in women in the postpartum state. It is now recognized as a disorder that can affect both men and women over a rather large age range, from young adults to the elderly, and may be linked to a number of autoimmune phenomena. We have reviewed the literature on lymphocytic hypophysitis, and added the detailed analysis of seven cases of presumed lymphocytic hypophysitis from our own experience. The case studies demonstrate the wide spectrum of manifestations of this disorder, the differential diagnosis and the rationale and the nuances of treatment. Diagnostic studies, including laboratory evaluation and the characteristic MRIs, are reviewed. The histopathology and results of special stains are also presented, emphasizing the characteristic pathologic findings in this spectrum of pituitary disease. Our experience and that of others demonstrates that this condition has a characteristic presentation – that is, headache, hypopituitarism and diabetes insipidus. When significant mass effect is present, suprasellar and parasellar extension may produce visual loss and/or diplopia. Treatment strategies and outcomes are given for each of the seven patients. Corticosteroids are recommended as first-line management, and are usually successful. Relapses requiring additional therapy, however, are not uncommon. In such cases, trans-sphenoidal surgery, other more aggressive forms of medical therapy and rarely, radiosurgery can also be employed. Lymphocytic hypophysitis is a challenging entity. With accurate diagnosis and sequential, expertly managed therapeutic measures, it can be controlled. Continued surveillance of the patients is essential, as relapses may occur.