An Elderly Patient with Sarcoidosis Manifesting Panhypopituitarism with Central Diabetes Insipidus

Abstract

Abstract. We here report a 77-year-old Japanese male who suffered general fatigue with progressive thirst and polyuria. Central diabetes insipidus was diagnosed by depletion of vasopressin secretion in response to increases in serum osmolality. Secretory responses of anterior pituitary hormones including adrenocorticotropin, thyrotropin, gonadotropins and growth hormone were severely impaired. Diffuse swelling of the infundibulum as well as lack of T1-hyperintense signal in the posterior lobe was noted by pituitary magnetic resonance imaging. The presence of bilateral hilar lymphadenopathy and increased CD4/CD8 ratio in bronchoalveolar lavage fluid was diagnostic for lung sarcoidosis. Physiological doses of corticosteroid and thyroid hormone were administered in addition to desmopressin supplementation. Complete regression of the neurohypophysial swelling was notable two years after corticosteroid replacement. Diffuse damage of anterior pituitary combined with hypothalamic involvement leading to central diabetes insipidus is a rare manifestation in such elderly patients with neurosarcoidosis.

Partial Improvement of Anterior Pituitary Deficiency Following Steroid Treatment in a Patient with Neurosarcoidosis Accompanied by Central Diabetes Insipidus

“…There have been few case reports that diabetes insipidus could be cured in this situation, except for one spontaneous remission case (11). It has been considered that anterior pituitary function is difficult to restore, because hypopituitarism is not improved although the thickened pituitary stalk and enlarged pituitary gland return to their normal shapes after steroid treatment (3,12). However, a few case reports have shown that steroid therapy is able to partially improve anterior pituitary hormone secretion, which was confirmed by an endocrine provocation test (12)(13)(14).…”

“…Diabetes insipidus is one clinical manifestation of neurosarcoidosis in patients with involved pituitary lesions (2), and this presentation of the disease is generally not cured by steroid treatment (3). In contrast, whether glucocorticoid administration improves the endocrine dysfunction of the anterior pituitary gland in neurosarcoidosis is unclear.…”

Spontaneous Remission of Diabetes Insipidus Due to CNS Sarcoidosis

“…The steroid therapy is a major treatment for CNS sarcoidosis (1,5,6 It is reported that CNS sarcoidosis progressively deteriorates and has a poor prognosis (7). In a literature of CDI due to sarcoidosis (10) …”

“…The incidence is 6 out of 100,000 persons in a year, and affects mainly objects in 20s to 40s (1). About 5% of patients with sarcoidosis have central nervous system (CNS) sarcoidosis (5,6) (3,7,8).…”

Medical Therapy of Sarcoidosis

“…76,79 This can lead to a panhypopituitarism. 80,81 In some cases, a pituitary lesion from sarcoidosis itself can be found. 82 In patients with panhypopituitarism, replacement with thyroid and glucocorticoid medications should be given.…”