An Overview of Diagnosis of Primary Autoimmune Hypophysitis in a Single Prospective Monocentric Experience

INTRODUCTION: Autoimmune hypophysitis (AH) is a rare disease with a not well\r\nknown natural progression Aim: To collect representative data on clinical\r\nfeatures of AH and better characterize the disease.\r\nPATIENTS AND METHODS: A monocentric prospective study was designed. AH affected\r\npatients, evaluated at our tertiary care Pituitary Unit from 2011 were enrolled. \r\nAfter ruling out other pituitary mass and secondary causes of hypophysitis, AH\r\nwas the exclusion diagnosis. AH was classified as adeno-hypophysitis,\r\npan-hypophysitis and infundibulo-neuro-hypophysitis according to clinical and\r\nneuroradiological findings.\r\nRESULTS: 21 patients met the inclusion criteria: 9 were diagnosed as\r\nadeno-hypophysitis, 4 as pan-hypophysitis and 8 as\r\ninfundibulo-neuro-hypophysitis. Frequency of secondary hypoadrenalism overlapped \r\nin adeno-hypophysitis, pan-hypophysitis and infundibulo-neuro-hypophysitis.\r\nGrowth hormone deficit and secondary hypogonadism occurred more frequently in\r\ninfundibulo-neuro-hypophysitis as compared to adeno-hypophysitis and\r\npan-hypophysitis (p = 0.009; p = 0.04). All cases of multiple pituitary secretion\r\ndeficits occurred in cases of infundibulo-neuro-hypophysitis (p = 0.04). No\r\ncorrelation between hypophysitis subtypes and the anti-pituitary and\r\nanti-hypothalamus autoantibodies were found. A higher frequency of ENA and ANA\r\nantibodies was found in cases of pan-hypophysitis (respectively OR: 5.0 95% CI:\r\n0.86-28.8; p < 0.001 and 1.8 95% CI: 1.1-3.2; p = 0.02) as compared to\r\nadeno-hypophysitis and infundibulo-neuro-hypophysitis.\r\nCONCLUSION: Infundibulo-neuro-hypophysitis should be taken into account in the\r\naetiological diagnosis of hypopituitarism, particularly if associated with\r\ndiabetes insipidus and in cases of growth hormone deficit, secondary hypogonadism\r\nor multiple hormone deficits. Contrast enhanced-MRI is crucial in the clinical\r\nand non invasive diagnosis of hypophysitis. A screening of auto-antibodies,\r\nparticularly anti-ENA and anti-ANA, is strongly suggested, in the clinical\r\ncontest of hypophysitis.