Clinical Characteristics, Management, and Treatment Outcomes of Primary Hypophysitis: A Monocentric Cohort

Abstract

Primary hypophysitis (PH) is a rare autoimmune inflammatory disease of the pituitary gland. The aim of the study was to evaluate clinical characteristics, disease management, and outcomes of cases with PH. Medical records of PH patients admitted to Hacettepe University Hospital between 1999 and 2017 were analyzed retrospectively. Paraffin-embedded pathology blocks were obtained for both re-examination and IgG4 immunostaining. Twenty PH patients (15 females, 5 males) were evaluated. Mean age at diagnosis was 41.5±13.4 years. Some form of hormonal disorder was present in 63.2% of cases, hypogonadism (66.6%) being the most common. Panhypopituitarism was present in 36.8%. All patients had pituitary gland enlargement on magnetic resonance imaging; stalk thickening and loss of neurohypophyseal bright spot were present in 17.6 and 23.5%, respectively. Lymphocytic hypophysitis was the most common histopathological subtype (50%). Among pathology specimens available for IgG and IgG4 immunostaining (n=10), none fulfilled the criteria for IgG4-related hypophysitis. Four patients were given glucocorticoid treatment in diverse protocols; as initial therapy in 3. Sixteen cases underwent surgery, 7 of whom due to neuro-ophthalmologic involvement. Only 1 patient was observed without any intervention. Reduction of pituitary enlargement was seen in all surgical and glucocorticoid treated cases. None of the surgical patients showed hormonal improvement while one case in glucocorticoid group improved. PH should be considered in the differential diagnosis of sellar masses causing hormonal deficiencies. MRI findings are usually helpful, but not yet sufficient for definitive diagnosis of PH. Treatment usually improves symptoms and reduces sellar masses while hormonal recovery is less common.

Clinical presentation and magnetic resonance imaging characteristics of lymphocytic hypophysitis: a systematic review with meta-analysis

“…Thirteen studies [5][6][7][8][9][10][15][16][17][18][19][20][21] , involving a total of 241 patients, reported that LYH onset in female patients is related to pregnancy. Fifteen studies [5,[7][8][9][10][11][12][14][15][16][17][18][19][20][21] , involving a total of 340 patients, reported an association between LYH and other autoimmune diseases. In both study series, heterogeneity analysis revealed that there was statistical heterogeneity; thus, the random effects model was used for combined analysis.…”

“…All 17 studies [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] , involving a total of 364 patients, reported headache as a symptom caused by LYH. Fifteen studies [6][7][8][9][10][11][12][13][15][16][17][18][19][20][21] , involving a total of 350 patients, reported visual field loss and visual impairment caused by LYH.…”

“…All 17 studies [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] , involving a total of 364 patients, reported the occurrence of hypoadrenalism, hypothyroidism, and hypogonadism in LYH patients. Heterogeneity analysis revealed that there was statistical heterogeneity among these 17 studies; thus, the random effects model was used for combined analysis.…”

“…Thirteen studies [5][6][7][8][10][11][15][16][17][18][19][20][21] , involving a total of 333 patients, reported the occurrence of low growth hormone levels in LYH patients. Sixteen studies [5][6][7][8][9][10][11][12][14][15][16][17][18][19][20][21] , involving a total of P r e p r i n t series, heterogeneity analysis revealed that there was statistical heterogeneity; thus, the random effects model was used for combined analysis. The results showed that the incidence of growth hormone deficit in LYH patients was 0.22 (95% CI, 0.13 ~ 0.32) and the incidence of hyperprolactinemia in LYH patients was 0.29 (95% CI, 0.19 ~ 0.39) (Figure 5).…”

“…Ten different MRI characteristics of LYH were reported in 16 studies [5][6][7][8][9][10][12][13][14][15][16][17][18][19][20][21] , and each these MRI characteristics was reported in between 2 and 13 of the 16 studies. The results showed that the commonest MRI characteristics of LYH were pituitary contrast enhancement (63%), symmetric pituitary enlargement (60%), thickening of the pituitary stalk (58%), sella mass or suprasellar extension (58%), loss of posterior pituitary hyperintensity (50%), chiasmal compression (38%), central pituitary necrosis (24%), cystic appearance (17%), cavernous sinus compression (17%), and dural tail sign (9%).…”