Abstract
The inflammation of the pituitary gland is known as hypophysitis. It is a rare disease accounting for approximately 0.24%-0.88% of all pituitary diseases. The natural course of hypophysitis is variable. Main forms are histologically classified as lymphocytic, granulomatous, IgG4 related and xanthomatous. We aim to present our patients with hypophysitis and compare clinical, laboratory and radiological features. We retrospectively reviewed our database of 1.293 patients diagnosed with pituitary diseases between 2010 and 2017. Twelve patients with hypophysitis were identified. Demographical data, clinical features, endocrinological dysfunction, imaging findings, treatment courses and follow-up periods were evaluated. The frequency of hypophysitis was found 0.93% in all cases of the pituitary disease. Twelve patients (nine females and three males), ages between 17-61 years, were evaluated. The characteristic features of our patients tended to be predominantly female and young. Diagnosis of hypophysitis was made after pituitary biopsy in four patients, and in eight patients after pituitary operation due to adenoma. Headache (67%) and visual problems (33%) were the most frequent nonendocrine symptoms. Anterior pituitary hormone deficiencies (63.7%) and/or diabetes insipidus (17%) were seen among patients. According to histopathological forms, four had lymphocytic, seven had granulomatous and one had xanthogranulomatous types. Contrast enhancement heterogeneous and thickened pituitary stalk were the most common radiological alterations. Hypophysitis should be considered in the differential diagnosis of sellar masses. It can mimic pituitary adenomas in radiological and endocrinological aspects. The different patterns of pituitary hormone deficiencies may be seen in the course of the disease.
Highlights
All three cases of secondary hypophysitis were diagnosed with tuberculosis hypophysitis and the diagnosis of pituitary tuberculosis was made through a positive tuberculin skin test and response of an interferon-gamma release by QuantiFERON TB- Gold test
The prevalence and incidence of hypophysitis are not known with accuracy
Our study confirmed the male/female ratio of 1:3 with the dominance of females reported in recent studies
Summary
Department of Endocrinology between 2010 and 2017. Out of 1.293 patients, 12 patients who had histological confirmation of hypophysitis were included to the study. Demographical and laboratory data, clinical features, endocrinological dysfunction, magnetic resonance imaging (MRI) findings of the pituitary gland, treatment courses and follow-up period were all evaluated. All patients had initial and control MRI images taken of their pituitary gland. Pituitary gland volume was measured by the volume = 1/2 x length x width x height formula, as described in a previous study [8]. All patients underwent transsphenoidal microscopic surgery (TSS). After TSS, all specimens were exposed to hematoxylin and eosin (H&E) staining and immunohistochemical staining. After that, according to underlying disease, they were divided into primary or secondary hypophysitis. By the anatomical location, the cases were classified into adenohypophysitis, infundibuloneurohypophysitis or panhypophysitis. Pretreatment and post-treatment plasma concentrations of hormones related to the pituitary gland, such as ACTH, basal cortisol, thyrotropin stimulating hormone (TSH), free T4, free T3, growth hormone (GH), IGF-1, prolactin, folliclestimulating hormone (FSH), luteinizing hormone (LH) and estradiol or total and free testosterone levels, were evaluated. In addition, the function of the posterior pituitary gland was evaluated with clinical symptoms according to plasma and urinary osmolality and plasma sodium levels. The laboratory analyses of all these cases were evaluated. Growth hormone deficiency (GHD) was diagnosed by the insulin tolerance test (ITT) or glucagon test. In patients with clear-cut characteristics of GHD and three other documented pituitary hormone deficits the diagnosis of GHD was made without biochemical testing. Steroid replacement therapy was given to patients with adrenal insufficiency. Adrenal insufficiency diagnosed if a morning cortisol level < 3 μg/dL and a level > 15 μg/dL likely excluded an adrenal insufficiency diagnosis. In case of cortisol levels between 3-15 μg/dL adrenal insufficiency was assessed using ITT or standarddose ACTH stimulation tests. Peak cortisol levels > 18.1 μ/dL at 30 or 60 minutes excluded adrenal insufficiency. Central hypothyroidism diagnosis confirmed by free T4 level below the laboratory reference range in concurrence with a low, normal, or mildly elevated TSH [9]. For the follow-up in the first year, the patients were evaluated every six months after the surgery, followed by every year. The local ethical committee of Ankara Numune Education and Research Hospital approved the study.
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