Abstract
Objective Clinical data on primary hypophysitis are still scarce.
Especially non-surgical cases are underreported. We sought to analyse
clinical characteristics of primary hypophysitis, particularly in clinically
diagnosed patients.
Design Retrospective single centre study in 60 patients with primary
hypophysitis.
Methods Symptoms, MRI, histopathological findings, treatment and
outcomes were analysed in 12 histopathologically and 48 clinically diagnosed
patients. Diagnostic criteria for clinical diagnosis were: a) MRI findings
compatible with primary hypophysitis; b) course of disease excluding other
differential diagnoses. Mean duration of follow-up was 69 months.
Results Female sex was predominant (73%). Fatigue
(52%), headache (38%) and diabetes insipidus (38%)
were the most frequent symptoms. 42% had a concomitant autoimmune
disease. The corticotropic, thyrotropic, gonadotropic, somatotropic axis was
impaired in 67%, 57%, 52%, 20%,
respectively. Men had a higher number of impaired hormone axes
(p=0.022) with the gonadotropic axis being affected more frequently
in men (p=0.001). Infundibular thickening (56%) and space
occupying lesions (46%) were typical MRI findings. Pituitary size
was frequently enlarged at presentation (37%) but diminished during
observation (p=0.029). Histopathologically and clinically diagnosed
cases did not differ.
Conclusions The cohort of clinically diagnosed patients did not differ
from our histopathologically diagnosed patients or from published cohorts
with predominantly surgical patients. Thus, diagnosis of primary
hypophysitis using clinical criteria seems feasible.
Key words
autoimmune hypophysitis - hypopituitarism - insufficiencies - diabetes insipidus - autoimmunity
