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.
Key words
hypophysitis - primary hypophysitis - hypopituitarism - immunoglobulin G4 - pituitary adenoma - apoplexy
