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Lymphocytic panhypophysitis: its clinical features in Japanese cases
Yoshiharu Wada1, Yoshiyuki Hamamoto, Yoshio Nakamura
1Center for Diabetes and Endocrinology, The Tazuke Kofukai Foundation Medical Institute Kitano Hospital, Osaka, Japan.
Japanese Clinical Medicine
|July 26, 2013
Summary
Lymphocytic panhypophysitis (LPH) is a controversial pituitary disorder. This study reports five female cases, highlighting its distinct clinical and imaging features, and diffuse lymphocytic infiltration on biopsy.
Area of Science:
- Endocrinology
- Immunology
- Neurology
Background:
- Lymphocytic hypophysitis presents in three forms: adenohypophysitis, infundibulo-neurohypophysitis, and panhypophysitis (LPH).
- The entity and pathogenesis of LPH remain debated.
- This study presents five cases of LPH diagnosed between 1994 and 2009.
Observation:
- The five LPH cases were female, aged 20-77 years, with one pregnancy-associated case.
- Presenting symptoms included polyuria, headache, malaise, polydipsia, visual disturbances, and amenorrhea.
- Magnetic resonance imaging revealed pituitary swelling, thickened stalk, loss of neurohypophysis T1 signal, or pituitary atrophy.
Findings:
- Endocrinological evaluation showed universal deficiencies in TSH, ADH, and variable deficiencies in GH, ACTH, LH, PRL, and FSH.
- Diabetes insipidus severity varied among patients.
- Biopsies, except for one case, confirmed diffuse lymphocytic infiltration, suggesting LPH is an autoimmune pituitary disorder.
Implications:
- LPH exhibits female predominance and unique patterns of anterior pituitary hormone deficiencies.
- The variable degree of diabetes insipidus in LPH requires careful management.
- Further research is needed to elucidate the pathogenesis and establish diagnostic criteria for LPH.
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