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Original paper
Submitted: 2024-02-20
Accepted: 2024-04-13
Published online: 2024-05-06
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Lymphocytic hypophysitis — various course of the disease and individualized therapeutic approach. An algorithm of the follow-up

Łukasz Kluczyński1, Edyta Tkacz2, Anna Grochowska3, Małgorzata Wójcik4, Grzegorz Zieliński5, Alicja Hubalewska-Dydejczyk1, Aleksadra Gilis-Januszewska1
·
Pubmed: 38708912
Affiliations
  1. Chair and Department of Endocrinology, Jagiellonian University, Medical College, Krakow, Poland
  2. Department of Endocrinology, Oncological Endocrinology, Nuclear Medicine and Internal Medicine, University Hospital, Krakow, Poland
  3. Department of Radiology, University Hospital, Krakow, Poland
  4. Department of Paediatric and Adolescent Endocrinology, Paediatric Institute, Jagiellonian University, Medical College, Krakow, Poland
  5. Department of Neurosurgery, Military Institute of Medicine, Warsaw, Poland

open access

Online first
Original Paper
Submitted: 2024-02-20
Accepted: 2024-04-13
Published online: 2024-05-06

Abstract

Introduction: Lymphocytic hypophysitis (LH) is a rare inflammatory disorder of the pituitary or/and hypothalamus with variable disease course: from spontaneous remission to pituitary atrophy. The diagnosis, treatment and follow-up remain challenging. The aim of the study is to present long-term data and an individualized therapeutic approach and propose an algorithm for the follow-up of patients
with probable LH.

Material and methods: A retrospective analysis of 18 consecutive adult patients (13 W/5 M, mean age 45.2 years) with LH diagnosed and treated in a tertiary referral center.

Results: The first manifestations were headaches (50.0%), polyuria/polydipsia (33.3%) and symptoms of hypopituitarism (16.7%). Somatotropic, adrenal, gonadal and thyroid axis insufficiencies were found in 44.4%, 33.3%, 33.3%, and 27.8% of patients, respectively. Arginine vasopressin deficiency was diagnosed in 8 patients (44.4%). Some of the dysfunctions were transient. Magnetic resonance imaging (MRI) revealed thickened pituitary stalk in all but 2 cases. In 2 patients an anterior pituitary lesion, most likely inflammatory was described. Four patients were given steroids (severe headaches) with clinical recovery and stable/improved MRI. One woman was operated on due to the progressive mass-related symptoms — histopathological examination confirmed LH. In the remaining 13/18 patients watchful waiting approach allowed to obtain hormonal and radiological stabilization/improvement.

Conclusions: LH is a disease with a complex clinical picture and challenging diagnosis. Treatment requires an individual approach: vigilant
observation is the cornerstone of therapy, with steroid/surgical treatment reserved for cases with mass-related symptoms. Further multicenter
research might help in better understanding of the LH and creating standards of care in this rare disease.

Abstract

Introduction: Lymphocytic hypophysitis (LH) is a rare inflammatory disorder of the pituitary or/and hypothalamus with variable disease course: from spontaneous remission to pituitary atrophy. The diagnosis, treatment and follow-up remain challenging. The aim of the study is to present long-term data and an individualized therapeutic approach and propose an algorithm for the follow-up of patients
with probable LH.

Material and methods: A retrospective analysis of 18 consecutive adult patients (13 W/5 M, mean age 45.2 years) with LH diagnosed and treated in a tertiary referral center.

Results: The first manifestations were headaches (50.0%), polyuria/polydipsia (33.3%) and symptoms of hypopituitarism (16.7%). Somatotropic, adrenal, gonadal and thyroid axis insufficiencies were found in 44.4%, 33.3%, 33.3%, and 27.8% of patients, respectively. Arginine vasopressin deficiency was diagnosed in 8 patients (44.4%). Some of the dysfunctions were transient. Magnetic resonance imaging (MRI) revealed thickened pituitary stalk in all but 2 cases. In 2 patients an anterior pituitary lesion, most likely inflammatory was described. Four patients were given steroids (severe headaches) with clinical recovery and stable/improved MRI. One woman was operated on due to the progressive mass-related symptoms — histopathological examination confirmed LH. In the remaining 13/18 patients watchful waiting approach allowed to obtain hormonal and radiological stabilization/improvement.

Conclusions: LH is a disease with a complex clinical picture and challenging diagnosis. Treatment requires an individual approach: vigilant
observation is the cornerstone of therapy, with steroid/surgical treatment reserved for cases with mass-related symptoms. Further multicenter
research might help in better understanding of the LH and creating standards of care in this rare disease.

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Keywords

arginine vasopressin deficiency; hypophysitis; hypopituitarism; stalk enlargement

About this article
Title

Lymphocytic hypophysitis — various course of the disease and individualized therapeutic approach. An algorithm of the follow-up

Journal

Endokrynologia Polska

Issue

Online first

Article type

Original paper

Published online

2024-05-06

Page views

167

Article views/downloads

109

DOI

10.5603/ep.99452

Pubmed

38708912

Keywords

arginine vasopressin deficiency
hypophysitis
hypopituitarism
stalk enlargement

Authors

Łukasz Kluczyński
Edyta Tkacz
Anna Grochowska
Małgorzata Wójcik
Grzegorz Zieliński
Alicja Hubalewska-Dydejczyk
Aleksadra Gilis-Januszewska

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