Two children with lymphocytic hypophysitis presenting with positive anti-rabphilin-3A antibody

Lymphocytic hypophysitis (LYH) is a rare chronic inflammatory disease characterized by lymphocytic infiltration of the anterior or posterior pituitary gland and hypothalamus. LYH is subdivided into lymphocytic adenohypophysitis (LAH), lymphocytic infundibulo-neurohypophysitis (LINH), and lymphocytic...

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Published in:	Endocrine Journal Vol. 70; no. 7; pp. 703 - 709
Main Authors:	Murai, An, Shinojima, Naoki, Ikuta, Genki, Ozono, Kazutaka, Ueda, Yutaka, Mabe, Hiroyo, Nakamura, Kenji, Iwata, Naoko, Fujisawa, Haruki, Nagamatsu, Fusa, Komatsu, Nagisa, Uekawa, Ken, Nishikawa, Shigeyuki, Nakamura, Kimitoshi, Mikami, Yoshiki, Suzuki, Atsushi, Sugimura, Yoshihisa, Mukasa, Akitake
Format:	Journal Article
Language:	English
Published:	Japan The Japan Endocrine Society 01-01-2023 Japan Science and Technology Agency
Subjects:	Anti-rabphilin-3A antibody Antibodies Biopsy Children Diagnosis Hypothalamus Inflammatory diseases Invasiveness Lymphocytic hypophysitis Malignancy Metastases Pediatrics Pituitary (anterior) Pituitary (posterior) Anti-rabphilin-3A antibody Pediatrics Lymphocytic hypophysitis
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Summary:	Lymphocytic hypophysitis (LYH) is a rare chronic inflammatory disease characterized by lymphocytic infiltration of the anterior or posterior pituitary gland and hypothalamus. LYH is subdivided into lymphocytic adenohypophysitis (LAH), lymphocytic infundibulo-neurohypophysitis (LINH), and lymphocytic panhypophysitis (LPH) depending on the primary site. Most cases occur in adults, with few cases reported in children, and it is especially important to distinguish LYH from suprasellar malignancies, such as germ cell tumors and other neoplastic diseases. Although a biopsy is necessary for definitive diagnosis, it is desirable to be able to diagnose the disease without biopsy if possible, especially in children, because of the surgical invasiveness of the procedure. Recently, serum anti-rabphilin-3A antibodies have attracted attention as diagnostic markers for LYH, especially in LINH, but there are only a few reports on pediatric patients. In the present study, we experienced two children with LPH and LAH, respectively, who tested positive for anti-rabphilin-3A antibodies. This is the first report of children with LYH other than LINH positive for anti-rabphilin-3A antibodies, and anti-rabphilin-3A antibodies may be a useful non-invasive diagnostic marker not only for LINH but also for LYH in general. We also discuss the sensitivity and specificity of anti-rabphilin-3A antibody testing in cases where histological diagnosis has been made.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0918-8959 1348-4540
DOI:	10.1507/endocrj.EJ22-0637