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Erschienen in: Pituitary 2/2022

28.01.2022

Lymphocytic panhypophysitis and anti-rabphilin-3A antibody with pulmonary sarcoidosis

verfasst von: Yuka Takahashi, Hiraku Kameda, Aika Miya, Hiroshi Nomoto, Kyu Yong Cho, Akinobu Nakamura, Hiroki Nishimura, Hirokazu Kimura, Masaru Suzuki, Satoshi Konno, Ai Shimizu, Yoshihiro Matsuno, Michinari Okamoto, Hiroaki Motegi, Naoko Iwata, Haruki Fujisawa, Atsushi Suzuki, Yoshihisa Sugimura, Hideaki Miyoshi, Tatsuya Atsumi

Erschienen in: Pituitary | Ausgabe 2/2022

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Abstract

Purpose

To explore the clinical significance of anti-rabphillin-3A antibody for the differential diagnosis of lymphocytic panhypophysitis.

Methods and results

A 58-year-old Japanese man developed uveitis of unknown cause in 2017. In 2019, he became aware of polyuria. In August 2020, he noticed transient diplopia and was diagnosed with right abducens nerve palsy. At the same time, he complained of fatigue and loss of appetite. Head magnetic resonance imaging demonstrated enlargement of the pituitary stalk and pituitary gland, corresponding to hypophysitis. Hormone stimulation tests showed blunted responses with respect to all anterior pituitary hormones. Central diabetes insipidus was diagnosed on the basis of a hypertonic saline loading test. Taking these findings together, a diagnosis of panhypopituitarism was made. Computed tomography showed enlargement of hilar lymph nodes. Biopsies of the hilar lymph nodes revealed non-caseating epithelioid cell granulomas that were consistent with sarcoidosis. Biopsy of the anterior pituitary revealed mild lymphocyte infiltration in the absence of IgG4-positive cells, non-caseating granulomas, or neoplasia. Western blotting revealed the presence of anti-rabphilin-3A antibody, supporting a diagnosis of lymphocytic panhypophysitis. Because the patient had no visual impairment or severe uveitis, we continued physiological hormone replacement therapy and topical steroid therapy for the uveitis.

Conclusion

To the best of our knowledge, this is the first case of anti-rabphilin 3A antibody positive lymphocytic panhypophysitis comorbid with sarcoidosis, diagnosed by both pituitary and hilar lymph node biopsy. The utility of anti-rabphilin-3A antibody for the differential diagnosis of hypophysitis like this case should be clarified with further case studies.
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Metadaten
Titel
Lymphocytic panhypophysitis and anti-rabphilin-3A antibody with pulmonary sarcoidosis
verfasst von
Yuka Takahashi
Hiraku Kameda
Aika Miya
Hiroshi Nomoto
Kyu Yong Cho
Akinobu Nakamura
Hiroki Nishimura
Hirokazu Kimura
Masaru Suzuki
Satoshi Konno
Ai Shimizu
Yoshihiro Matsuno
Michinari Okamoto
Hiroaki Motegi
Naoko Iwata
Haruki Fujisawa
Atsushi Suzuki
Yoshihisa Sugimura
Hideaki Miyoshi
Tatsuya Atsumi
Publikationsdatum
28.01.2022
Verlag
Springer US
Erschienen in
Pituitary / Ausgabe 2/2022
Print ISSN: 1386-341X
Elektronische ISSN: 1573-7403
DOI
https://doi.org/10.1007/s11102-021-01200-0

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