Successful Sirolimus Treatment of Lymphangioleiomyomatosis in a Hepatitis B Virus Carrier

Shoko Sonobe; Toru Arai; Yasushi Tanimoto; Chikatoshi Sugimoto; Masanori Kitaichi; Masanori Akira; Takahiko Kasai; Masaki Hirose; Yoshikazu Inoue

doi:10.2169/internalmedicine.1329-18

CASE REPORTS

Successful Sirolimus Treatment of Lymphangioleiomyomatosis in a Hepatitis B Virus Carrier

Shoko Sonobe, Toru Arai, Yasushi Tanimoto, Chikatoshi Sugimoto, Masanori Kitaichi, Masanori Akira, Takahiko Kasai, Masaki Hirose, Yoshikazu Inoue

Author information

Shoko Sonobe
Department of Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Toru Arai
Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Yasushi Tanimoto
Department of Allergy and Respiratory Medicine, National Hospital Organization Minami-Okayama Medical Center, Japan
Chikatoshi Sugimoto
Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Masanori Kitaichi
Department of Pathology, National Hospital Organization Minami Wakayama Medical Center, Japan
Masanori Akira
Department of Radiology, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Takahiko Kasai
Department of Pathology, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Masaki Hirose
Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Yoshikazu Inoue
Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan

Keywords: lymphangioleiomyomatosis, lymphangioleiomyomas, hepatitis B virus, sirolimus, entecavir

JOURNAL OPEN ACCESS

2019 Volume 58 Issue 4 Pages 569-574

DOI https://doi.org/10.2169/internalmedicine.1329-18

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Abstract

A 34-year-old woman experiencing shortness of breath was referred to our hospital. The patient was diagnosed with sporadic lymphangioleiomyomatosis based on the observation of bilateral diffuse multiple thin-walled cysts on computed tomography of the chest, chylous effusion, elevated serum vascular endothelial growth factor-D levels and transbronchial biopsy findings. This patient was a hepatitis B virus (HBV) carrier. Treatment with 1 mg daily of sirolimus was started after HBV DNA was brought below the cut-off level using entecavir. Sirolimus was effective, as the chylous effusion resolved completely and the dyspnea improved. The sirolimus dosage was increased to 2 mg daily without causing HBV reactivation.

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