Successful Sirolimus Treatment of Lymphangioleiomyomatosis in a Hepatitis B Virus Carrier

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...

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Detalles Bibliográficos
Autores principales: Sonobe, Shoko, Arai, Toru, Tanimoto, Yasushi, Sugimoto, Chikatoshi, Kitaichi, Masanori, Akira, Masanori, Kasai, Takahiko, Hirose, Masaki, Inoue, Yoshikazu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2018
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6421154/
https://www.ncbi.nlm.nih.gov/pubmed/30333386
http://dx.doi.org/10.2169/internalmedicine.1329-18
Descripción
Sumario: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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