Pulmonary and Intestinal Involvement in a Patient with Myeloperoxidase-specific Antineutrophil Cytoplasmic Antibody-positive Hermansky-Pudlak Syndrome

A 26-year-old Japanese woman was admitted with a 1-month history of diarrhea, a high fever for a few days, and exacerbation of dyspnea. She was treated with an antifibrotic drug and long-term oxygen therapy for Hermansky-Pudlak syndrome-related pulmonary fibrosis. New ground-glass attenuation appear...

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Detalles Bibliográficos
Autores principales: Tokito, Takatomo, Sakamoto, Noriho, Ishimoto, Hiroshi, Okuno, Daisuke, Miyamura, Takuto, Hara, Atsuko, Kido, Takashi, Yamamoto, Kazuko, Yamaguchi, Hiroyuki, Obase, Yasushi, Akazawa, Yuko, Okamura, Ken, Suzuki, Tamio, Ishimatsu, Yuji, Mukae, Hiroshi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2022
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9876727/
https://www.ncbi.nlm.nih.gov/pubmed/35676038
http://dx.doi.org/10.2169/internalmedicine.9350-22
Descripción
Sumario:A 26-year-old Japanese woman was admitted with a 1-month history of diarrhea, a high fever for a few days, and exacerbation of dyspnea. She was treated with an antifibrotic drug and long-term oxygen therapy for Hermansky-Pudlak syndrome-related pulmonary fibrosis. New ground-glass attenuation appeared on chest computed tomography (CT), and a colon biopsy showed an inflammatory cell accumulation with a high titer of myeloperoxidase (MPO)-specific anti-neutrophil cytoplasmic antibodies (ANCA). Systemic inflammation related to MPO-ANCA titer elevation was suspected. Steroid pulse therapy and intravenous cyclophosphamide improved chest CT findings and diarrhea. Therefore, immunosuppressant treatment should be considered for systemic inflammation related to MPO-ANCA.

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