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

Takatomo Tokito; Noriho Sakamoto; Hiroshi Ishimoto; Daisuke Okuno; Takuto Miyamura; Atsuko Hara; Takashi Kido; Kazuko Yamamoto; Hiroyuki Yamaguchi; Yasushi Obase; Yuko Akazawa; Ken Okamura; Tamio Suzuki; Yuji Ishimatsu; Hiroshi Mukae

doi:10.2169/internalmedicine.9350-22

CASE REPORTS

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

Takatomo Tokito, Noriho Sakamoto, Hiroshi Ishimoto, Daisuke Okuno, Takuto Miyamura, Atsuko Hara, Takashi Kido, Kazuko Yamamoto, Hiroyuki Yamaguchi, Yasushi Obase, Yuko Akazawa, Ken Okamura, Tamio Suzuki, Yuji Ishimatsu, Hiroshi Mukae

Author information

Takatomo Tokito
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Noriho Sakamoto
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Hiroshi Ishimoto
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Daisuke Okuno
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Takuto Miyamura
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Atsuko Hara
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Takashi Kido
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Kazuko Yamamoto
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Hiroyuki Yamaguchi
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Yasushi Obase
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Yuko Akazawa
Tissue and Histopathology Section, Atomic Bomb Disease Institute, Nagasaki University, Japan
Ken Okamura
Department of Dermatology, Faculty of Medicine, Yamagata University, Japan
Tamio Suzuki
Department of Dermatology, Faculty of Medicine, Yamagata University, Japan
Yuji Ishimatsu
Department of Nursing, Nagasaki University Graduate School of Biomedical Sciences, Japan
Hiroshi Mukae
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan

Keywords: Hermansky-Pudlak syndrome, pulmonary fibrosis, MPO-ANCA, granulomatous colitis, steroid pulse treatment, vasculitis

JOURNAL OPEN ACCESS

2023 Volume 62 Issue 1 Pages 103-106

DOI https://doi.org/10.2169/internalmedicine.9350-22

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Abstract

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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