Pulmonary and Intestinal Involvement in a Patient with Myeloperoxidase-specific Antineutrophil Cytoplasmic Antibody-positive Hermansky-Pudlak Syndrome |
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| Authors: | 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 |
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| Affiliation: | 1.Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan; 2.Tissue and Histopathology Section, Atomic Bomb Disease Institute, Nagasaki University, Japan; 3.Department of Dermatology, Faculty of Medicine, Yamagata University, Japan; 4.Department of Nursing, Nagasaki University Graduate School of Biomedical Sciences, Japan |
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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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| Keywords: | Hermansky-Pudlak syndrome pulmonary fibrosis MPO-ANCA granulomatous colitis steroid pulse treatment vasculitis |
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