AI Article Synopsis
- - A 26-year-old Japanese woman with Hermansky-Pudlak syndrome experienced worsened pulmonary fibrosis, high fever, diarrhea, and dyspnea, prompting medical treatment.
- - Imaging revealed new ground-glass opacities in her lungs, and a colon biopsy indicated significant inflammation linked to elevated myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibodies.
- - Treatment with steroid pulse therapy and intravenous cyclophosphamide led to improvement in her symptoms and chest CT results, suggesting the need for immunosuppressant therapy for the related systemic inflammation.
Article 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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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9876727 | PMC |
| http://dx.doi.org/10.2169/internalmedicine.9350-22 | DOI Listing |
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