Pulmonary and Intestinal Involvement in a Patient with Myeloperoxidase-specific Antineutrophil Cytoplasmic Antibody-positive Hermansky-Pudlak Syndrome.
Hermansky-Pudlak syndrome
MPO-ANCA
granulomatous colitis
pulmonary fibrosis
steroid pulse treatment
vasculitis
Journal
Internal medicine (Tokyo, Japan)
ISSN: 1349-7235
Titre abrégé: Intern Med
Pays: Japan
ID NLM: 9204241
Informations de publication
Date de publication:
01 Jan 2023
01 Jan 2023
Historique:
pubmed:
9
6
2022
medline:
6
1
2023
entrez:
8
6
2022
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 35676038
doi: 10.2169/internalmedicine.9350-22
pmc: PMC9876727
doi:
Substances chimiques
Antibodies, Antineutrophil Cytoplasmic
0
Peroxidase
EC 1.11.1.7
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
103-106Références
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