[PMID]: | 29238021 |
[Au] Autor: | Kiboshi T; Isoda K; Furukawa K; Wakahara T; Otani K; Ueda K; Konma J; Teramura K; Ueno N; Fujiwara H; Shoda T |
[Ad] Endereço: | Department of Rheumatology, Yodogawa Christian Hospital. |
[Ti] Título: | [Granulomatosis with Polyangiitis Complicated with Gastrointestinal Perforation: A Case Report and Review of Literature]. |
[So] Source: | Nihon Rinsho Meneki Gakkai Kaishi;40(5):382-386, 2017. |
[Is] ISSN: | 1349-7413 |
[Cp] País de publicação: | Japan |
[La] Idioma: | jpn |
[Ab] Resumo: | A 51-year-old man was detected nasal bleeding, multiple pulmonary nodule and mass, urinalysis abnormality, renal involvement and high titer of proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and was suspected of granulomatosis with polyangiitis and initiated with steroid pulse therapy. On the day after the start of steroid pulse therapy, generalized peritonitis due to ileal perforation occurred, and emergency ileectomy and peritonitis surgery were performed. Induction therapy with steroid pulse therapy, plasma exchange and intravenous cyclophosphamide therapy (IVCY) and maintenance therapy with glucocorticoid and azathioprine led to good therapeutic outcomes. Gastrointestinal perforation in GPA is a rare complication, and we examined the clinical features, treatment contents, and prognosis of GPA with gastrointestinal perforation from this case and previous reports. Lung involvements were complicated in all reported cases. Gastrointestinal perforations in GPA were frequent in the small intestine, occurred just before and immediately after the start of treatment, and were severe involvement with poor prognosis because of the high mortality rate (46.7%). The frequency of ear, nose and upper respiratory tract lesions in the surviving group was significantly higher than in the dead group (survival 87.5%, death 28.3%, P = 0.041). IVCY were more frequently used in the surviving group (62.5%) than the death group (16.7%), but it was not significantly. GPA complicated with gastrointestinal perforation is a severe condition with poor prognosis, but there is a possibility to improve prognosis by early diagnosis and early initiation of strong treatment. |
[Mh] Termos MeSH primário: |
Granulomatose com Poliangiite/complicações Granulomatose com Poliangiite/terapia Íleo Perfuração Intestinal/etiologia Troca Plasmática
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[Mh] Termos MeSH secundário: |
Anticorpos Anticitoplasma de Neutrófilos/sangue Azatioprina/administração & dosagem Biomarcadores/sangue Ciclofosfamida/administração & dosagem Diagnóstico Precoce Granulomatose com Poliangiite/diagnóstico Seres Humanos Perfuração Intestinal/cirurgia Masculino Metilprednisolona/administração & dosagem Meia-Idade Mieloblastina/imunologia Prognóstico Pulsoterapia
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[Pt] Tipo de publicação: | CASE REPORTS; JOURNAL ARTICLE; REVIEW |
[Nm] Nome de substância:
| 0 (Antibodies, Antineutrophil Cytoplasmic); 0 (Biomarkers); 8N3DW7272P (Cyclophosphamide); EC 3.4.21.76 (Myeloblastin); MRK240IY2L (Azathioprine); X4W7ZR7023 (Methylprednisolone) |
[Em] Mês de entrada: | 1801 |
[Cu] Atualização por classe: | 180112 |
[Lr] Data última revisão:
| 180112 |
[Sb] Subgrupo de revista: | IM |
[Da] Data de entrada para processamento: | 171215 |
[St] Status: | MEDLINE |
[do] DOI: | 10.2177/jsci.40.382 |
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