Clinical impact of proteinase 3-antineutrophil cytoplasmic antibody positivity in eosinophilic granulomatosis with polyangiitis
Background/Aims This study investigated the clinical implication of proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA) in Korean patients with eosinophilic granulomatosis with polyangiitis (EGPA). Methods Among the 242 patients with ANCA-associated vasculitis identified from the hospital...
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The Korean Association of Internal Medicine
2022-11-01
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Series: | The Korean Journal of Internal Medicine |
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Online Access: | http://kjim.org/upload/pdf/kjim-2021-043.pdf |
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author | Sung Soo Ahn Jason Jungsik Song Yong-Beom Park Sang-Won Lee |
author_facet | Sung Soo Ahn Jason Jungsik Song Yong-Beom Park Sang-Won Lee |
author_sort | Sung Soo Ahn |
collection | DOAJ |
description | Background/Aims This study investigated the clinical implication of proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA) in Korean patients with eosinophilic granulomatosis with polyangiitis (EGPA). Methods Among the 242 patients with ANCA-associated vasculitis identified from the hospital database, 49 patients with EGPA were selected and analysed in this study. Demographic, clinical, and laboratory data at diagnosis were reviewed to compare the features of patients with PR3-ANCA and without, as well as the clinical outcomes of relapse and end-stage renal disease (ESRD) during the follow-up period. The outcomes of patients with PR3-ANCA and without were compared by using the Kaplan-Meier survival analysis. Results The median age of the patients was 54 years, 17 (34.7%) were male, and six (12.2%) patients had PR3-ANCA at baseline. The most common items of the 1990 American College of Rheumatology criteria for EGPA were sinusitis (95.9%) and asthma (or asthmatic history) (93.9%). During the follow-up, none died, eight experienced relapse and two progressed to ESRD. EGPA patients with PR3-ANCA exhibited peripheral eosinophilia less frequently than those without (50.0% vs. 88.4%, p = 0.047). On the other hand, EGPA patients with PR3-ANCA experienced relapse more often compared to those without (50.0% vs. 11.6%, p = 0.047), and the cumulative relapse-free survival rate was lower compared to those without PR3-ANCA (p = 0.012). Conclusions EGPA patients possessing PR3-ANCA at disease diagnosis had distinct clinical feature and outcome compared to those without PR3-ANCA. These results should be taken into account in the management of patients with EGPA. |
first_indexed | 2024-04-11T13:51:41Z |
format | Article |
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issn | 1226-3303 2005-6648 |
language | English |
last_indexed | 2024-04-11T13:51:41Z |
publishDate | 2022-11-01 |
publisher | The Korean Association of Internal Medicine |
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series | The Korean Journal of Internal Medicine |
spelling | doaj.art-987eda7c60d740b59ad12ab185833e742022-12-22T04:20:33ZengThe Korean Association of Internal MedicineThe Korean Journal of Internal Medicine1226-33032005-66482022-11-013761241124910.3904/kjim.2021.043170566Clinical impact of proteinase 3-antineutrophil cytoplasmic antibody positivity in eosinophilic granulomatosis with polyangiitisSung Soo Ahn0Jason Jungsik Song1Yong-Beom Park2Sang-Won Lee3 Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, KoreaBackground/Aims This study investigated the clinical implication of proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA) in Korean patients with eosinophilic granulomatosis with polyangiitis (EGPA). Methods Among the 242 patients with ANCA-associated vasculitis identified from the hospital database, 49 patients with EGPA were selected and analysed in this study. Demographic, clinical, and laboratory data at diagnosis were reviewed to compare the features of patients with PR3-ANCA and without, as well as the clinical outcomes of relapse and end-stage renal disease (ESRD) during the follow-up period. The outcomes of patients with PR3-ANCA and without were compared by using the Kaplan-Meier survival analysis. Results The median age of the patients was 54 years, 17 (34.7%) were male, and six (12.2%) patients had PR3-ANCA at baseline. The most common items of the 1990 American College of Rheumatology criteria for EGPA were sinusitis (95.9%) and asthma (or asthmatic history) (93.9%). During the follow-up, none died, eight experienced relapse and two progressed to ESRD. EGPA patients with PR3-ANCA exhibited peripheral eosinophilia less frequently than those without (50.0% vs. 88.4%, p = 0.047). On the other hand, EGPA patients with PR3-ANCA experienced relapse more often compared to those without (50.0% vs. 11.6%, p = 0.047), and the cumulative relapse-free survival rate was lower compared to those without PR3-ANCA (p = 0.012). Conclusions EGPA patients possessing PR3-ANCA at disease diagnosis had distinct clinical feature and outcome compared to those without PR3-ANCA. These results should be taken into account in the management of patients with EGPA.http://kjim.org/upload/pdf/kjim-2021-043.pdfeosinophilic granulomatous vasculitispr3anti-neutrophil cytoplasmic antibodyprognosisrecurrence |
spellingShingle | Sung Soo Ahn Jason Jungsik Song Yong-Beom Park Sang-Won Lee Clinical impact of proteinase 3-antineutrophil cytoplasmic antibody positivity in eosinophilic granulomatosis with polyangiitis The Korean Journal of Internal Medicine eosinophilic granulomatous vasculitis pr3 anti-neutrophil cytoplasmic antibody prognosis recurrence |
title | Clinical impact of proteinase 3-antineutrophil cytoplasmic antibody positivity in eosinophilic granulomatosis with polyangiitis |
title_full | Clinical impact of proteinase 3-antineutrophil cytoplasmic antibody positivity in eosinophilic granulomatosis with polyangiitis |
title_fullStr | Clinical impact of proteinase 3-antineutrophil cytoplasmic antibody positivity in eosinophilic granulomatosis with polyangiitis |
title_full_unstemmed | Clinical impact of proteinase 3-antineutrophil cytoplasmic antibody positivity in eosinophilic granulomatosis with polyangiitis |
title_short | Clinical impact of proteinase 3-antineutrophil cytoplasmic antibody positivity in eosinophilic granulomatosis with polyangiitis |
title_sort | clinical impact of proteinase 3 antineutrophil cytoplasmic antibody positivity in eosinophilic granulomatosis with polyangiitis |
topic | eosinophilic granulomatous vasculitis pr3 anti-neutrophil cytoplasmic antibody prognosis recurrence |
url | http://kjim.org/upload/pdf/kjim-2021-043.pdf |
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