ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS IN “REAL LIFE” – SERIES OF CLINICAL CASES IN A ROMANIAN REFERENCE CENTER

Introduction. Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) represent a group of conditions evolving with necrotizing inflammation in small and medium-sized blood vessels. AAV are composed of GPA (granulomatosis with polyangiitis, former Wegener’s granulomatosis), MPA (micro...

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Main Authors: Mihaela-Lavinia Popescu, Denisa Predeteanu, Andra Balanescu, F. Berghea, Sanziana Daia, Andreea Borangiu, C.L. Constantinescu, Laura Groseanu, Ruxandra Ionescu, Daniela Opris-Belinski
Format: Article
Language:English
Published: Amaltea Medical Publishing House 2017-12-01
Series:Romanian Journal of Rheumatology
Subjects:
Online Access:https://rjr.com.ro/articles/2017.4/RJR_2017_4_Art-05.pdf
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author Mihaela-Lavinia Popescu
Denisa Predeteanu
Andra Balanescu
F. Berghea
Sanziana Daia
Andreea Borangiu
C.L. Constantinescu
Laura Groseanu
Ruxandra Ionescu
Daniela Opris-Belinski
author_facet Mihaela-Lavinia Popescu
Denisa Predeteanu
Andra Balanescu
F. Berghea
Sanziana Daia
Andreea Borangiu
C.L. Constantinescu
Laura Groseanu
Ruxandra Ionescu
Daniela Opris-Belinski
author_sort Mihaela-Lavinia Popescu
collection DOAJ
description Introduction. Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) represent a group of conditions evolving with necrotizing inflammation in small and medium-sized blood vessels. AAV are composed of GPA (granulomatosis with polyangiitis, former Wegener’s granulomatosis), MPA (microscopic polyangiitis) and EGPA (eosinophilic granulomatosis with polyangiitis, former Churg-Strauss syndrome). AAV receive immunosuppressive therapy associated with a high risk of complications. Objective. The aim of this study was to characterize a single center cohort of AAV patients regarding clinical, biological and therapeutic features. Method. We realized a cross-sectional study by consequently enrolling all the patients registered with AAV diagnosis between 2009 and 2017 in Department of Rheumatology of “Sfânta Maria” Hospital. Demographic, diseaserelated and therapeutic-related parameters were collected. The data was extracted from the clinical files. Results. The study sample included 26 cases, 15 females and 11 males: 20 patients GPA, 4 MPA and 2 cases EGPA. Mean age at the time of diagnosis was around 48 but 12 patients presented delays between age at the onset and age at the time of diagnosis (the mean delay was 2 years). The most frequent clinical manifestation identified where pulmonary, musculoskeletal and renal. 15 patients had a diagnostic biopsy performed. ANCA detection revealed 16 cases of c-ANCA and 7 cases of p-ANCA and 11 patients presented other positive serology. A combination of glucocorticoids and cyclophosphamide was used in most of the cases for remission-induction treatment and the same scheme was used for relapse cases. For maintenance phase a combination of glucocorticoids and azathioprine was preferred. 13 patients (50%) developed treatment related complications. Conclusion. Most of the patients were diagnosed with GPA (20) and the least were diagnosed with EGPA (2). Biopsy was performed in 15 cases and it was mostly nasal. For remission-induction prevailed the combination of glucocorticoids and cyclophosphamide. Most treatment related complications were due to glucocorticoids administration. Osteoporosis was predominant.
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spelling doaj.art-a391372ea1d447e8a950a6d1d806a9592022-12-21T18:29:01ZengAmaltea Medical Publishing HouseRomanian Journal of Rheumatology1843-07912069-60862017-12-0126416416810.37897/RJR.2017.4.5ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS IN “REAL LIFE” – SERIES OF CLINICAL CASES IN A ROMANIAN REFERENCE CENTERMihaela-Lavinia Popescu0Denisa Predeteanu1Andra Balanescu2F. Berghea3Sanziana Daia4Andreea Borangiu5C.L. Constantinescu6Laura Groseanu7Ruxandra Ionescu8Daniela Opris-Belinski9Clinical Department of Rheumatology and Internal Medicine, Sfanta Maria Clinical Hospital, BucharestClinical Department of Rheumatology and Internal Medicine, Sfanta Maria Clinical Hospital, BucharestClinical Department of Rheumatology and Internal Medicine, Sfanta Maria Clinical Hospital, Bucharest; Carol Davila University of Medicine and Pharmacy, BucharestClinical Department of Rheumatology and Internal Medicine, Sfanta Maria Clinical Hospital, Bucharest; Carol Davila University of Medicine and Pharmacy, BucharestClinical Department of Rheumatology and Internal Medicine, Sfanta Maria Clinical Hospital, Bucharest; Carol Davila University of Medicine and Pharmacy, BucharestClinical Department of Rheumatology and Internal Medicine, Sfanta Maria Clinical Hospital, Bucharest; Carol Davila University of Medicine and Pharmacy, BucharestClinical Department of Rheumatology and Internal Medicine, Sfanta Maria Clinical Hospital, Bucharest; Carol Davila University of Medicine and Pharmacy, BucharestClinical Department of Rheumatology and Internal Medicine, Sfanta Maria Clinical Hospital, Bucharest; Carol Davila University of Medicine and Pharmacy, BucharestClinical Department of Rheumatology and Internal Medicine, Sfanta Maria Clinical Hospital, Bucharest; Carol Davila University of Medicine and Pharmacy, BucharestClinical Department of Rheumatology and Internal Medicine, Sfanta Maria Clinical Hospital, Bucharest; Carol Davila University of Medicine and Pharmacy, BucharestIntroduction. Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) represent a group of conditions evolving with necrotizing inflammation in small and medium-sized blood vessels. AAV are composed of GPA (granulomatosis with polyangiitis, former Wegener’s granulomatosis), MPA (microscopic polyangiitis) and EGPA (eosinophilic granulomatosis with polyangiitis, former Churg-Strauss syndrome). AAV receive immunosuppressive therapy associated with a high risk of complications. Objective. The aim of this study was to characterize a single center cohort of AAV patients regarding clinical, biological and therapeutic features. Method. We realized a cross-sectional study by consequently enrolling all the patients registered with AAV diagnosis between 2009 and 2017 in Department of Rheumatology of “Sfânta Maria” Hospital. Demographic, diseaserelated and therapeutic-related parameters were collected. The data was extracted from the clinical files. Results. The study sample included 26 cases, 15 females and 11 males: 20 patients GPA, 4 MPA and 2 cases EGPA. Mean age at the time of diagnosis was around 48 but 12 patients presented delays between age at the onset and age at the time of diagnosis (the mean delay was 2 years). The most frequent clinical manifestation identified where pulmonary, musculoskeletal and renal. 15 patients had a diagnostic biopsy performed. ANCA detection revealed 16 cases of c-ANCA and 7 cases of p-ANCA and 11 patients presented other positive serology. A combination of glucocorticoids and cyclophosphamide was used in most of the cases for remission-induction treatment and the same scheme was used for relapse cases. For maintenance phase a combination of glucocorticoids and azathioprine was preferred. 13 patients (50%) developed treatment related complications. Conclusion. Most of the patients were diagnosed with GPA (20) and the least were diagnosed with EGPA (2). Biopsy was performed in 15 cases and it was mostly nasal. For remission-induction prevailed the combination of glucocorticoids and cyclophosphamide. Most treatment related complications were due to glucocorticoids administration. Osteoporosis was predominant.https://rjr.com.ro/articles/2017.4/RJR_2017_4_Art-05.pdfantineutrophil cytoplasmic antibody (anca)vasculitisglucocorticoids
spellingShingle Mihaela-Lavinia Popescu
Denisa Predeteanu
Andra Balanescu
F. Berghea
Sanziana Daia
Andreea Borangiu
C.L. Constantinescu
Laura Groseanu
Ruxandra Ionescu
Daniela Opris-Belinski
ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS IN “REAL LIFE” – SERIES OF CLINICAL CASES IN A ROMANIAN REFERENCE CENTER
Romanian Journal of Rheumatology
antineutrophil cytoplasmic antibody (anca)
vasculitis
glucocorticoids
title ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS IN “REAL LIFE” – SERIES OF CLINICAL CASES IN A ROMANIAN REFERENCE CENTER
title_full ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS IN “REAL LIFE” – SERIES OF CLINICAL CASES IN A ROMANIAN REFERENCE CENTER
title_fullStr ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS IN “REAL LIFE” – SERIES OF CLINICAL CASES IN A ROMANIAN REFERENCE CENTER
title_full_unstemmed ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS IN “REAL LIFE” – SERIES OF CLINICAL CASES IN A ROMANIAN REFERENCE CENTER
title_short ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS IN “REAL LIFE” – SERIES OF CLINICAL CASES IN A ROMANIAN REFERENCE CENTER
title_sort antineutrophil cytoplasmic antibody associated vasculitis in real life series of clinical cases in a romanian reference center
topic antineutrophil cytoplasmic antibody (anca)
vasculitis
glucocorticoids
url https://rjr.com.ro/articles/2017.4/RJR_2017_4_Art-05.pdf
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