Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study
Perinuclear Anti Neutrophil Cytoplasmic Antibody (p-ANCA) is a serological marker of Microscopic Polyangiitis (MPA), a vasculitis associated with lung involvement potentially mimicking Idiopathic Pulmonary Fibrosis (IPF). In this study, we evaluated the role of p-ANCA in predicting clinical evolutio...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-05-01
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| Series: | Diagnostics |
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| Online Access: | https://www.mdpi.com/2075-4418/13/11/1882 |
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| author | Alessandro Libra Giuseppe Muscato Giuseppe Ielo Lucia Spicuzza Stefano Palmucci Evelina Fagone Mary Fruciano Elisa Gili Gianluca Sambataro Carlo Vancheri |
| author_facet | Alessandro Libra Giuseppe Muscato Giuseppe Ielo Lucia Spicuzza Stefano Palmucci Evelina Fagone Mary Fruciano Elisa Gili Gianluca Sambataro Carlo Vancheri |
| author_sort | Alessandro Libra |
| collection | DOAJ |
| description | Perinuclear Anti Neutrophil Cytoplasmic Antibody (p-ANCA) is a serological marker of Microscopic Polyangiitis (MPA), a vasculitis associated with lung involvement potentially mimicking Idiopathic Pulmonary Fibrosis (IPF). In this study, we evaluated the role of p-ANCA in predicting clinical evolution and prognosis in a cohort of IPF patients. In this observational, retrospective, case–control study, we compared 18 patients with an IPF diagnosis and p-ANCA positivity with 36 patients with seronegative IPF, matched for age and sex. IPF patients with and without p-ANCA showed similar lung function decline during the follow-up, but IPF p-ANCA+ showed better survival. Half of IPF p-ANCA+ patients were classified as MPA for the development of renal involvement (55%) or skin signs (45%). The progression towards MPA was associated with high levels of Rheumatoid Factor (RF) at baseline. In conclusion, p-ANCA, mainly when associated with RF, could predict the evolution of Usual Interstitial Pneumonia (UIP) towards a definite vasculitis in patients, with a better prognosis compared with IPF. In this view, ANCA testing should be included in the diagnostic workup of UIP patients. |
| first_indexed | 2024-03-11T03:09:44Z |
| format | Article |
| id | doaj.art-a115b985aa6e4b43ade1b05d507d03e5 |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-11T03:09:44Z |
| publishDate | 2023-05-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-a115b985aa6e4b43ade1b05d507d03e52023-11-18T07:42:24ZengMDPI AGDiagnostics2075-44182023-05-011311188210.3390/diagnostics13111882Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational StudyAlessandro Libra0Giuseppe Muscato1Giuseppe Ielo2Lucia Spicuzza3Stefano Palmucci4Evelina Fagone5Mary Fruciano6Elisa Gili7Gianluca Sambataro8Carlo Vancheri9Regional Referral Centre for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital “Policlinico-San Marco”, University of Catania, 95123 Catania, ItalyRegional Referral Centre for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital “Policlinico-San Marco”, University of Catania, 95123 Catania, ItalyRegional Referral Centre for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital “Policlinico-San Marco”, University of Catania, 95123 Catania, ItalyRegional Referral Centre for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital “Policlinico-San Marco”, University of Catania, 95123 Catania, ItalyRadiology Unit I, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital “Policlinico-San Marco”, University of Catania, 95123 Catania, ItalyRegional Referral Centre for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital “Policlinico-San Marco”, University of Catania, 95123 Catania, ItalyRegional Referral Centre for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital “Policlinico-San Marco”, University of Catania, 95123 Catania, ItalyRegional Referral Centre for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital “Policlinico-San Marco”, University of Catania, 95123 Catania, ItalyRegional Referral Centre for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital “Policlinico-San Marco”, University of Catania, 95123 Catania, ItalyRegional Referral Centre for Rare Lung Disease, Department of Clinical and Experimental Medicine, University Hospital “Policlinico-San Marco”, University of Catania, 95123 Catania, ItalyPerinuclear Anti Neutrophil Cytoplasmic Antibody (p-ANCA) is a serological marker of Microscopic Polyangiitis (MPA), a vasculitis associated with lung involvement potentially mimicking Idiopathic Pulmonary Fibrosis (IPF). In this study, we evaluated the role of p-ANCA in predicting clinical evolution and prognosis in a cohort of IPF patients. In this observational, retrospective, case–control study, we compared 18 patients with an IPF diagnosis and p-ANCA positivity with 36 patients with seronegative IPF, matched for age and sex. IPF patients with and without p-ANCA showed similar lung function decline during the follow-up, but IPF p-ANCA+ showed better survival. Half of IPF p-ANCA+ patients were classified as MPA for the development of renal involvement (55%) or skin signs (45%). The progression towards MPA was associated with high levels of Rheumatoid Factor (RF) at baseline. In conclusion, p-ANCA, mainly when associated with RF, could predict the evolution of Usual Interstitial Pneumonia (UIP) towards a definite vasculitis in patients, with a better prognosis compared with IPF. In this view, ANCA testing should be included in the diagnostic workup of UIP patients.https://www.mdpi.com/2075-4418/13/11/1882idiopathic pulmonary fibrosisANCAvasculitisMicroscopic PolyangiitisRheumatoid FactorUIP pattern |
| spellingShingle | Alessandro Libra Giuseppe Muscato Giuseppe Ielo Lucia Spicuzza Stefano Palmucci Evelina Fagone Mary Fruciano Elisa Gili Gianluca Sambataro Carlo Vancheri Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study Diagnostics idiopathic pulmonary fibrosis ANCA vasculitis Microscopic Polyangiitis Rheumatoid Factor UIP pattern |
| title | Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study |
| title_full | Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study |
| title_fullStr | Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study |
| title_full_unstemmed | Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study |
| title_short | Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study |
| title_sort | clinical and prognostic significance of p anca positivity in idiopathic pulmonary fibrosis a retrospective observational study |
| topic | idiopathic pulmonary fibrosis ANCA vasculitis Microscopic Polyangiitis Rheumatoid Factor UIP pattern |
| url | https://www.mdpi.com/2075-4418/13/11/1882 |
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