Idiopathic pulmonary fibrosis and p-ANCA positivity predating manifestations of systemic vasculitis

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dc.contributor.authorKhan, Kashif Ali
dc.contributor.authorFitzgerald, Deirdre
dc.contributor.authorHarrison, Mike
dc.contributor.authorMaher, Michael M.
dc.contributor.authorHenry, Michael T.
dc.contributor.authorKennedy, Marcus P.
dc.contributor.authorPlant, Barry J.
dc.contributor.authorMurphy, Desmond
dc.date.accessioned2022-02-04T09:29:28Z
dc.date.available2022-02-04T09:29:28Z
dc.date.issued2015
dc.date.updated2022-02-03T15:54:53Z
dc.description.abstractThe occurrence of perinuclear anti-neutrophilic cytoplasmic antibodies (p-ANCA) in pulmonary fibrosis has been reported in a number of small studies, however, the appearance of p-ANCA in already established idiopathic pulmonary fibrosis (IPF), predating the manifestations of vasculitis has not been widely appreciated. We report 2 cases with radiographic evidence of established IPF where p-ANCA was negative at the time of diagnosis and became positive during the course of the disease, for a variable period of time, prior to the subsequent development of systemic vasculitis. Our cases suggest a possible rationale for serial p-ANCA measurements in patients with pulmonary fibrosis even in the absence of features suggestive of vasculitis. Furthermore, the required frequency of ANCA measurement in the absence of clinical features of vasculitis is unknown. Larger studies with repetitive p-ANCA measurement in tandem with ongoing clinical assessment for vasculitis are required to assess the prevalence of ANCA and to confirm our observation.en
dc.description.statusPeer revieweden
dc.description.versionPublished Versionen
dc.format.mimetypeapplication/pdfen
dc.identifier.articleidArticle 1en
dc.identifier.citationKhan, K. A., Fitzgerald, D., Harrison, M., Maher, M. M., Henry, M. T., Kennedy, M. P., Plant, B. J. and Murphy, D. (2015) 'Idiopathic pulmonary fibrosis and p-ANCA positivity predating manifestations of systemic vasculitis', Pulmonology and Respiratory Research, 3, Article 1 (4pp). doi: 10.7243/2053-6739-3-1en
dc.identifier.doi10.7243/2053-6739-3-1en
dc.identifier.endpage4en
dc.identifier.issn2053-6739
dc.identifier.journaltitlePulmonology and Respiratory Researchen
dc.identifier.startpage1en
dc.identifier.urihttps://hdl.handle.net/10468/12527
dc.identifier.volume3en
dc.language.isoenen
dc.publisherHerbert Publications Limited.en
dc.rights© 2015, Khan et al; licensee Herbert Publications Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.en
dc.rights.urihttps://creativecommons.org/licenses/by/4.0/en
dc.subjectPulmonary fibrosisen
dc.subjectVasculitisen
dc.subjectANCAen
dc.subjectMicroscopic polyangiitisen
dc.titleIdiopathic pulmonary fibrosis and p-ANCA positivity predating manifestations of systemic vasculitisen
dc.typeArticle (peer-reviewed)en
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