MDA-5 associated rapidly progressive interstitial lung disease with recurrent Pneumothoraces: a case report

Show simple item record

dc.contributor.author Alqatari, Safi
dc.contributor.author Riddell, Peter
dc.contributor.author Harney, Sinead
dc.contributor.author Henry, Michael T.
dc.contributor.author Murphy, Grainne
dc.date.accessioned 2018-05-31T11:56:26Z
dc.date.available 2018-05-31T11:56:26Z
dc.identifier.citation Alqatari, S., Riddell, P., Harney, S., Henry, M. and Murphy, G. (2018) 'MDA-5 associated rapidly progressive interstitial lung disease with recurrent Pneumothoraces: a case report', BMC Pulmonary Medicine, 18, 59 (5pp). doi: 10.1186/s12890-018-0622-8 en
dc.identifier.volume 18
dc.identifier.issued 2018
dc.identifier.startpage 1
dc.identifier.endpage 5
dc.identifier.issn 1471-2466
dc.identifier.uri http://hdl.handle.net/10468/6215
dc.identifier.doi 10.1186/s12890-018-0622-8
dc.description.abstract Background: Clinically hypomyopathic dermatomyositis is a rare disease that is important to recognize, investigate and treat early as it is associated with poor prognosis. In a proportion of patients, myositis specific antibodies could be negative, but with high clinical suspicion, myositis associated antibodies should be ordered. Anti-MDA-5 antibodies was reported in literature to be associated with severe and rapidly progressive interstitial lung disease, with few case reports of pneumothorax and/or pneumomediastinum. Case presentation: A 49-year-old previously healthy lady, presented with a 6 week history of skin rash, photosensitivity, mouth ulcers, fatiguability, arthralgia and myalgia. She denied subjective weakness, respiratory symptoms or dysphagia. She had Raynaud's phenomenon affecting her fingers only. Initial examination showed synovitis in her hands with skin rash. Autoimmune screen was negative. She was started on hydroxychloroquine. 4 weeks later on follow-up, she developed proximal muscle pain, dysphagia, dyspnea and dry cough. Examination showed mild proximal muscle weakness and bi-basal crackles. She was admitted and extended myositis screen was sent. She had mild anemia, lymphopenia and neutropenia, normal inflammatory markers, liver and renal panels. Capillaroscopy showed pattern of systemic sclerosis. CT chest showed early ILD. Electromyography and MRI showed features of mild myositis. PFT showed muscle weakness with low DLCO. She was given intravenous steroid and Rituximab. As she continued to deteriorate, intravenous immunoglobulins and cyclophosphamide were given. There was a brief clinical response that was short-lived with increasing oxygen dependency necessitating transfer to the ICU. At this point, the extended myositis screen confirmed the presence of anti-MDA-5 antibodies. She commenced plasmapharesis and required intubation. Unfortunately, she developed multiple pneumothoraces, and was transferred urgently for ECMO. Subsequent immunosuppression included rituximab and tacrolimus. There was progression of her ILD and recurrent pneumothoraces and pneumomediastinum. Unfortunately, she passed away as a consequence of her disease. Conclusion: This case highlights a number of considerations in approaching patients with inflammatory myositis, particularly to pulmonary involvement. It is important to highlight the utility of extended myositis antibody testing in predicting disease phenotypes and its impact on therapeutic decisions. From a management perspective, aggressive immunosuppression should be considered with potential need of earlier utilization of ECMO. en
dc.format.mimetype application/pdf en
dc.language.iso en en
dc.publisher BioMed Central Ltd en
dc.relation.uri https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-018-0622-8
dc.rights © 2018, the Author(s). Open Access. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. en
dc.rights.uri http://creativecommons.org/licenses/by/4.0/
dc.subject Myositis en
dc.subject Interstitial lung disease en
dc.subject MDA-5 en
dc.title MDA-5 associated rapidly progressive interstitial lung disease with recurrent Pneumothoraces: a case report en
dc.type Article (peer-reviewed) en
dc.internal.authorcontactother Michael Henry, Medicine, University College Cork, Cork, Ireland. +353-21-490-3000 Email: michael.henry@ucc.ie en
dc.internal.availability Full text available en
dc.description.version Published Version en
dc.description.status Peer reviewed en
dc.identifier.journaltitle BMC Pulmonary Medicine en
dc.internal.IRISemailaddress michael.henry@ucc.ie en
dc.identifier.articleid 59


Files in this item

This item appears in the following Collection(s)

Show simple item record

© 2018, the Author(s). Open Access. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Except where otherwise noted, this item's license is described as © 2018, the Author(s). Open Access. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
This website uses cookies. By using this website, you consent to the use of cookies in accordance with the UCC Privacy and Cookies Statement. For more information about cookies and how you can disable them, visit our Privacy and Cookies statement