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Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome
We describe the first case of antisynthetase syndrome (ASS) with antibodies to anti‐glycyl tRNA synthetase (EJ‐1) with antiphospholipid syndrome (APLS). A 66‐year‐old man presented with progressive dyspnoea, fever, dry cough and proximal muscle weakness over several months on a background of cryptog...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley & Sons, Ltd
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9194464/ https://www.ncbi.nlm.nih.gov/pubmed/35734553 http://dx.doi.org/10.1002/rcr2.990 |
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author | Chhor, Louis Tu, Steven Fogarty, Paul Fong, Christopher |
author_facet | Chhor, Louis Tu, Steven Fogarty, Paul Fong, Christopher |
author_sort | Chhor, Louis |
collection | PubMed |
description | We describe the first case of antisynthetase syndrome (ASS) with antibodies to anti‐glycyl tRNA synthetase (EJ‐1) with antiphospholipid syndrome (APLS). A 66‐year‐old man presented with progressive dyspnoea, fever, dry cough and proximal muscle weakness over several months on a background of cryptogenic organizing pneumonia. Examination revealed bibasal fine chest crackles, proximal muscle weakness of the upper and lower limbs, digital skin thickening and facial telangiectasias. Creatine kinase was elevated and autoimmune screening was positive for anti‐EJ‐1, anti‐beta‐2‐glycoprotein, anti‐Ro and anti‐La antibodies. Computed tomography of the chest revealed a usual interstitial pneumonia pattern and a ventilation–perfusion scan demonstrated scintigraphic evidence of bilateral pulmonary emboli. A diagnosis of ASS and APLS was made. Immunosuppressive therapy including pulsed methylprednisolone, rituximab and mycophenolate was commenced with improvement in symptoms. This case highlights the importance of evaluation for ASS in idiopathic interstitial pneumonia, and APLS in ASS patients. |
format | Online Article Text |
id | pubmed-9194464 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | John Wiley & Sons, Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-91944642022-06-21 Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome Chhor, Louis Tu, Steven Fogarty, Paul Fong, Christopher Respirol Case Rep Case Reports We describe the first case of antisynthetase syndrome (ASS) with antibodies to anti‐glycyl tRNA synthetase (EJ‐1) with antiphospholipid syndrome (APLS). A 66‐year‐old man presented with progressive dyspnoea, fever, dry cough and proximal muscle weakness over several months on a background of cryptogenic organizing pneumonia. Examination revealed bibasal fine chest crackles, proximal muscle weakness of the upper and lower limbs, digital skin thickening and facial telangiectasias. Creatine kinase was elevated and autoimmune screening was positive for anti‐EJ‐1, anti‐beta‐2‐glycoprotein, anti‐Ro and anti‐La antibodies. Computed tomography of the chest revealed a usual interstitial pneumonia pattern and a ventilation–perfusion scan demonstrated scintigraphic evidence of bilateral pulmonary emboli. A diagnosis of ASS and APLS was made. Immunosuppressive therapy including pulsed methylprednisolone, rituximab and mycophenolate was commenced with improvement in symptoms. This case highlights the importance of evaluation for ASS in idiopathic interstitial pneumonia, and APLS in ASS patients. John Wiley & Sons, Ltd 2022-06-13 /pmc/articles/PMC9194464/ /pubmed/35734553 http://dx.doi.org/10.1002/rcr2.990 Text en © 2022 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made. |
spellingShingle | Case Reports Chhor, Louis Tu, Steven Fogarty, Paul Fong, Christopher Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome |
title | Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome |
title_full | Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome |
title_fullStr | Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome |
title_full_unstemmed | Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome |
title_short | Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome |
title_sort | antisynthetase syndrome with rare ej‐1 antibodies with antiphospholipid syndrome |
topic | Case Reports |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9194464/ https://www.ncbi.nlm.nih.gov/pubmed/35734553 http://dx.doi.org/10.1002/rcr2.990 |
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