Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome
Abstract 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 o...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2022-07-01
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| Series: | Respirology Case Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/rcr2.990 |
| _version_ | 1818545802677583872 |
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| author | Louis Chhor Steven Tu Paul Fogarty Christopher Fong |
| author_facet | Louis Chhor Steven Tu Paul Fogarty Christopher Fong |
| author_sort | Louis Chhor |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-12-12T07:44:47Z |
| format | Article |
| id | doaj.art-b40cc5d542ad4d9b90078199950be22e |
| institution | Directory Open Access Journal |
| issn | 2051-3380 |
| language | English |
| last_indexed | 2024-12-12T07:44:47Z |
| publishDate | 2022-07-01 |
| publisher | Wiley |
| record_format | Article |
| series | Respirology Case Reports |
| spelling | doaj.art-b40cc5d542ad4d9b90078199950be22e2022-12-22T00:32:40ZengWileyRespirology Case Reports2051-33802022-07-01107n/an/a10.1002/rcr2.990Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndromeLouis Chhor0Steven Tu1Paul Fogarty2Christopher Fong3Department of Rheumatology Eastern Health Box Hill Victoria AustraliaDepartment of Rheumatology Eastern Health Box Hill Victoria AustraliaDepartment of Respiratory and Sleep Medicine Eastern Health Box Hill Victoria AustraliaDepartment of Rheumatology Eastern Health Box Hill Victoria AustraliaAbstract 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.https://doi.org/10.1002/rcr2.990antiphospholipid syndromeantisynthetase syndromeEJ‐1 antibodyinterstitial lung diseasemyositis |
| spellingShingle | Louis Chhor Steven Tu Paul Fogarty Christopher Fong Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome Respirology Case Reports antiphospholipid syndrome antisynthetase syndrome EJ‐1 antibody interstitial lung disease myositis |
| 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 | antiphospholipid syndrome antisynthetase syndrome EJ‐1 antibody interstitial lung disease myositis |
| url | https://doi.org/10.1002/rcr2.990 |
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