Interstitial lung disease is a dominant feature in patients with circulating myositis-specific antibodies
Abstract Background Many patients with polymyositis (PM) or dermatomyositis (DM) have circulating myositis-specific antibodies (MSAs). Interstitial lung disease (ILD) is a common manifestation of PM/DM, and it can even precede the onset of characteristic muscle or skin manifestations. Furthermore, t...
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| Format: | Article |
| Language: | English |
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BMC
2021-11-01
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| Series: | BMC Pulmonary Medicine |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12890-021-01737-7 |
| _version_ | 1818837147287814144 |
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| author | Abhinav K. Misra Nathan L. Wong Terrance T. Healey Edward V. Lally Barry S. Shea |
| author_facet | Abhinav K. Misra Nathan L. Wong Terrance T. Healey Edward V. Lally Barry S. Shea |
| author_sort | Abhinav K. Misra |
| collection | DOAJ |
| description | Abstract Background Many patients with polymyositis (PM) or dermatomyositis (DM) have circulating myositis-specific antibodies (MSAs). Interstitial lung disease (ILD) is a common manifestation of PM/DM, and it can even precede the onset of characteristic muscle or skin manifestations. Furthermore, there appear to be some patients with ILD and circulating MSAs who do not develop muscle or skin disease even after prolonged follow-up. We sought to determine whether ILD is equally or more common than myositis or dermatitis at the time of initial detection of MSAs. Methods We identified all patients found to have circulating MSAs at our institution over a 4-year period and assessed for the presence of lung, muscle, and skin disease at the time of initial detection of MSAs. Among those found to have ILD, we compared demographic and clinical features, chest CT scan findings, and outcomes between those with PM/DM-associated ILD and those with ILD but no muscle or skin disease. Results A total of 3078 patients were tested for MSAs, and of these 40 were positive. Nine different MSAs were detected, with anti-histidyl tRNA synthetase (anti-Jo-1) being the most common (35% of MSAs). Among patients with positive MSAs, 86% were found to have ILD, compared to 39% and 28% with muscle and skin involvement, respectively (p < 0.001). Fifty percent of all MSA-positive patients had isolated ILD, with no evidence of muscle or skin disease. Those with isolated ILD were more likely to be older and have fibrotic changes on chest CT, less likely to receive immunomodulatory therapy, and had worse overall survival. Conclusions In this study we found that individuals with circulating MSAs were more likely to have ILD than classic muscle or skin manifestations of PM/DM at the time of initial detection of MSAs. Our findings suggest that the presence of ILD should be considered a disease-defining manifestation in the presence of MSAs and incorporated into classification criteria for PM/DM. |
| first_indexed | 2024-12-19T03:17:53Z |
| format | Article |
| id | doaj.art-4b7d055effb443ce8e13b1e660035f3c |
| institution | Directory Open Access Journal |
| issn | 1471-2466 |
| language | English |
| last_indexed | 2024-12-19T03:17:53Z |
| publishDate | 2021-11-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pulmonary Medicine |
| spelling | doaj.art-4b7d055effb443ce8e13b1e660035f3c2022-12-21T20:37:50ZengBMCBMC Pulmonary Medicine1471-24662021-11-012111810.1186/s12890-021-01737-7Interstitial lung disease is a dominant feature in patients with circulating myositis-specific antibodiesAbhinav K. Misra0Nathan L. Wong1Terrance T. Healey2Edward V. Lally3Barry S. Shea4Division of Pulmonary, Critical Care and Sleep Medicine, Rhode Island Hospital and Alpert Medical School of Brown UniversityDepartment of Medicine, Rhode Island Hospital and Alpert Medical School of Brown UniversityDepartment of Diagnostic Imaging, Rhode Island Hospital and Alpert Medical School of Brown UniversityDivision of Rheumatology, Rhode Island Hospital and Alpert Medical School of Brown UniversityDivision of Pulmonary, Critical Care and Sleep Medicine, Rhode Island Hospital and Alpert Medical School of Brown UniversityAbstract Background Many patients with polymyositis (PM) or dermatomyositis (DM) have circulating myositis-specific antibodies (MSAs). Interstitial lung disease (ILD) is a common manifestation of PM/DM, and it can even precede the onset of characteristic muscle or skin manifestations. Furthermore, there appear to be some patients with ILD and circulating MSAs who do not develop muscle or skin disease even after prolonged follow-up. We sought to determine whether ILD is equally or more common than myositis or dermatitis at the time of initial detection of MSAs. Methods We identified all patients found to have circulating MSAs at our institution over a 4-year period and assessed for the presence of lung, muscle, and skin disease at the time of initial detection of MSAs. Among those found to have ILD, we compared demographic and clinical features, chest CT scan findings, and outcomes between those with PM/DM-associated ILD and those with ILD but no muscle or skin disease. Results A total of 3078 patients were tested for MSAs, and of these 40 were positive. Nine different MSAs were detected, with anti-histidyl tRNA synthetase (anti-Jo-1) being the most common (35% of MSAs). Among patients with positive MSAs, 86% were found to have ILD, compared to 39% and 28% with muscle and skin involvement, respectively (p < 0.001). Fifty percent of all MSA-positive patients had isolated ILD, with no evidence of muscle or skin disease. Those with isolated ILD were more likely to be older and have fibrotic changes on chest CT, less likely to receive immunomodulatory therapy, and had worse overall survival. Conclusions In this study we found that individuals with circulating MSAs were more likely to have ILD than classic muscle or skin manifestations of PM/DM at the time of initial detection of MSAs. Our findings suggest that the presence of ILD should be considered a disease-defining manifestation in the presence of MSAs and incorporated into classification criteria for PM/DM.https://doi.org/10.1186/s12890-021-01737-7Interstitial lung diseaseMyositis-specific antibodiesPolymyositisDermatomyositis |
| spellingShingle | Abhinav K. Misra Nathan L. Wong Terrance T. Healey Edward V. Lally Barry S. Shea Interstitial lung disease is a dominant feature in patients with circulating myositis-specific antibodies BMC Pulmonary Medicine Interstitial lung disease Myositis-specific antibodies Polymyositis Dermatomyositis |
| title | Interstitial lung disease is a dominant feature in patients with circulating myositis-specific antibodies |
| title_full | Interstitial lung disease is a dominant feature in patients with circulating myositis-specific antibodies |
| title_fullStr | Interstitial lung disease is a dominant feature in patients with circulating myositis-specific antibodies |
| title_full_unstemmed | Interstitial lung disease is a dominant feature in patients with circulating myositis-specific antibodies |
| title_short | Interstitial lung disease is a dominant feature in patients with circulating myositis-specific antibodies |
| title_sort | interstitial lung disease is a dominant feature in patients with circulating myositis specific antibodies |
| topic | Interstitial lung disease Myositis-specific antibodies Polymyositis Dermatomyositis |
| url | https://doi.org/10.1186/s12890-021-01737-7 |
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