Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment
Inflammatory myopathies (IM) are auto-immune connective tissue diseases characterized by muscle involvement and by extramuscular manifestations. As such, pulmonary manifestations, which mainly include interstitial lung disease (ILD), often darken two out of four distinct IM, namely dermatomyositis a...
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2020-01-01
|
| Series: | Frontiers in Medicine |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/article/10.3389/fmed.2019.00326/full |
| _version_ | 1818292507921874944 |
|---|---|
| author | Baptiste Hervier Baptiste Hervier Yurdagül Uzunhan Yurdagül Uzunhan |
| author_facet | Baptiste Hervier Baptiste Hervier Yurdagül Uzunhan Yurdagül Uzunhan |
| author_sort | Baptiste Hervier |
| collection | DOAJ |
| description | Inflammatory myopathies (IM) are auto-immune connective tissue diseases characterized by muscle involvement and by extramuscular manifestations. As such, pulmonary manifestations, which mainly include interstitial lung disease (ILD), often darken two out of four distinct IM, namely dermatomyositis and overlapping myositis. Being the initiation site of the disease and being the leading cause of morbidity and mortality, ILD is of major importance in this context. ILD has a heterogeneous expression among the patients, with various onset mode, various radiological pattern, various severity and finally with different prognoses, which are particularly difficult to predict at the time of IM diagnosis. Therefore, ILD is a challenging issue. Treatments are based on steroids and immunosuppressive or targeted therapies. Their respective place is yet poorly codified however and remains often based on clinician expertise. Dedicated clinical trials are lacking to date and are also difficult to build, due to difficulty of constituting large and homogeneous patient groups and to rigorously evaluate disease outcomes. Indeed, pulmonary function tests alone are being regularly defeated in IM, in which respiratory muscles are often involved. Composite scores, bringing together several lung parameters, should thus be developed and validated in the future, to better assess the disease response to treatment. This review aims to describe the current knowledge of IM immuno-pathogenesis, the clinical features associated with IM related-ILD, focusing of both severity and prognosis, and the actual therapeutic approaches. |
| first_indexed | 2024-12-13T03:01:04Z |
| format | Article |
| id | doaj.art-faf9b19663b34f6d823824c27995476b |
| institution | Directory Open Access Journal |
| issn | 2296-858X |
| language | English |
| last_indexed | 2024-12-13T03:01:04Z |
| publishDate | 2020-01-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Medicine |
| spelling | doaj.art-faf9b19663b34f6d823824c27995476b2022-12-22T00:01:50ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2020-01-01610.3389/fmed.2019.00326487406Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to TreatmentBaptiste Hervier0Baptiste Hervier1Yurdagül Uzunhan2Yurdagül Uzunhan3Internal Medicine and Clinical Immunology Department, French Referral Centre for Rare Neuromuscular Disorders, Hôpital Pitié-Salpêtrière, APHP, Paris, FranceINSERM UMR-S 1135, CIMI-Paris, UPMC & Sorbonne Université, Paris, FrancePneumology Department, Reference Center for Rare Pulmonary Diseases, Hôpital Avicenne, APHP, Bobigny, FranceINSERM UMR1272, Université Paris 13, Bobigny, FranceInflammatory myopathies (IM) are auto-immune connective tissue diseases characterized by muscle involvement and by extramuscular manifestations. As such, pulmonary manifestations, which mainly include interstitial lung disease (ILD), often darken two out of four distinct IM, namely dermatomyositis and overlapping myositis. Being the initiation site of the disease and being the leading cause of morbidity and mortality, ILD is of major importance in this context. ILD has a heterogeneous expression among the patients, with various onset mode, various radiological pattern, various severity and finally with different prognoses, which are particularly difficult to predict at the time of IM diagnosis. Therefore, ILD is a challenging issue. Treatments are based on steroids and immunosuppressive or targeted therapies. Their respective place is yet poorly codified however and remains often based on clinician expertise. Dedicated clinical trials are lacking to date and are also difficult to build, due to difficulty of constituting large and homogeneous patient groups and to rigorously evaluate disease outcomes. Indeed, pulmonary function tests alone are being regularly defeated in IM, in which respiratory muscles are often involved. Composite scores, bringing together several lung parameters, should thus be developed and validated in the future, to better assess the disease response to treatment. This review aims to describe the current knowledge of IM immuno-pathogenesis, the clinical features associated with IM related-ILD, focusing of both severity and prognosis, and the actual therapeutic approaches.https://www.frontiersin.org/article/10.3389/fmed.2019.00326/fullinflammatory myopathymyositisinterstitial lung diseaseauto-immunityantisynthetaseanti-MDA-5 autoantibody |
| spellingShingle | Baptiste Hervier Baptiste Hervier Yurdagül Uzunhan Yurdagül Uzunhan Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment Frontiers in Medicine inflammatory myopathy myositis interstitial lung disease auto-immunity antisynthetase anti-MDA-5 autoantibody |
| title | Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment |
| title_full | Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment |
| title_fullStr | Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment |
| title_full_unstemmed | Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment |
| title_short | Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment |
| title_sort | inflammatory myopathy related interstitial lung disease from pathophysiology to treatment |
| topic | inflammatory myopathy myositis interstitial lung disease auto-immunity antisynthetase anti-MDA-5 autoantibody |
| url | https://www.frontiersin.org/article/10.3389/fmed.2019.00326/full |
| work_keys_str_mv | AT baptistehervier inflammatorymyopathyrelatedinterstitiallungdiseasefrompathophysiologytotreatment AT baptistehervier inflammatorymyopathyrelatedinterstitiallungdiseasefrompathophysiologytotreatment AT yurdaguluzunhan inflammatorymyopathyrelatedinterstitiallungdiseasefrompathophysiologytotreatment AT yurdaguluzunhan inflammatorymyopathyrelatedinterstitiallungdiseasefrompathophysiologytotreatment |