Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients
IntroductionThis study aimed to provide an updated analysis of the different prognostic trajectories of patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibodies.MethodsAmong a cohort of 70 patients, baseline characteristics and phenotypes, treatments and outcomes were analyzed...
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Frontiers Media S.A.
2024-01-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2023.1319957/full |
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| author | Hubert de Boysson Marie Cuchet Charles Cassius Pierre Cuchet Christian Agard Alexandra Audemard-Verger Sylvain Marchand-Adam Raphaëlla Cohen-Sors Laure Gallay Julie Graveleau Cécile Lesort Kim Ly Alain Meyer Grégoire Monseau Antoine Néel Bernard Bonnotte Laurent Pérard Nicolas Schleinitz Delphine Mariotte Brigitte Le Mauff Gwladys Bourdenet Gwladys Bourdenet Wafa Masmoudi Samuel Deshayes Anaël Dumont Anne Dompmartin Diane Kottler Achille Aouba |
| author_facet | Hubert de Boysson Marie Cuchet Charles Cassius Pierre Cuchet Christian Agard Alexandra Audemard-Verger Sylvain Marchand-Adam Raphaëlla Cohen-Sors Laure Gallay Julie Graveleau Cécile Lesort Kim Ly Alain Meyer Grégoire Monseau Antoine Néel Bernard Bonnotte Laurent Pérard Nicolas Schleinitz Delphine Mariotte Brigitte Le Mauff Gwladys Bourdenet Gwladys Bourdenet Wafa Masmoudi Samuel Deshayes Anaël Dumont Anne Dompmartin Diane Kottler Achille Aouba |
| author_sort | Hubert de Boysson |
| collection | DOAJ |
| description | IntroductionThis study aimed to provide an updated analysis of the different prognostic trajectories of patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibodies.MethodsAmong a cohort of 70 patients, baseline characteristics and phenotypes, treatments and outcomes were analyzed. A Cox proportional hazards model was used to identify factors associated with poor outcomes, i.e., death or progressive disease at the last follow-up.ResultsAmong the 70 patients, 45 were women, and 54 were Caucasian. A dermatologic involvement was observed in 58 (83%) patients, including 40 with MDA5 vasculopathy-related skin lesions. Muscular involvement was observed in 39 (56%) patients. Interstitial lung disease (ILD) was observed at baseline in 52 (74%) patients, including 23 (44%) who developed rapidly progressive (RP) ILD. Seven (10%) patients showed thromboembolic complications within the first weeks of diagnosis, and eight (11%) other patients developed a malignancy (4 before the diagnosis of anti-MDA5 disease). Poor outcomes were observed in 28 (40%) patients, including 13 (19%) deaths. Among the 23 patients with RP-ILD, 19 (79%) showed poor outcomes, including 12 (63%) who died. In multivariate analyses, RP-ILD (hazard ratio (HR), 95% CI: 8.24 [3.21–22], p<0.0001), the occurrence of thromboembolic events (HR: 5.22 [1.61–14.77], p=0.008) and the presence of any malignancy (HR: 19.73 [6.67–60], p<0.0001) were the three factors independently associated with poor outcomes.DiscussionThis new independent cohort confirms the presence of different clinical phenotypes of anti-MDA5 diseases at baseline and the poor prognosis associated with RP-ILD. Thromboembolic events and malignancies were also identified as prognostic factors. |
| first_indexed | 2024-03-08T16:07:53Z |
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| issn | 1664-3224 |
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| last_indexed | 2024-03-08T16:07:53Z |
| publishDate | 2024-01-01 |
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| series | Frontiers in Immunology |
| spelling | doaj.art-e6e426ae52284ffda7e09c0923a85de42024-01-08T05:09:40ZengFrontiers Media S.A.Frontiers in Immunology1664-32242024-01-011410.3389/fimmu.2023.13199571319957Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patientsHubert de Boysson0Marie Cuchet1Charles Cassius2Pierre Cuchet3Christian Agard4Alexandra Audemard-Verger5Sylvain Marchand-Adam6Raphaëlla Cohen-Sors7Laure Gallay8Julie Graveleau9Cécile Lesort10Kim Ly11Alain Meyer12Grégoire Monseau13Antoine Néel14Bernard Bonnotte15Laurent Pérard16Nicolas Schleinitz17Delphine Mariotte18Brigitte Le Mauff19Gwladys Bourdenet20Gwladys Bourdenet21Wafa Masmoudi22Samuel Deshayes23Anaël Dumont24Anne Dompmartin25Diane Kottler26Achille Aouba27Department of Internal Medicine, Caen University Hospital, Caen, FranceDepartment of Dermatology, Caen University Hospital, Caen, FranceDepartment of Dermatology, France Saint Louis Hospital, (AP-HP), Paris, FranceDepartment of Pneumology, Caen University Hospital, Caen, FranceNantes Université, Centre Hospitalier et Universitaire (CHU) Nantes, Service de Médecine Interne, Nantes, FranceDepartment of Internal Medicine, Tours University Hospital, Tours, FranceDepartment of Pneumology, Tours University Hospital, Tours, FranceDepartment of Dermatology, Amiens University Hospital, Amiens, FranceService de Médecine Interne et Immunologie Clinique, Hôpital Édouard Herriot, Hospices Civils de Lyon, Lyon, France0Department of Internal Medicine, Saint-Nazaire Hospital, Saint-Nazaire, France1Department of Dermatology, Edouard Herriot Hospital, Hospices civiles de Lyon (HCL), Lyon, France2Department of Internal Medicine, Limoges University Hospital, Limoges, France3Department of Rheumatology, Strasbourg University Hospital, Strasbourg, France4Department of Intensive Medicine, Poitiers University Hospital Center, Poitiers, FranceNantes Université, Centre Hospitalier et Universitaire (CHU) Nantes, Service de Médecine Interne, Nantes, France5Department of Internal Medicine, Dijon University Hospital, Dijon, France6Department of Internal Medicine, Saint Joseph Saint Luc Hospital, Lyon, France7Department of Internal Medicine, La Timone University Hospital, Assistance Publique - Hopitaux de Marseille (AP-HM), Marseille, France8Department of Immunology, Caen University Hospital, Caen, France8Department of Immunology, Caen University Hospital, Caen, France9Department of Immunology, Amiens University Hospital, Amiens, France0HEMATIM – EA4666, Jules Verne University of Picardie, Amiens, France1Department of Dermatology, Rouen University Hospital, Rouen, FranceDepartment of Internal Medicine, Caen University Hospital, Caen, FranceDepartment of Internal Medicine, Caen University Hospital, Caen, FranceDepartment of Dermatology, Caen University Hospital, Caen, FranceDepartment of Dermatology, Caen University Hospital, Caen, FranceDepartment of Internal Medicine, Caen University Hospital, Caen, FranceIntroductionThis study aimed to provide an updated analysis of the different prognostic trajectories of patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibodies.MethodsAmong a cohort of 70 patients, baseline characteristics and phenotypes, treatments and outcomes were analyzed. A Cox proportional hazards model was used to identify factors associated with poor outcomes, i.e., death or progressive disease at the last follow-up.ResultsAmong the 70 patients, 45 were women, and 54 were Caucasian. A dermatologic involvement was observed in 58 (83%) patients, including 40 with MDA5 vasculopathy-related skin lesions. Muscular involvement was observed in 39 (56%) patients. Interstitial lung disease (ILD) was observed at baseline in 52 (74%) patients, including 23 (44%) who developed rapidly progressive (RP) ILD. Seven (10%) patients showed thromboembolic complications within the first weeks of diagnosis, and eight (11%) other patients developed a malignancy (4 before the diagnosis of anti-MDA5 disease). Poor outcomes were observed in 28 (40%) patients, including 13 (19%) deaths. Among the 23 patients with RP-ILD, 19 (79%) showed poor outcomes, including 12 (63%) who died. In multivariate analyses, RP-ILD (hazard ratio (HR), 95% CI: 8.24 [3.21–22], p<0.0001), the occurrence of thromboembolic events (HR: 5.22 [1.61–14.77], p=0.008) and the presence of any malignancy (HR: 19.73 [6.67–60], p<0.0001) were the three factors independently associated with poor outcomes.DiscussionThis new independent cohort confirms the presence of different clinical phenotypes of anti-MDA5 diseases at baseline and the poor prognosis associated with RP-ILD. Thromboembolic events and malignancies were also identified as prognostic factors.https://www.frontiersin.org/articles/10.3389/fimmu.2023.1319957/fullanti-MDA5 dermatomyositisprognosisrapidly progressive interstitial lung diseasethromboembolic eventsmalignancy |
| spellingShingle | Hubert de Boysson Marie Cuchet Charles Cassius Pierre Cuchet Christian Agard Alexandra Audemard-Verger Sylvain Marchand-Adam Raphaëlla Cohen-Sors Laure Gallay Julie Graveleau Cécile Lesort Kim Ly Alain Meyer Grégoire Monseau Antoine Néel Bernard Bonnotte Laurent Pérard Nicolas Schleinitz Delphine Mariotte Brigitte Le Mauff Gwladys Bourdenet Gwladys Bourdenet Wafa Masmoudi Samuel Deshayes Anaël Dumont Anne Dompmartin Diane Kottler Achille Aouba Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients Frontiers in Immunology anti-MDA5 dermatomyositis prognosis rapidly progressive interstitial lung disease thromboembolic events malignancy |
| title | Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients |
| title_full | Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients |
| title_fullStr | Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients |
| title_full_unstemmed | Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients |
| title_short | Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients |
| title_sort | disease patterns and specific trajectories of anti mda5 related disease a multicentre retrospective study of 70 adult patients |
| topic | anti-MDA5 dermatomyositis prognosis rapidly progressive interstitial lung disease thromboembolic events malignancy |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2023.1319957/full |
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