<abstract xmlns="http://www.w3.org/1999/xhtml">

<sec><h3>Purpose</h3>
<p>To assess clinical features, therapy, and outcome of the myositis associated with anti-MDA5 antibodies, and to propose a successful therapeutic protocol for rapidly progressive interstitial lung disease (RP-ILD) in anti-MDA5 dermatomyositis (DM).</p>
</sec>
<sec><h3>Methods</h3>
<p>A retrospective and descriptive study of three cases of anti-MDA5 associated myositis was conducted in the Department of Internal Medicine in the University Hospital Hedi Chaker, Sfax, Tunisia, between 1996 and 2016.</p>
</sec>
<sec><h3>Results</h3>
<p>From a series of 115 cases of myositis, three cases of anti-MDA5-positive DM were identified. They were three men with a mean age of 63 years. They manifested specific cutaneous manifestations including ulcers and palmar papules, mild muscular involvement, and RP-ILD. The severity of the disease was correlated to the ILD in all patients. Aggressive therapies were tried including high-dose corticoids, cyclophosphamide (CYC) cures, intravenous immunoglobulins, and rituximab (RTX), with a good outcome in the patient who received combined high steroids, CYC, and RTX pulses. The two other patients died because of a rapid worsening of their respiratory condition.</p>
</sec>
<sec><h3>Conclusion</h3>
<p>Anti-MDA positive myositis is characterised by a specific cutaneous phenotype, the discretion of muscular signs, and the correlation with RP-ILD. The poor prognosis of this entity is correlated to the high resistance of pulmonary involvement despite aggressive therapeutics. The combination between high-dose steroids, CYC, and RTX has shown good results in many reports, as well as in one of our patients.</p>
</sec>
</abstract>

Purpose
To assess clinical features, therapy, and outcome of the myositis associated with anti-MDA5 antibodies, and to propose a successful therapeutic protocol for rapidly progressive interstitial lung disease (RP-ILD) in anti-MDA5 dermatomyositis (DM).

Methods
A retrospective and descriptive study of three cases of anti-MDA5 associated myositis was conducted in the Department of Internal Medicine in the University Hospital Hedi Chaker, Sfax, Tunisia, between 1996 and 2016.

Results
From a series of 115 cases of myositis, three cases of anti-MDA5-positive DM were identified. They were three men with a mean age of 63 years. They manifested specific cutaneous manifestations including ulcers and palmar papules, mild muscular involvement, and RP-ILD. The severity of the disease was correlated to the ILD in all patients. Aggressive therapies were tried including high-dose corticoids, cyclophosphamide (CYC) cures, intravenous immunoglobulins, and rituximab (RTX), with a good outcome in the patient who received combined high steroids, CYC, and RTX pulses. The two other patients died because of a rapid worsening of their respiratory condition.

Conclusion
Anti-MDA positive myositis is characterised by a specific cutaneous phenotype, the discretion of muscular signs, and the correlation with RP-ILD. The poor prognosis of this entity is correlated to the high resistance of pulmonary involvement despite aggressive therapeutics. The combination between high-dose steroids, CYC, and RTX has shown good results in many reports, as well as in one of our patients.

Clinical and therapeutic features of myositis associated with anti-MDA5 antibodies: three new cases

Pneumologia

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Purpose
To assess clinical features, therapy, and outcome of the myositis associated with anti-MDA5 antibodies, and to propose a successful therapeutic...

Clinical and therapeutic features of myositis associated with anti-MDA5 antibodies: three new cases

Published Online: Jun 28, 2023

Page range: 46 - 52

DOI: https://doi.org/10.2478/pneum-2023-0007

Keywords
dermatomyositis, anti-MDA5 antibody, interstitial lung disease, cryptogenic organising pneumonia, rituximab

© 2021 Donia Chebbi et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Clinical and therapeutic features of myositis associated with anti-MDA5 antibodies: three new cases

Published Online: Jun 28, 2023

Page range: 46 - 52

DOI: https://doi.org/10.2478/pneum-2023-0007

Keywordsdermatomyositis, anti-MDA5 antibody, interstitial lung disease, cryptogenic organising pneumonia, rituximab

© 2021 Donia Chebbi et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Keywords
dermatomyositis, anti-MDA5 antibody, interstitial lung disease, cryptogenic organising pneumonia, rituximab