COVID-19 as a putative trigger of anti-MDA5-associated dermatomyositis with acute respiratory distress syndrome (ARDS) requiring lung transplantation, a case report

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Background

Autoimmune disease following COVID-19 has been studied intensely since the beginning of the pandemic. Growing evidence indicates that SARS-CoV-2 infection, by virtue of molecular mimicry can lead to an antigen-mediated cross-reaction promoting the development of a plethora of autoimmune spectrum diseases involving lungs and extrapulmonary tissues alike. In both COVID-19 and autoimmune disease, the immune self-tolerance breaks, leading to an overreaction of the immune system with production of a variety of autoantibodies, sharing similarities in clinical manifestation, laboratory, imaging, and pathology findings. Anti-Melanoma Differentiation-Associated gene 5 dermatomyositis (anti-MDA5 DM) comprises a rare subtype of systemic inflammatory myopathies associated with characteristic cutaneous features and life-threatening rapidly progressive interstitial lung disease (RP-ILD). The production of anti-MDA5 autoantibodies was proposed to be triggered by viral infections.

Case presentation

A 20-year-old male patient with polyarthritis, fatigue and exertional dyspnea was referred to our department. An elevated anti-MDA5 autoantibody titer, myositis on MRI, ground glass opacifications on lung CT and histological features of Wong-type dermatomyositis were confirmed, suggesting the diagnosis of an anti-MDA5 DM. Amid further diagnostic procedures, a serologic proof of a recent SARS-CoV-2 infection emerged. Subsequently, the patient deteriorated into a fulminant respiratory failure and an urgent lung transplantation was performed, leading to remission ever since (i.e. 12 months as of now).

Conclusions

We report a unique case of a patient with a new-onset anti-MDA5 DM with fulminant ARDS emerging in a post-infectious stage of COVID-19, who underwent a successful lung transplantation and achieved remission. Given the high mortality of anti-MDA5 DM associated RP-ILD, we would like to highlight that the timely recognition of this condition and urgent therapy initiation are of utmost importance.

Supplementary Information

The online version contains supplementary material available at 10.1186/s41927-022-00271-1.

Related collections

Most cited references 31

Record: found
Abstract: found
Article: not found

Differential roles of MDA5 and RIG-I helicases in the recognition of RNA viruses.

Hiroki Kato, Osamu Takeuchi, Shintaro Sato … (2006)

The innate immune system senses viral infection by recognizing a variety of viral components (including double-stranded (ds)RNA) and triggers antiviral responses. The cytoplasmic helicase proteins RIG-I (retinoic-acid-inducible protein I, also known as Ddx58) and MDA5 (melanoma-differentiation-associated gene 5, also known as Ifih1 or Helicard) have been implicated in viral dsRNA recognition. In vitro studies suggest that both RIG-I and MDA5 detect RNA viruses and polyinosine-polycytidylic acid (poly(I:C)), a synthetic dsRNA analogue. Although a critical role for RIG-I in the recognition of several RNA viruses has been clarified, the functional role of MDA5 and the relationship between these dsRNA detectors in vivo are yet to be determined. Here we use mice deficient in MDA5 (MDA5-/-) to show that MDA5 and RIG-I recognize different types of dsRNAs: MDA5 recognizes poly(I:C), and RIG-I detects in vitro transcribed dsRNAs. RNA viruses are also differentially recognized by RIG-I and MDA5. We find that RIG-I is essential for the production of interferons in response to RNA viruses including paramyxoviruses, influenza virus and Japanese encephalitis virus, whereas MDA5 is critical for picornavirus detection. Furthermore, RIG-I-/- and MDA5-/- mice are highly susceptible to infection with these respective RNA viruses compared to control mice. Together, our data show that RIG-I and MDA5 distinguish different RNA viruses and are critical for host antiviral responses.

0 comments Cited 408 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

COVID-19 and autoimmune diseases

Yu. Liu, Amr Sawalha, Qianjin Lu (2020)

Purpose of review The aim of this study was to evaluate the relationship between infection with SARS-CoV-2 and autoimmunity. Recent findings Coronavirus disease 2019 (COVID-19) is an infectious disease caused by severe acute respiratory syndrome (SARS) associated coronavirus 2 (SARS-CoV-2). Although most of the infected individuals are asymptomatic, a proportion of patients with COVID-19 develop severe disease with multiple organ injuries. Evidence suggests that some medications used to treat autoimmune rheumatologic diseases might have therapeutic effect in patients with severe COVID-19 infections, drawing attention to the relationship between COVID-19 and autoimmune diseases. COVID-19 shares similarities with autoimmune diseases in clinical manifestations, immune responses and pathogenic mechanisms. Robust immune reactions participate in the pathogenesis of both disease conditions. Autoantibodies as a hallmark of autoimmune diseases can also be detected in COVID-19 patients. Moreover, some patients have been reported to develop autoimmune diseases, such as Guillain--Barré syndrome or systemic lupus erythematosus, after COVID-19 infection. It is speculated that SARS-CoV-2 can disturb self-tolerance and trigger autoimmune responses through cross-reactivity with host cells. The infection risk and prognosis of COVID-19 in patients with autoimmune diseases remains controversial, but patient adherence to medication regimens to prevent autoimmune disease flares is strongly recommended. Summary We present a review of the association between COVID-19 and autoimmune diseases, focusing on similarities in immune responses, cross-reactivity of SARS-CoV-2, the development of autoimmune diseases in COVID-19 patients and the risk of COVID-19 infection in patients with preexisting autoimmune conditions.

0 comments Cited 204 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: found

Is Open Access

Effect of Colchicine vs Standard Care on Cardiac and Inflammatory Biomarkers and Clinical Outcomes in Patients Hospitalized With Coronavirus Disease 2019

Spyridon Deftereos, Georgios Giannopoulos, Dimitrios Vrachatis … (2020)

Key Points Question Is the receipt of colchicine among patients hospitalized with symptomatic coronavirus disease 2019 associated with clinical benefit? Findings In this randomized clinical trial of 105 patients, the rate of the primary clinical end point (clinical deterioration) was higher in the control group than in the colchicine group, and the time to clinical deterioration was shorter in the control group than in the colchicine arm. No difference was observed in the primary biochemical end point (high-sensitivity troponin concentration), but patients in the colchicine group had a smaller increase in dimerized plasma fragment D compared with patients in the control group. Meaning The hypothesis-generating findings of this study suggest a role for colchicine in the treatment of patients with coronavirus disease 2019.

0 comments Cited 189 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Contributors

Stephan Blüml:

ORCID: http://orcid.org/0000-0002-2758-4400

stephan.blueml@meduniwien.ac.at

Journal

Journal ID (nlm-ta): BMC Rheumatol

Journal ID (iso-abbrev): BMC Rheumatol

Title: BMC Rheumatology

Publisher: BioMed Central (London )

ISSN (Electronic): 2520-1026

Publication date (Electronic): 13 July 2022

Publication date PMC-release: 13 July 2022

Publication date Collection: 2022

Volume: 6

Electronic Location Identifier: 42

Affiliations

[1 ]GRID grid.22937.3d, ISNI 0000 0000 9259 8492, Division of Rheumatology, Department of Medicine III, , Medical University of Vienna, ; Waehringer Guertel 18-20, 1090 Vienna, Austria

[2 ]GRID grid.22937.3d, ISNI 0000 0000 9259 8492, Department of Clinical Pharmacology, , Medical University of Vienna, ; Waehringer Guertel 18-20, 1090 Vienna, Austria

[3 ]GRID grid.22937.3d, ISNI 0000 0000 9259 8492, Department of Thoracic Surgery, , Medical University of Vienna, ; Waehringer Guertel 18-20, 1090 Vienna, Austria

[4 ]GRID grid.22937.3d, ISNI 0000 0000 9259 8492, Clinical Institute of Pathology, , Medical University of Vienna, ; Waehringer Guertel 18-20, 1090 Vienna, Austria

[5 ]GRID grid.22937.3d, ISNI 0000 0000 9259 8492, Department of Biomedical Imaging and Image-Guided Therapy, , Medical University of Vienna, ; Waehringer Guertel 18-20, 109, Vienna, Austria

[6 ]GRID grid.22937.3d, ISNI 0000 0000 9259 8492, Institute of Medical Genetics, , Medical University of Vienna, ; Waehringer Guertel 18-20, 1090 Vienna, Austria

[7 ]GRID grid.22937.3d, ISNI 0000 0000 9259 8492, Department of Dermatology, , Medical University of Vienna, ; Waehringer Guertel 18-20, 1090 Vienna, Austria

Author information

Stephan Blüml http://orcid.org/0000-0002-2758-4400

Article

Publisher ID: 271

DOI: 10.1186/s41927-022-00271-1

PMC ID: 9277832

PubMed ID: 35821079

SO-VID: 03973b02-1405-4fee-b9c7-20ad4d9b0bd2

License:

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

History

Date received : 8 February 2022

Date accepted : 12 April 2022

Funding

Funded by: FundRef http://dx.doi.org/10.13039/501100005788, Medizinische Universität Wien;

Custom metadata

Keywords: dermatomyositis,anti-mda5,ards,lung transplantation,covid-19,case report

Data availability:

Keywords: dermatomyositis, anti-mda5, ards, lung transplantation, covid-19, case report

COVID-19 as a putative trigger of anti-MDA5-associated dermatomyositis with acute respiratory distress syndrome (ARDS) requiring lung transplantation, a case report

Read this article at

Abstract

Background

Case presentation

Conclusions

Supplementary Information

Related collections

Novel Coronavirus Disease COVID-19

Most cited references 31

Differential roles of MDA5 and RIG-I helicases in the recognition of RNA viruses.

COVID-19 and autoimmune diseases

Effect of Colchicine vs Standard Care on Cardiac and Inflammatory Biomarkers and Clinical Outcomes in Patients Hospitalized With Coronavirus Disease 2019

Author and article information

Contributors

Journal

Affiliations

Author information

Article

History

Funding

Categories

Custom metadata

Comments

Comment on this article

Similar content 515

Cited by 2

Most referenced authors 610