Clinical spectrum and therapeutics in Canadian patients with anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis: a case-based review

Huang, Kun; Vinik, Ophir; Shojania, Kam; Yeung, James; Shupak, Rachel; Nimmo, Michael; Aviña‐Zubieta, J Antonio

doi:10.1007/s00296-019-04398-2

Cited by 84 publications

(85 citation statements)

References 32 publications

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“…Other similar cases have been reported since then, with favorable outcomes (18,19). Like our two patients, at least one of them had anti-SSA 52 antibodies (18), which are frequently associated with anti-MDA5 antibodies (10) and seem to be correlated with severe forms of ILD (2,20). As illustrated by our patient #2, some cases may present with RP-ILD and poor or no cutaneous, articular or muscle involvement (2,21), making diagnosis even more tricky and may delay proper support.…”

Section: Discussionsupporting

confidence: 79%

Interstitial Lung Disease-Complicated Anti-MDA5 Antibody in Clinically Amyopathic Dermatomyositis Patients: Report of Two Cases With Distinct Clinical Features

Pacot

Pouchot

Prost³

et al. 2020

Front. Med.

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Section: Discussionsupporting

confidence: 79%

Interstitial Lung Disease-Complicated Anti-MDA5 Antibody in Clinically Amyopathic Dermatomyositis Patients: Report of Two Cases With Distinct Clinical Features

Pacot

Pouchot

Prost³

et al. 2020

Front. Med.

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“…Extracorporeal Membrane Oxidation as a bridge therapy awaiting response to immunosuppressants or to a lung transplant may be used in equipped centers. There are 8 reported cases of lung transplant with an overall survival rate of 75% over 7 months to 12 years [96,97].…”

Section: Treatmentmentioning

confidence: 99%

Understanding and managing anti-MDA 5 dermatomyositis, including potential COVID-19 mimicry

2021

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Anti-Melanoma Differentiation-Associated gene 5 (MDA-5) Dermatomyositis (MDA5, DM) is a recently identified subtype of myositis characteristically associated with Rapidly Progressive Interstitial Lung Disease (RP-ILD) and unique cutaneous features. We reviewed PubMed, SCOPUS and Web of Science databases and selected 87 relevant articles after screening 1485 search results, aiming to gain a better understanding of the pathophysiology, clinical features, diagnosis, and treatment approaches of anti-MDA-5 DM described in the literature. The etiopathogenesis is speculatively linked to an unidentified viral trigger on the background of genetic predisposition culminating in an acquired type I interferonopathy. The clinical phenotype is highly varied in different ethnicities, with new clinical features having been recently described, expanding the spectrum of cases that should raise the suspicion of anti-MDA-5 DM. Unfortunately, the diagnosis is frequently missed despite excessive mortality, calling for wider awareness of suspect symptoms. RP ILD is the major determinant of survival, treatment being largely based on observational studies with recent insights into aggressive combined immunosuppression at the outset.

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“… 2 In addition to the typical skin lesions seen in anti-MDA5 + patients, such as V-sign, shawl sign, Gottron sign, skin ulcers, 3 and subcutaneous calcification, 4 rapidly progressive interstitial lung disease (RP-ILD), 5 , 6 as well as high inflammation status 7 has received increasing attention for its association with poor prognosis and high mortality. 8 , 9 …”

Section: Introductionmentioning

confidence: 99%

Clinical Characteristics of Lipid Metabolism in Untreated Patients with Anti-MDA5 Antibody-Positive

Huang¹,

Ren²,

Luo³

et al. 2021

IJGM

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Objective Clinical characterization of lipid metabolism in untreated patients with anti-melanoma differentiation-associated gene 5 antibodies-positive (anti-MDA5 + ). Methods Body-mass index (BMI), autoantibodies, lipid levels, and serum ferritin levels in 57 anti-MDA5 + patients were determined in the Department of Rheumatology and Immunology of the Second Affiliated Hospital of Chongqing Medical University. Results Plasma high-density lipoprotein (HDL) and apolipoprotein A1 (ApoA1) levels were significantly lower in deceased group than in the survival group (P < 0.05). Plasma levels of HDL and ApoA1 were significantly lower in patients who were simultaneously anti-MDA5 + and anti-Ro-52 + than in patients who were anti-MDA5 + alone (P < 0.05). Plasma levels of total cholesterol, low-density lipoprotein, HDL, and ApoA1 were significantly decreased in patients with high levels of serum ferritin compared with patients with low levels (P < 0.05). There were no significant differences in blood lipid levels between patients grouped according to BMI. Conclusion 1) HDL and ApoA1 levels are important indicators of poor prognosis in anti-MDA5 + patients; 2) Dysregulated lipid metabolism in anti-MDA5 + patients is closely associated with anti-Ro-52 antibody and ferritin levels but independent of BMI; 3) HDL involvement in inflammation and immune regulation merits close attention by rheumatologists.

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Clinical spectrum and therapeutics in Canadian patients with anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis: a case-based review

Cited by 84 publications

References 32 publications

Interstitial Lung Disease-Complicated Anti-MDA5 Antibody in Clinically Amyopathic Dermatomyositis Patients: Report of Two Cases With Distinct Clinical Features

Interstitial Lung Disease-Complicated Anti-MDA5 Antibody in Clinically Amyopathic Dermatomyositis Patients: Report of Two Cases With Distinct Clinical Features

Understanding and managing anti-MDA 5 dermatomyositis, including potential COVID-19 mimicry

Clinical Characteristics of Lipid Metabolism in Untreated Patients with Anti-MDA5 Antibody-Positive

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