Clinical features of dermatomyositis associated with anti-MDA5 antibodies by age

Yamaguchi, Kazutaka; Yamaguchi, Aya; Onuki, Yuji; Itai, Miki; Kashiwagi, Chiharu; Takehara, Ken; Aoki, Shuhei; Kanaya, Azusa; Taguchi, Kohei; Umetsu, Kazue; Oshima, K.; Uchida, Megumi; Kimura, Hayato; Kasahara, Morimitsu; Takemura, Masao; Hara, Kenichiro; Sekiguchi, Akiko; Motegi, Sei‐ichiro; Muro, Yoshinao; Nakasatomi, Masao; Motohashi, Rena; Sakairi, Toru; Nakagawa, Junichi; Hiromura, Keiju; Obokata, Masaru; Kurabayashi, Masahiko; Maeno, Toshitaka

doi:10.1080/14397595.2020.1740400

Cited by 22 publications

(18 citation statements)

References 39 publications

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“…Anti-MDA5 DM is also associated with a more specific cutaneous phenotype, that includes palmar papules and skin ulcerations, reported in both Caucasian ( 2 , 15 , 16 , 25 – 28 ) and Asian populations ( 9 , 10 , 12 , 29 – 31 ). Unlike Gottron’s papules, palmar papules are often located on the palmar surface or lateral sides of the fingers, especially over metacarpophalangeal and interphalangeal joints.…”

Section: Clinical Spectrum In Adults Of the Dermatomyositis With Anti-mda5 Antibodiesmentioning

confidence: 99%

“…Inversely, CD4 + and CD8 + T cell counts decrease and the CD4 + /CD8 + ratio increases in patients with ILD refractory to treatment ( 54 ). Finally, positivity for anti-TRIM21 (Ro52) Abs ( 55 , 56 ) and older age ( 14 , 29 ) are indicators of poor prognosis while the female sex and articular form might be factors of good prognosis ( 57 ).…”

Section: Clinical Spectrum In Adults Of the Dermatomyositis With Anti-mda5 Antibodiesmentioning

confidence: 99%

“…Renal function alteration is mostly due to calcineurin inhibitors and lead to treatment interruption in most cases. Bacterial, viral and fungal infections are reported, the most frequent being the reactivation of cytomegalovirus ( 29 , 46 , 171 , 172 ). Pneumocystis jirovecii pneumonia is also encountered ( 29 , 173 , 174 ).…”

Section: Treatment Of Anti-mda5 Dermatomyositismentioning

confidence: 99%

“…Bacterial, viral and fungal infections are reported, the most frequent being the reactivation of cytomegalovirus ( 29 , 46 , 171 , 172 ). Pneumocystis jirovecii pneumonia is also encountered ( 29 , 173 , 174 ). Whether the infections are the result of IS treatment or of the disease itself is not clear, although DM was associated with a higher risk of developing pneumocystosis in a French retrospective study ( 175 ).…”

Section: Treatment Of Anti-mda5 Dermatomyositismentioning

confidence: 99%

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Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies

2021

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Anti-MDA5 dermatomyositis is a rare systemic autoimmune disease, historically described in Japanese patients with clinically amyopathic dermatomyositis and life-threatening rapidly progressive interstitial lung disease. Subsequently, the complete clinical spectrum of the disease was enriched by skin, articular and vascular manifestations. Depending on the predominance of these symptoms, three distinct clinical phenotypes with different prognosis are now defined. To date, the only known molecular component shared by the three entities are specific antibodies targeting MDA5, a cytosolic protein essential for antiviral host immune responses. Several biological tools have emerged to detect these antibodies, with drawbacks and limitations for each of them. However, the identification of this highly specific serological marker of the disease raises the question of its role in the pathogenesis. Although current knowledge on the pathogenic mechanisms that take place in the disease are still in their enfancy, several lines of evidence support a central role of interferon-mediated vasculopathy in the development of skin and lung lesions, as well as a possible pathogenic involvement of anti-MDA5 antibodies. Here, we review the clinical and biological evidences in favor of these hypothesis, and we discuss the contribution of emerging therapies that shed some light on the pathogenesis of the disease.

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Section: Clinical Spectrum In Adults Of the Dermatomyositis With Anti-mda5 Antibodiesmentioning

confidence: 99%

Section: Clinical Spectrum In Adults Of the Dermatomyositis With Anti-mda5 Antibodiesmentioning

confidence: 99%

Section: Treatment Of Anti-mda5 Dermatomyositismentioning

confidence: 99%

Section: Treatment Of Anti-mda5 Dermatomyositismentioning

confidence: 99%

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Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies

2021

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“…Older age (> 60 years), male gender, fever, elevated CRP more than 1 mg/dL and oxygen saturation less than 95% are associated with poor prognosis and mortality [99,100] Sato et al derived a prediction model in a large cohort of Japanese patients of myositis-ILD using a composite of age, CRP, oxygen saturation and anti-MDA5 antibodies for prediction of mortality. Another risk model, 'FLAIR', using…”

Section: Prognosismentioning

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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Quantitative CT analysis of interstitial pneumonia in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis: a single center, retrospective study

et al. 2022

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Clinical features of dermatomyositis associated with anti-MDA5 antibodies by age

Cited by 22 publications

References 39 publications

Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies

Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies

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

Quantitative CT analysis of interstitial pneumonia in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis: a single center, retrospective study

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