Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: A systematic review

McPherson, Mark; Economidou, Sofia C.; Liampas, Andreas; Zis, Panagiotis; Parperis, Konstantinos

doi:10.1016/j.semarthrit.2022.151959

Cited by 59 publications

(53 citation statements)

References 42 publications

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“…A recent systematic review suggested that combination therapy should be the first option in patients with DM-related ILD with MDA5 seropositivity. The usual approach includes glucocorticoids (GC) and calcineurin inhibitors (Cyclosporine A, Tacrolimus) or, alternatively, triple therapy, adding to the previous scheme, intravenous Cyclophosphamide (CYC), or Mycophenolate Mofetil (MM) if CYC is not feasible [ 44 ]. GC can be administered orally (Prednisone or Prednisolone) or intravenous (Methylprednisolone) with tapering dosages over several months, dependent on the clinical response [ 1 ].…”

Section: Treatmentmentioning

confidence: 99%

“…Regarding the calcineurin inhibitors, Cyclosporine A and Tacrolimus showed similar efficacy [ 32 ]. In the absence of a response to treatment with GC plus calcineurin inhibitors, adding CYC, MM, Rituximab, Basilixumab or Tofacitinib should be considered [ 44 ].…”

Section: Treatmentmentioning

confidence: 99%

“…Kurasawa K. et al consider the titer of these antibodies as a marker of disease activity which can be used to evaluate the efficacy of the treatment [ 33 ]. The level of ferritin, C-reactive protein, Ro-52 antibodies and cutaneous vasculopathy also correlate with the severity of the disease and prognosis [ 5 , 20 , 44 , 61 , 62 , 63 ].…”

Section: Prognosismentioning

confidence: 99%

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Anti-MDA5 Amyopathic Dermatomyositis—A Diagnostic and Therapeutic Challenge

Bobircă

Alexandru

Mușetescu

et al. 2022

Life

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Clinically amyopathic Dermatomyositis (CADM) is a rare subtype of idiopathic inflammatory myositis, associated with no muscular manifestations, which is more frequent in Asian women. Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies are a recently discovered type of specific autoantibodies associated with myositis. The anti-MDA5 DM was initially described in Japan and later it was discovered that the target antigen was a protein implicated in the innate immune response against viruses, that is encoded by the melanoma differentiation-associated gene 5. Anti-MDA5 DM is characteristically associated with distinguished mucocutaneus and systemic manifestations, including skin ulcerations, palmar papules, arthritis, and interstitial-lung disease. Patients with anti-MDA5 positivity have a high risk of developing rapid progressive interstitial-lung disease (RP-ILD), with a poor outcome. As a result, despite high mortality, diagnosis is often delayed, necessitating increased awareness of this possible condition. Despite a severe course of lung disease and an increased mortality rate, there is currently no standard treatment. Recent insights based on observational studies and case reports support combined therapy with immunosuppressive drugs and corticotherapy, as soon as the symptoms appear. The aim of this paper is to describe anti-MDA5 DM, focusing on the recent literature about the unique clinical manifestations and therapeutic options, starting from a severe clinical case diagnosed in our Rheumatology Department.

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Section: Treatmentmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Section: Prognosismentioning

confidence: 99%

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Anti-MDA5 Amyopathic Dermatomyositis—A Diagnostic and Therapeutic Challenge

Bobircă

Alexandru

Mușetescu

et al. 2022

Life

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“…Die klinischen Anzeichen einer Myositis sind in der Regel nicht vorhanden oder leicht ausgeprägt [92]. Die überwiegende Mehrheit der Studien zu Anti-MDA-5-DM stammt aus Asien [93, 94]. Anti-MDA-5-Antikörpertiter korrelieren mit dem Schweregrad der Erkrankung, und höhere Werte werden mit schnell fortschreitender ILD bei juveniler DM in Verbindung gebracht [95, 96].…”

Section: Humorale Immunität Bei Dm Und Imnm: Die Rolle Der B-zellen U...unclassified

Pathophysiologische Mechanismen und Behandlung von Dermatomyositis und immunvermittelten nekrotisierenden Myopathien: Ein fokussiertes Review

et al. 2022

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Idiopathische entzündliche Myopathien (IIM), allgemein als Myositis bekannt, sind eine gemischte Gruppe von seltenen Autoimmunerkrankungen, die hauptsächlich Skelettmuskeln betreffen, obwohl auch andere Organe oder Gewebe beteiligt sein können. Das wichtigste klinische Merkmal der Myositis ist eine subakute, fortschreitende, symmetrische Muskelschwäche in den proximalen Armen und Beinen, während Subtypen der Myositis auch außermuskuläre Merkmale wie Hautbeteiligung, Arthritis oder interstitielle Lungenerkrankung (ILD) aufweisen können. Zu den etablierten Untergruppen der IIM gehören die Dermatomyositis (DM), die immunvermittelte nekrotisierende Myopathie (IMNM), das Antisynthetase-Syndrom (ASyS), die Myositis bei Overlap-Syndrom (OM) und die Einschlusskörpermyositis (IBM). Obwohl sich die klinischen Merkmale dieser Untergruppen überschneiden, deuten die großen Unterschiede in den klinischen Manifestationen der IIM auf unterschiedliche pathophysiologische Mechanismen hin. Es ist bekannt, dass verschiedene Komponenten des Immunsystems bei der IIM eine wichtige Rolle spielen, obwohl die genauen pathophysiologischen Mechanismen, die zu den Muskelschäden führen, noch unbekannt sind. Die derzeitige Behandlung, die aus Glukokortikoiden und anderen immunsuppressiven oder immunmodulierenden Wirkstoffen besteht, führt häufig nicht zu einer anhaltenden positiven Reaktion und ist mit verschiedenen unerwünschten Wirkungen verbunden. Es wurden neue therapeutische Ziele identifiziert, die die Ergebnisse bei Patienten mit IIM verbessern könnten. Ein besseres Verständnis der sich überschneidenden und abweichenden pathophysiologischen Mechanismen der wichtigsten Untergruppen der Myositis ist notwendig, um die Behandlung zu optimieren. Ziel dieser Übersicht ist es, über die jüngsten Fortschritte bei DM und IMNM zu berichten.

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“…Anti-melanoma differentiation-associated gene-5 (MDA-5) antibody is an autoantibody found in patients with dermatomyositis, especially those with typical skin findings but no myositis (1). These antibody-positive patients are clinically characterized by complications of rapidly progressive interstitial lung disease (ILD) resistant to treatment and with poor prognosis (1). MDA-5 is a protein molecule belonging to the retinoic acid-inducible gene-I (RIG-I) family.…”

Section: Introductionmentioning

confidence: 99%

Rapidly Progressive Interstitial Lung Disease Associated with Melanoma Differentiation-Associated Gene 5 Antibody

Maezawa¹,

Narita²,

Tanimura³

et al. 2022

Acta Med. (Hradec Kralove, Czech Repub.)

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Anti-melanoma differentiation-associated gene-5 (MDA-5) antibody is an autoantibody found in patients with dermatomyositis. These antibody-positive patients are clinically characterized by complications of rapidly progressive interstitial pneumonia resistant to treatment and with poor prognosis. We describe herein a patient with MDA-5 antibody-positive interstitial lung disease, which progressed rapidly to death after a period of slow progress. Recently, attention has been paid to the similarities in clinical courses and CT images between MDA-5 antibody-positive interstitial lung disease and coronavirus disease 2019 (COVID-19)-associated pneumonia. Patients with MDA-5 antibody do not always have diffuse and evenly distributed bilateral opacities at the time of first presentation. This patient had significant laterality of such opacities. It should be considered that MDA-5 antibody-positive patients with such laterality in opacities might progress rapidly. Chest physicians, dermatologists, and dermatologists need to be aware of the characteristics of the disease for optimal treatment choices.

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Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: A systematic review

Cited by 59 publications

References 42 publications

Anti-MDA5 Amyopathic Dermatomyositis—A Diagnostic and Therapeutic Challenge

Anti-MDA5 Amyopathic Dermatomyositis—A Diagnostic and Therapeutic Challenge

Pathophysiologische Mechanismen und Behandlung von Dermatomyositis und immunvermittelten nekrotisierenden Myopathien: Ein fokussiertes Review

Rapidly Progressive Interstitial Lung Disease Associated with Melanoma Differentiation-Associated Gene 5 Antibody

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