Risk factors for mortality in patients with anti-MDA5 antibody-positive dermatomyositis: A meta-analysis and systematic review

Xie, H Y; Zhang, Dingding; Wang, Yuanzhuo; Shi, Yixin; Yuan, Yuan; Luo, Wei; Fan, Jin‐Hu; Tian, Xinlun; Wang, Jinglan

doi:10.1016/j.semarthrit.2023.152231

Cited by 17 publications

(11 citation statements)

References 41 publications

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“…A current meta-analysis included 29 with 2645 patients found that old age, male sex, hypoxemia, low FVC, lymphocytopenia, and high levels of ferritin, CRP, creatine kinase, and LDH predicted poor prognosis in patients with MDA5 + DM [3] . In a large Chinese cohort study of patients with MDA5-DM, patients were classi ed into three subgroups according to peripheral lymphocyte count.…”

Section: Completely Learning Of Clinical Conditions Is Helpful For Pr...mentioning

confidence: 88%

Triple Positive for Anti-MDA5, Anti-EJ and Anti-Ro52 Antibodies Dermatomyositis Associated Interstitial Lung Disease：a case report

Guo,

Du,

et al. 2024

Preprint

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Background Clinically amyopathic dermatomyositis (CADM) is a disease with the typical skin manifestations of dermatomyositis (DM) and few or no obvious muscle weakness. The anti-melanoma differentiation associated protein 5（MDA5）antibody positive CADM patients are at a high risk of developing rapidly progressive interstitial lung disease (RP-ILD)，and exhibit immensely poor prognosis and high mortality. The disease course and severity of MDA5+CAMD are highly different, wherein some patients presented marked treatment response and didn’t occur exacerbation. Analysis to these special groups bring us better understanding of MDA5+CADM. Case presentation We presented a patient with anti-MD5 anti-Ro52 anti-EJ-triple positive DM patient. She was initially characteristic with darken skin for 2 years before gradually occurred dry cough, fever and dyspnea. She had a low oxygen saturation and diffusing capacity of the lungs for carbon monoxide. With combination therapy of GCs and two immunosuppressants, the woman obtained a rapid remission and persisted for a long time. Conclusions Patients with anti-MD5 anti-Ro52 anti-EJ-triple positive DM-ILD may present a less aggressive disease course and an individual appropriate therapy may be promising for long-term maintenance.

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Section: Completely Learning Of Clinical Conditions Is Helpful For Pr...mentioning

confidence: 88%

Triple Positive for Anti-MDA5, Anti-EJ and Anti-Ro52 Antibodies Dermatomyositis Associated Interstitial Lung Disease：a case report

Guo,

Du,

et al. 2024

Preprint

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“…Although very severe forms of ILD have been described in seronegative patients 20 , the involvement occurs at an even higher frequency in the subgroup associated with the MDA5 antibody, reaching 90–95%, and 50–80% progress to a rapidly progressive form (RP-ILD). This complication has a high mortality, reaching up to 50% 21 , and is generally refractory to immunosuppression 5 .…”

Section: Clinical Discussionmentioning

confidence: 99%

“…** Interstitial lung disease, severe dysphagia, or cutaneous ulcerations. Consider in patients with elevated ferritin or lactate dehydrogenase 21 . Consider monthly pulses until, at least, moderate response is achieved.…”

Section: Clinical Discussionmentioning

confidence: 99%

Clinically amyopathic dermatomyositis associated with cutaneous ulcerations: a case-based review

De Jesus,

De Souza

2024

Annals of Medicine &Amp; Surgery

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Introduction and Importance: Dermatomyositis (DM) is an autoimmune myopathy primarily affecting both muscles and skin. When muscle weakness is not clinically apparent, but characteristic skin lesions are present, the condition is referred to as clinically amyopathic dermatomyositis (CADM). Case Presentation: We present the case of a 52-year-old female with a typical DM rash, interstitial pneumonia, and multiple skin ulcers. The skin biopsy was consistent with DM, and there were no signs of muscle involvement. Myositis-related and myositis-specific autoantibodies were also negative. Significant improvement was not observed until the patient received successive monthly pulses of methylprednisolone and the introduction of methotrexate. This treatment regimen allowed for the complete tapering of prednisone and resulted in sustained disease control. Clinical Discussion: In addition to the case presentation, a narrative literature review was conducted using the MEDLINE database, and an evidence-based treatment flowchart is proposed. CADM is a subtype of DM, related to higher incidences of interstitial lung disease, skin vasculopathy and malignancy. When ulcers or interstitial pneumonia are present, treatment should be early and aggressive. Active screening for neoplasms is recommended, particularly within the first 5 years. Conclusion: We presented a case of seronegative CADM featuring skin vasculopathy, successfully treated with consecutive methylprednisolone pulses. Our literature review emphasized the importance of focused CADM management trials, highlighting the need for further research.

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“…erhöhtes Interleukin-6 und eine erhöhte LDH Risikofaktoren für eine erhöhte Mortalität sind [14,15]. Außerdem korrelierte der Interferon-Typ I-Biomarker SIGLEC1 in einer Studie mit der Erkrankungsaktivität von Myositis-Patient:innen [16] und der Interleukin-18-Spiegel mit der Erkrankungsaktivität sowie dem Auftreten einer ILD in DM-Patient:innen [17].…”

Section: Labordiagnostikunclassified

Dermatomyositis, Antisynthetase-Syndrom und immunvermittelte nekrotisierende Myopathie

Holzer,

Krusche,

Kötter

2024

Aktuelle Rheumatologie

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ZusammenfassungDie inflammatorischen Myopathien werden heutzutage anhand von Klinik, Biopsie und Antikörperstatus genauer gegliedert. Zumeist sind sowohl die Dermatomyositis, das Antisynthetase-Syndrom (ASyS) und die Immunvermittelte nekrotisierende Myopathie (IMNM) durch proximale Muskelschwäche gekennzeichnet, wobei jedoch einige Entitäten auch amyopathisch verlaufen können. Je nach vorliegender Antikörperart kann die Dermatomyositis jeweils unterschiedliche Manifestationen aufweisen, wobei die interstitielle Lungenerkrankung (ILD) als häufigste extramuskuläre Beteiligung zu erwähnen ist. Ein besonderes Augenmerk sollte auf das Tumorscreening bei Vorliegen entsprechender Risikofaktoren, wie beispielsweise bei anti-TIF1γ oder anti-NXP2-positivem Antikörperstatus bei Erwachsenen, gelegt werden. Die IMNMs sind bekanntermaßen mit einer Statinexposition assoziiert, allerdings sollte bedacht werden, dass eine Statinexposition nicht zwingend nötig ist, um eine IMNM zu entwickeln. Das ASyS präsentiert sich regulär mit einer Triade aus Myositis, Arthritis und ILD, welche jedoch nicht immer vorliegen muss. Therapeutisch unterscheiden sich die drei Gruppen initial nicht. Eine überbrückende Prednisolontherapie (je nach Schweregrad oral oder intravenös) wird initiiert und je nach Grunderkrankung und Organbeteiligung parallel eine steroidsparende Erhaltungstherapie etabliert. Eine ergänzende Physiotherapie sowie bei Bedarf auch Ergo- oder Logopädie sind wichtige Bausteine der Therapie und sollten frühzeitig begonnen werden.

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Risk factors for mortality in patients with anti-MDA5 antibody-positive dermatomyositis: A meta-analysis and systematic review

Cited by 17 publications

References 41 publications

Triple Positive for Anti-MDA5, Anti-EJ and Anti-Ro52 Antibodies Dermatomyositis Associated Interstitial Lung Disease：a case report

Triple Positive for Anti-MDA5, Anti-EJ and Anti-Ro52 Antibodies Dermatomyositis Associated Interstitial Lung Disease：a case report

Clinically amyopathic dermatomyositis associated with cutaneous ulcerations: a case-based review

Dermatomyositis, Antisynthetase-Syndrom und immunvermittelte nekrotisierende Myopathie

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