Lung transplantation for anti-MDA5-positive dermatomyositis-associated rapid progressive interstitial lung disease: report of two cases and review of the literature

Lian, Qiaoyan; Chen, Ao; Zhang, Jianheng; Xu, Xin; Huang, Danxia; Luo, Qun; He, Jianxing; Chen, Ju

doi:10.1007/s10067-022-06422-6

Cited by 6 publications

(7 citation statements)

References 23 publications

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“…Diffuse ground-glass opacities appeared in the HRCT images of both the native and transplanted lungs alongside elevated anti-MDA-5 Ab titers in the early post-lung transplantation phase. These cases suggest that achieving a decline in, or complete clearance of, the anti-MDA-5 Ab may be necessary to prevent the recurrence of DM and RP-ILD in patients after transplantation [59].…”

Section: Other Therapies Described In the Literaturementioning

confidence: 99%

“…Extracorporeal membrane oxygenation can transitionally support patients waiting for a clinical response to intensive immunosuppressive therapy or lung transplantation [57,59]. It is regarded as the ultimate therapeutic recourse for hypoxic respiratory failure when standard treatments prove ineffective, serving exclusively as an interim measure until a definitive resolution of the underlying cause of respiratory failure can be implemented [43].…”

Section: Jak Inhibitorsmentioning

confidence: 99%

“…Some studies report survival with a good prognosis of more than 12 years post lung transplantation [56]. However, complications including infections and DM flare with RP-ILD post-transplant have also been described [57,59]. The recurrence of disease post-transplantation and monitoring guidelines (imaging and Ab measurement) are intriguing topics that require larger studies, preferably RCTs with multi-center collaborations.…”

Section: Jak Inhibitorsmentioning

confidence: 99%

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A Review of MDA-5 Dermatomyositis and Associated Interstitial Lung Disease

Bhandari,

Zickuhr,

Baral

et al. 2024

Rheumato

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Anti-melanoma differentiation-associated gene 5 (MDA-5) dermatomyositis (DM) is noteworthy for its association with rapidly progressive interstitial lung disease (RP-ILD), vasculopathy, and distinctive cutaneous features. First identified in a Japanese cohort in 2005, MDA-5 DM carries a significant mortality risk, emphasizing the crucial need for early diagnosis. This review explores the pathogenesis, clinical presentation, diagnosis, management, and prognosis of MDA-5 DM and ILD and includes new research and recommendations regarding disease management.

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Section: Other Therapies Described In the Literaturementioning

confidence: 99%

Section: Jak Inhibitorsmentioning

confidence: 99%

Section: Jak Inhibitorsmentioning

confidence: 99%

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A Review of MDA-5 Dermatomyositis and Associated Interstitial Lung Disease

Bhandari,

Zickuhr,

Baral

et al. 2024

Rheumato

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“…Finally, extracorporeal membrane oxygenation (ECMO) bridging to lung transplantation may serve as a life-saving salvage therapy for MDA5-associated DM patients with refractory RP-ILD based on several case reports and series. 124 , 135 …”

Section: Unmet Needsmentioning

confidence: 99%

Dermatomyositis: Practical Guidance and Unmet Needs

Cassard,

Seraly,

Riegert

et al. 2024

ITT

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Dermatomyositis is a heterogeneous idiopathic inflammatory myopathy associated with various cutaneous manifestations and variable presence of myositis, interstitial lung disease, and other visceral organ involvement. An accurate diagnosis of dermatomyositis requires correlating clinical examination findings with serological and histological findings. Familiarity with pathognomonic and common cutaneous manifestations of dermatomyositis, which are highlighted here, can be especially helpful in making an accurate diagnosis. Additionally, evaluating patients for presence of myositis-specific autoantibodies can further support or refute a dermatomyositis diagnosis. When present, myositis-specific autoantibodies can also help guide workups for various dermatomyositis-associated manifestations, as each is associated with relatively distinct clinical characteristics. Evaluating patients for various systemic manifestations often relies on expert opinion recommendations; however, societal guideline statements concerning the evaluation of some manifestations have recently been described. Although malignancy-associated dermatomyositis is a well-accepted subtype, there is limited evidence to support extensive malignancy screening has a favorable benefit–risk ratio in most dermatomyositis patients. However, recent research has uncovered novel associations between dermatomyositis and malignancy, suggesting the possibility of identifying high-risk subsets of dermatomyositis patients in whom malignancy screening may have a high value. Treatment for dermatomyositis has remained largely unchanged over the past several decades. Although many dermatomyositis patients can be effectively treated with current options, either as monotherapy or with combination regimens, there is a need for more targeted and effective DM therapies, in general, and for MDA5(+) dermatomyositis-associated rapidly progressive interstitial lung disease. Fortunately, significant current and emerging research activities evaluating various novel medications for dermatomyositis provide hope for exciting future advances in patients with this intriguing immune-mediated disease.

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“…Lung transplantation can be considered when patients develop severe respiratory failure despite intensive immunosuppressive therapy. Eleven cases with MDA5-DM have been reported, and all of them underwent bilateral lung transplantation [38,[67][68][69][70][71][72]. In addition, 10 of 11 cases received extracorporeal membrane oxygenation as a bridging therapy before lung transplantation.…”

Section: Lung Transplantationmentioning

confidence: 99%

New therapies in anti-MDA5 antibody-positive dermatomyositis

Yasui,

Iwamoto,

Furuta

2023

Current Opinion in Rheumatology

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Purpose of review This review focuses on treatments for anti-MDA5 antibody-positive dermatomyositis (MDA5-DM), which is a subgroup of dermatomyositis and characterized by frequent rapidly progressive interstitial lung disease and the high mortality rate. Despite conventional immunosuppressive therapies, there are still refractory cases. Newer treatment options are needed. Recent findings The triple combination therapy (high-dose glucocorticoids, calcineurin inhibitor, and intravenous cyclophosphamide) improved patient survival compared to high-dose glucocorticoids and step-wise addition of the immunosuppressants. The triple therapy now has been widely used, but there are still refractory cases. In addition to the conventional-type immunosuppressants, recently the efficacy of Janus kinase inhibitors, biologic agents such as rituximab, plasma exchange, and polymyxin B perfusion for refractory MDA5-DM patients have been reported. However, the majority of those reports regarding new treatments are limited to case series, retrospective studies, and small single-arm studies. Adding antifibrotic drugs to immunosuppressive therapies might have some ancillary benefits. Summary Several new therapies for MDA5-DM patients have emerged, although the optimal use of those therapies is still unknown. Further research and evidence accumulation will be needed. It is also noted that the intensive immunosuppressive therapies are associated with the higher infection risk.

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Lung transplantation for anti-MDA5-positive dermatomyositis-associated rapid progressive interstitial lung disease: report of two cases and review of the literature

Cited by 6 publications

References 23 publications

A Review of MDA-5 Dermatomyositis and Associated Interstitial Lung Disease

A Review of MDA-5 Dermatomyositis and Associated Interstitial Lung Disease

Dermatomyositis: Practical Guidance and Unmet Needs

New therapies in anti-MDA5 antibody-positive dermatomyositis

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