Efficiency of Therapeutic Plasma-Exchange in Acute Interstitial Lung Disease, Associated With Polymyositis/Dermatomyositis Resistant to Glucocorticoids and Immunosuppressive Drugs: A Retrospective Study

Ning, Y.N.; Yang, Ge; Yan, Sun; Chen, Shiju; Liu, Yuan; Shi, Guixiu

doi:10.3389/fmed.2019.00239

Cited by 18 publications

(12 citation statements)

References 37 publications

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“…Although it also decreased the mean prednisone dose over time in patients with AS, roughly 53% of patients experienced side effects. Similarly, rare case reports have discussed the use of therapeutic plasma exchange for rapidly progressive ILD, refractory to treatment and have demonstrated favorable results with improvement of myositis and respiratory status with decreasing antibody index (64,65). These aforementioned medications take various routes to suppress normal immune function in an effort to combat aberrant pathogenic autoimmunity.…”

Section: Treatmentmentioning

confidence: 99%

A narrative review of interstitial lung disease in anti-synthetase syndrome: a clinical approach

et al. 2021

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Anti-synthetase syndrome (AS) is a rare autoimmune disorder characterized by the presence of aminoacyl-transfer RNA synthetase antibodies in conjunction with clinical features such as interstitial lung disease (ILD), Raynaud's phenomenon, nonerosive arthritis, and myopathy. AS distinguishes itself from other inflammatory myopathies by its significant lung involvement and rapidly progressive interstitial lung disease (AS-ILD), therefore the management of AS-ILD requires careful clinical, serologic and radiologic assessment. Glucocorticoids are considered the mainstay of therapy; however, additional immunosuppressive agents are often required to achieve disease control. Patient prognosis is highly dependent on early diagnosis and symptom recognition as the antibody profile is thought to influence therapy response. Since progressive ILD is the leading cause of morbidity and mortality, this review will discuss the clinical approach to patient with suspected AS, with particular emphasis on diagnosis and management of AS-ILD.

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Section: Treatmentmentioning

confidence: 99%

A narrative review of interstitial lung disease in anti-synthetase syndrome: a clinical approach

et al. 2021

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“…[15][16][17]30 An additional seven cases of PM/DM with ILD are noted in which 5 of 7 demonstrated improvement after TPE. 14 TPE has been reported to be effective in CADM associated with MDA5 and aggressive ILD, a disorder with poor prognosis. 2,14,15,[18][19][20][21][22][23][24][25][26][27][28][29]31,[37][38][39][40][41] In the peerreviewed medical literature, a total of 56 patients with CADM undergoing TPE or plasmapheresis, single or DFPP, for which 41 demonstrated clinical improvement in pulmonary function (Table 3).…”

Section: Discussionmentioning

confidence: 99%

“…8,12 Non-steroidal immunosuppressants such as cyclophosphamide, mycophenolate mofetil, cyclosporine, and rituximab are used as steroid-sparing agents or in combination with steroids. 8,12,13 Recent case reports demonstrate clinical and radiographic improvement after therapeutic plasma exchange (TPE) [14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29] or column filtration plasmapheresis in IIMs with ILD resistant to medical management. 30,31 DM and PM, two IIMs with significant overlap with AS, were category IV indications for TPE according to the 2016 American Society for Apheresis (ASFA) guidelines, but were retired in the 2019 guidelines.…”

Section: Introductionmentioning

confidence: 99%

Therapeutic plasma exchange for steroid refractory idiopathic inflammatory myopathies with interstitial lung disease

Thompson

Bobr

Juskewitch

et al. 2022

J of Clinical Apheresis

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Background Idiopathic inflammatory myopathies (IIMs) encompass many rheumatologic diseases characterized by inflammatory muscle disease, typically unified by proximal muscle weakness. A subset of patients with IIM present with interstitial lung disease (ILD) with identifiable antibodies such as in anti‐synthetase syndrome (AS) with antibodies to aminoacyl‐tRNA synthetases, and clinically amyopathic dermatomyositis (CADM) with anti‐melanoma differentiation‐associated protein 5 (MDA5). Recent case reports demonstrate response to therapeutic plasma exchange (TPE) or column filtration plasmapheresis in IIM with ILD resistant to medical management. We present our experience with eight patients with IIM with ILD undergoing TPE at a large US‐based hospital system. Patient characteristics Eight patients with IIM with ILD were treated with TPE over the last 10 years. The therapy consisted of 5‐7 one plasma volume exchanges every other day to daily. Seven of eight patients had identifiable antibodies. Results Following completion of TPE, seven of eight demonstrated improvement in pulmonary function despite lack of improvement of pulmonary function with standard therapy. Conclusion In antibody‐mediated, treatment refractory IIM with ILD, TPE may be a viable intervention. This is a disease for which the role of apheresis is evolving. Clinical trial registration Not application.

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“…Some other examples are supporting the possible benefit of plasmapheresis as an additional therapy in patients with refractory disease [ 48 – 51 ]. Nevertheless, the variety of local protocols and fluid replacement regimes used might yield different results and bias the evaluation of the efficacy of this procedure.…”

Section: How To Identify the Best Therapeutic Strategy?mentioning

confidence: 99%

Pharmacologic Treatment of Anti-MDA5 Rapidly Progressive Interstitial Lung Disease

Selva-O’Callaghan

Romero-Bueno

Trallero-Araguás

et al. 2021

Curr Treat Options in Rheum

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Purpose of the Review Idiopathic inflammatory myopathies are a heterogeneous group of autoimmune disorders. The presence of different autoantibodies allows clinicians to define distinct phenotypes. Antibodies against the melanoma differentiation-associated protein 5 gene, also called anti-MDA5 antibodies, are associated with a characteristic phenotype, the clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease. This review aims to analyze the different pharmacological options for the treatment of rapidly progressive interstitial lung disease in patients with anti-MDA5 antibodies. Recent Findings Evidence-based therapeutic recommendations suggest that the best initial approach to treat these patients is an early combination of immunosuppressive drugs including either glucocorticoids and calcineurin inhibitors or a triple therapy adding intravenous cyclophosphamide. Tofacitinib, a Janus kinase inhibitor, could be useful according to recent reports. High ferritin plasma levels, generalized worsening of pulmonary infiltrates, and ground-glass opacities should be considered predictive factors of a bad outcome. In this scenario, clinicians should consider rescue therapies such as therapeutic plasma exchange, polymyxin-B hemoperfusion, veno-venous extracorporeal membrane oxygenation, or even lung transplantation. Summary Combined immunosuppressive treatment should be considered the first-line therapy for patients with anti-MDA5 rapidly progressive interstitial lung disease. Aggressive rescue therapies may be useful in refractory patients.

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Efficiency of Therapeutic Plasma-Exchange in Acute Interstitial Lung Disease, Associated With Polymyositis/Dermatomyositis Resistant to Glucocorticoids and Immunosuppressive Drugs: A Retrospective Study

Cited by 18 publications

References 37 publications

A narrative review of interstitial lung disease in anti-synthetase syndrome: a clinical approach

A narrative review of interstitial lung disease in anti-synthetase syndrome: a clinical approach

Therapeutic plasma exchange for steroid refractory idiopathic inflammatory myopathies with interstitial lung disease

Pharmacologic Treatment of Anti-MDA5 Rapidly Progressive Interstitial Lung Disease

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