Therapeutic plasma exchange for clinically amyopathic dermatomyositis (<scp>CADM</scp>) associated with rapidly progressive interstitial pneumonia

Yamagata, Akira; Arita, Machiko; Tanaka, Ayaka; Tokioka, Fumiaki; Yoshida, Tomohiro; Nishimura, Keisuke; Ishida, Tadashi

doi:10.1002/jca.21824

Cited by 12 publications

(15 citation statements)

References 32 publications

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“… 4 , 5 Several case reports have described successful PE treatment for the patients with anti-MDA5 antibody-positive DM-associated RP-ILD in recent years. 3 , 6 - 11 However, as yet, standard protocols for the initiation/termination timing and the frequency of PE have not been established for these patients. Here, we present 2 cases of anti-MDA5 antibody-positive DM-associated RP-ILD for which PE was successfully executed.…”

Section: Introductionmentioning

confidence: 99%

“…PE has been recommended for the patients who are refractory to the immunosuppressive therapy, and several cases have been reported to respond to PE successfully. 3,[6][7][8][9][10][11] Moreover, the results of previous studies showed that PE significantly provided the higher survival rate for the patients who were refractory to immunosuppressive therapy. 3,10 Abe et al 3 showed that in patients refractory to immunosuppressive drugs, the 1-year survival rate of the PE group was significantly higher than that of the non-PE group (100% and 25%, respectively, P = .033).…”

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confidence: 99%

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Successful Treatment of Anti-MDA5 Antibody-Positive Dermatomyositis-Associated Rapidly Progressive-Interstitial Lung Disease by Plasma Exchange: Two Case Reports

Takahashi

Yoshida

Morimoto

et al. 2021

Clin Med Insights Case Rep

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Background: Patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM) are frequently accompanied by rapidly progressive-interstitial lung disease (RP-ILD). They are often refractory to intensive immunosuppressive therapy and have poor prognosis. Case presentation: A 73-year-old woman presented with fever, cold symptoms, and skin eruption for a month. She also exhibited muscle weakness on upper extremities slightly. The titer of anti-MDA5 antibody was extremely high, and computed tomography showed ground glass opacity and reticular shadows in the lungs. She was diagnosed as anti-MDA5 antibody-positive classical DM-associated RP-ILD and treated with intensive immunosuppressive therapy. However, the titer of anti-MDA5 antibody did not decrease satisfactorily, and plasma exchange was alternatively initiated. The titer decreased remarkably, and she obtained disease remission. Similarly, a 63-year-old woman presented with stiffness of the neck and hands, fever and cough. She was also diagnosed as anti-MDA5 antibody-positive classical DM-associated RP-ILD, because she had skin eruptions, slight muscle weakness, an elevation in anti-MDA5 antibody, and RP-ILD. She was unresponsive to intensive immunosuppressive therapy, but plasma exchange successfully improved the titer of anti-MDA5 antibody, the symptoms, and the findings of computed tomography. Conclusions: Although anti-MDA5 antibody-positive DM-associated RP-ILD has a high mortality rate, this report suggests the usefulness of plasma exchange to improve the prognosis.

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Section: Introductionmentioning

confidence: 99%

mentioning

confidence: 99%

Successful Treatment of Anti-MDA5 Antibody-Positive Dermatomyositis-Associated Rapidly Progressive-Interstitial Lung Disease by Plasma Exchange: Two Case Reports

Takahashi

Yoshida

Morimoto

et al. 2021

Clin Med Insights Case Rep

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“…Several recent reports have demonstrated the efficacy of PE in RP-ILD with anti-MDA5 Ab combined with intensive immunosuppressive treatment [ [5] , [6] , [7] ]. PE is usually performed in RP-ILD patients exhibiting resistance to immunosuppressive agents.…”

Section: Discussionmentioning

confidence: 99%

“…Plasma exchange (PE) is a therapeutic procedure used to treat a variety of diseases that involves the bulk removal of pathogenic substances, such as pathogenic antibodies, immune complexes, and cytokines with enhanced macrophage/monocyte function [ 4 ]. Recent reports suggest that PE is effective for steroid-refractory interstitial pneumonia associated with anti-MDA5 Ab [ [5] , [6] , [7] ]. However, the indication criteria, timing, and interval for PE in RP-ILD with anti-MDA5 Ab have not yet been established.…”

Section: Introductionmentioning

confidence: 99%

Early intervention of plasma exchange combined with intensive immunosuppressive treatment for anti-MDA-5 antibody–positive rapidly progressive interstitial pneumonia: Two case reports

Ishiwari

Togashi

Takoi

et al. 2021

Respiratory Medicine Case Reports

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Background Anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) has to be reported to often cause rapidly progressive interstitial lung disease (RP-ILD) especially in East Asian countries. Even with the recommended rapid administration of immunosuppressive agents with high-dose corticosteroids, intravenous pulse cyclophosphamide, and calcineurin inhibitors, the prognosis of anti-MDA5 Ab–related RP-ILD is poor. Plasma exchange (PE) has been reported to be effective for steroid-refractory RP-ILD with anti-MDA5 Ab. However, the timing, frequency, and interval of PE for the treatment of RP-ILD with anti-MDA5 Ab have not yet been established. Case presentation We report two cases of RP-ILD with anti-MDA5 Ab treated by early intervention of PE combined with immunosuppressive treatment. Blood biomarkers including titers of anti-MDA5 Ab, serum KL-6 and ferritin were promptly decreased after each session of PE. Clinical symptoms, oxygenation and chest computed tomography abnormalities were completely improved after immunosuppressive treatment with PE. Conclusion Early intervention of PE combined with immunosuppressive treatment may prevent the development to lethal severe respiratory failure in RP-ILD with anti-MDA5 Ab.

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“…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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Therapeutic plasma exchange for clinically amyopathic dermatomyositis (CADM) associated with rapidly progressive interstitial pneumonia

Cited by 12 publications

References 32 publications

Successful Treatment of Anti-MDA5 Antibody-Positive Dermatomyositis-Associated Rapidly Progressive-Interstitial Lung Disease by Plasma Exchange: Two Case Reports

Successful Treatment of Anti-MDA5 Antibody-Positive Dermatomyositis-Associated Rapidly Progressive-Interstitial Lung Disease by Plasma Exchange: Two Case Reports

Early intervention of plasma exchange combined with intensive immunosuppressive treatment for anti-MDA-5 antibody–positive rapidly progressive interstitial pneumonia: Two case reports

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

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