Clinical effects of direct hemoperfusion using a polymyxin B-immobilized fiber column in clinically amyopathic dermatomyositis-associated rapidly progressive interstitial pneumonias

Okabayashi, Hiroko; Ichiyasu, Hidenori; Hirooka, Sayuri; Akaike, Kimitaka; Kojima, Kensuke; Jodai, Takayuki; Sakamoto, Yasuhisa; Ideguchi, Hideharu; Hamada, Shohei; Yoshida, Chieko; Hirosako, Susumu; Okamoto, Shinichiro; Kohrogi, Hirotsugu

doi:10.1186/s12890-017-0479-2

Cited by 20 publications

(13 citation statements)

References 43 publications

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“…[28][29][30] The presence of anti-MDA5 antibody and groundglass opacity on HRCT images were related closely to a high 90-day mortality rate 29 ; both ferritin and LDH levels in the nonsurvivor group were significantly higher than those in the survivor group. 30,31 A diagnosis of ADM with older age, acute/subacute ILD, and lower estimated FVC were associated independently with unfavorable survival. 11 Anti-PL-7 antibody is also considered a risk factor for long-term pulmonary deterioration in patients with ADM-ILD.…”

Section: Discussionmentioning

confidence: 95%

Mortality Risk Prediction in Amyopathic Dermatomyositis Associated With Interstitial Lung Disease

Lian

Zou

Guo

et al. 2020

Chest

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BACKGROUND: The prognosis of amyopathic dermatomyositis (ADM)-associated interstitial lung disease (ILD) is poor. A mortality risk score model is needed to predict survival in patients with ADM-ILD and to guide clinical treatment. RESEARCH QUESTION: How to identify patients with ADM-ILD who are at high risk and to predict patient outcome based on a risk stratification model? STUDY DESIGN AND METHODS: We evaluated 207 patients with ADM-ILD in this prospective inception study. We used a multivariable Cox proportional hazards model to identify the independent prognostic risk factors and created a risk score model according to patient data from January 2012 to December 2016. We used the index of prediction accuracy that uses the Brier score to reflect both discrimination and calibration of the model. The model was validated in an independent group of patients from January 2017 to June 2018. RESULTS: We developed a combined risk score, the FLAIR score, that included the following values and scores: ferritin (<636 ng/mL, 0; $636 ng/mL, 2), lactate dehydrogenase (<355 U/ L, 0; $355 U/L, 2), antimelanoma differentiation-associated gene 5 antibody (negative, 0; þ, 2; þþ, 3; þþþ, 4), high-resolution CT imaging score (<133, 0; $133, 3), and rapidly progressive ILD (RPILD) (non-RPILD, 0; RPILD, 2). We divided patients into three risk groups according to the FLAIR score: low, 0 to 4; medium, 5 to 9; and high, 10 to 13. In both discovery and validation cohorts, high-risk patients had significantly higher mortality rates than low-and medium-risk patients (P < .001). INTERPRETATION: The FLAIR risk score model could help to predict survival in patients with ADM-ILD and to guide further clinical research on risk-based treatment.

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Section: Discussionmentioning

confidence: 95%

Mortality Risk Prediction in Amyopathic Dermatomyositis Associated With Interstitial Lung Disease

Lian

Zou

Guo

et al. 2020

Chest

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“…Notably, recent case reports have indicated the efficacy of plasmapheresis in patients with anti–MDA‐5–positive rapidly progressive ILD . Polymyxin B–immobilized fiber column direct hemoperfusion has also been suggested as potentially effective for ILD in CADM or anti–MDA‐5–positive rapidly progressive ILD , although the effect was controversial . In the future, prospective studies comparing patients who undergo plasmapheresis and those who do not are necessary.…”

Section: Discussionmentioning

confidence: 99%

Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High‐Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti–Melanoma Differentiation–Associated Gene 5–Positive Dermatomyositis

Tsuji

Nakashima

Hosono

et al. 2020

Arthritis & Rheumatology

238

168

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Objective Interstitial lung disease (ILD) accompanied by anti–melanoma differentiation–associated gene 5 (anti–MDA‐5)–positive dermatomyositis (DM) is often rapidly progressive and associated with poor prognosis. Because there is no established treatment, we undertook this study to prospectively evaluate the efficacy and safety of a combined immunosuppressive regimen for anti–MDA‐5–positive DM patients with ILD. Methods Adult Japanese patients with new‐onset anti–MDA‐5–positive DM with ILD (n = 29) were enrolled at multiple study centers from 2014 to 2017. They were treated with a regimen of high‐dose glucocorticoids (GCs), tacrolimus, and intravenous cyclophosphamide (IV CYC). Plasmapheresis was used if a patient's condition worsened after the regimen started. The primary end point was 6‐month survival, which was compared between this group of patients and a historical control group (n = 15) consisting of anti–MDA‐5–positive DM patients with ILD who received step‐up treatment (high‐dose GC and stepwise addition of immunosuppressant). Secondary end points were 12‐month survival rate, adverse events, and changes in laboratory data. Results The combined immunosuppressive regimen group showed significantly higher 6‐month survival rates than the step‐up treatment group (89% versus 33%; P < 0.0001). Over a period of 52 weeks, improvements in anti–MDA‐5 titers, serum ferritin levels, vital capacity, and chest high‐resolution computed tomography scores were observed. The combined immunosuppressive regimen group received IV CYC nearly 20 days earlier with shorter intervals and tended to receive plasmapheresis more often than patients undergoing step‐up treatment. Cytomegalovirus reactivation was frequently observed over 52 weeks. Conclusion A combined immunosuppressive regimen is effective for anti–MDA‐5–positive DM patients with ILD. Plasmapheresis can be used for additional effect in intractable disease. Patients should be carefully monitored for opportunistic infections during treatment.

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“…As for CADM-RPIP treated with PMX-DHP, a higher PaO 2 /fraction of inspiratory oxygen (FiO 2 ) ratio and surfactant protein-D level, lower LDH and ferritin levels, higher platelet counts, and anti-MDA5 Ab negativity were identified as important prognostic markers in a previous study. 27 We could not evaluate prognostic factors for CADM-RPIP treated with TPE, since all patients were survivors. In anti-MDA5 Ab-positive DM-IP patients, an initial serum ferritin level ≥ 450 ng/mL, P[A-a] O 2 ≥ 30 mmHg, and GGA ≥5% for the right middle lobe of the lung appear to represent factors associated with poor prognosis.…”

Section: Discussionmentioning

confidence: 99%

“…Regarding the removal of the pathogenic factors of CADM-RPIP, the efficacy of polymyxin B-immobilized fiber column direct hemoperfusion (PMX-DHP) and DNA immunoadsorption (IA) has also been reported. [26][27][28] However, data are currently insufficient to support the use of PMX-DHP for RPIP, according to a recent systematic review. 29 In addition, the fact that the incidence of cartridge clotting of PMX-DHP is not low represents a major problem.…”

Section: Discussionmentioning

confidence: 99%

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

Yamagata

Arita

Tanaka

et al. 2020

J of Clinical Apheresis

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Background Patients with clinically amyopathic dermatomyositis (CADM) with anti‐melanoma differentiation‐associated gene 5 antibody (anti‐MDA5 Ab) frequently develop rapidly progressive interstitial pneumonia (RPIP), often with fatal outcomes. Therapeutic plasma exchange (TPE) has been reported as effective against CADM‐RPIP refractory to conventional immunosuppressive therapy. However, the detailed mechanisms by which TPE improves disease activity of CADM‐RPIP remain unclear. Aim To elucidate the clinical and demographic characteristics of patients with anti‐MDA5 Ab‐positive CADM‐RPIP treated with TPE and to analyze changes in laboratory findings before, during, and after TPE. Materials & Methods Patients hospitalized for CADM‐RPIP and treated with TPE in 2017 and 2018 were analyzed retrospectively. Results Three patients were successfully treated with TPE, with good tolerance. Anti‐MDA5 Ab titers decreased significantly over the course of TPE. Conclusion We emphasize that TPE could represent an effective treatment option for CADM‐RPIP refractory to traditional therapy. Removal of anti‐MDA5 Ab and other pathogenic factors may facilitate favorable outcomes.

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Clinical effects of direct hemoperfusion using a polymyxin B-immobilized fiber column in clinically amyopathic dermatomyositis-associated rapidly progressive interstitial pneumonias

Cited by 20 publications

References 43 publications

Mortality Risk Prediction in Amyopathic Dermatomyositis Associated With Interstitial Lung Disease

Mortality Risk Prediction in Amyopathic Dermatomyositis Associated With Interstitial Lung Disease

Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High‐Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti–Melanoma Differentiation–Associated Gene 5–Positive Dermatomyositis

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

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