Noninvasive positive pressure ventilator deteriorates the outcome of pneumomediastinum in anti-MDA5 antibody-positive clinically amyopathic dermatomyositis

Zhou, Mengmeng; Yan, Yizhong; Yan, Ninghui; Lian, Xin‐Yue; Bao, Chunde; Guo, Qiang

doi:10.1007/s10067-019-04918-2

Cited by 17 publications

(23 citation statements)

References 41 publications

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“…erefore, it is imperative that complications of all MSA be monitored early, regardless of strength, as even a concurrent, weakly positive anti-MDA5 Ab ultimately led to bilateral lung transplantation. Prompt diagnosis and intervention is crucial in MDA5-SPM, as mortality in anti-MDA5-positive cases with and without SPM has been reported to be 60% and 37%, respectively [15,16]. Early aggressive immunosuppressive therapy and ECMO have been the mainstay treatment of MDA5-SPM.…”

Section: Discussionmentioning

confidence: 99%

“…Early aggressive immunosuppressive therapy and ECMO have been the mainstay treatment of MDA5-SPM. Supplementary ventilation options such as noninvasive positive pressure ventilation (NPPV) are futile given NPPV has been shown to aggravate mediastinal emphysema [15]. In one MDA5-SPM study, mortality was 100% in patients treated with NPPV in comparison to 49% in patients treated without NPPV at 100 days [15].…”

Section: Discussionmentioning

confidence: 99%

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Spontaneous Pneumomediastinum due to Anti-Melanoma Differentiation-Associated Protein 5 Requiring a Bilateral Lung Transplant

Jhajj

Yeung

2021

Case Reports in Rheumatology

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Anti-melanoma differentiation-associated protein 5 (anti-MDA5) is a subset of dermatomyositis associated with respiratory complications, in which rapidly progressive interstitial lung disease (RPILD) is commonly cited, and spontaneous pneumomediastinum (SPM) is a rare complication. In medical literature, aggressive immunosuppressive therapy has been the mainstay of anti-MDA5-associated SPM management. Here, we report the first MDA5 case with SPM which was successfully treated with a double-lung transplant. We present a 48-year-old male who presented with multiple constitutional symptoms such as fevers, weight loss, malaise, and arthralgias, in association with erythroderma over the ears and fingers. Imaging of the chest demonstrated peripheral airspace disease, and myositis-specific serology returned positive for anti-Jo1 (medium-positive), anti-Ro52 (high-positive), and anti-MDA5 (weak-positive) autoantibodies. Therefore, the patient was begun on immunosuppressive therapy as the leading diagnosis included autoimmune myositis, possibly antisynthetase syndrome with interstitial lung disease (ILD). A year later, the patient presented with progressive shortness of breath, widespread macular erythematous facial rash, and new erythematous ulcerations over the fingertips. Imaging demonstrated a new SPM at this juncture. As the patient’s respiratory status continued to decline despite the use of immunosuppressive agents, a double-lung transplant was performed. Therefore, we propose that lung transplantation should be considered early in MDA5-SPM.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Spontaneous Pneumomediastinum due to Anti-Melanoma Differentiation-Associated Protein 5 Requiring a Bilateral Lung Transplant

Jhajj

Yeung

2021

Case Reports in Rheumatology

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“…The probable explanation is that those fibrotic features are less common in this rapid progressive disease and likely to be presented, if it happens, in later stage instead of baseline. The same notion apparently holds true for the presence of PNM, which is a known severity indicator rather than a baseline predictor 17 .…”

Section: Discussionmentioning

confidence: 91%

A novel CT scoring method predicts the prognosis of interstitial lung disease associated with anti-MDA5 positive dermatomyositis

Zhang

et al. 2021

Sci Rep

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Anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated interstitial lung disease (MDA5+ DM-ILD) is a life-threatening disease. This study aimed to develop a novel pulmonary CT visual scoring method for assessing the prognosis of the disease, and an artificial intelligence (AI) algorithm-based analysis and an idiopathic pulmonary fibrosis (IPF)-based scoring were conducted as comparators. A retrospective cohort of hospitalized patients with MDA5+ DM-ILD was analyzed. Since most fatalities occur within the first half year of the disease course, the primary outcome was the six-month all-cause mortality since the time of admission. A ground glass opacity (GGO) and consolidation-weighted CT visual scoring model for MDA5+ DM-ILD, namely ‘MDA5 score’, was then developed with C-index values of 0.80 (95%CI 0.75–0.86) in the derivation dataset (n = 116) and 0.84 (95%CI 0.71–0.97) in the validation dataset (n = 57), respectively. While, the AI algorithm-based analysis, namely ‘AI score’, yielded C-index 0.78 (95%CI 0.72–0.84) for the derivation dataset and 0.77 (95%CI 0.64–0.90) for the validation dataset. These findings suggest that the newly derived ‘MDA5 score’ may serve as an applicable prognostic predictor for MDA5+ DM-ILD and facilitate further clinical trial design. The AI based CT quantitative analysis provided a promising solution for ILD evaluation.

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“…The probable explanation is that those brotic features are less common in this rapid progressive disease and likely to be presented, if it happens, in later stage instead of baseline. The same notion apparently holds true for the presence of PNM, which is a known severity indicator rather than a baseline predictor (20).…”

Section: Discussionmentioning

confidence: 91%

MDA5 score--A Novel Lung Computed Tomography Scoring Method for Anti-Melanoma Differentiation-associated Gene 5-positive Dermatomyositis

Zhang

et al. 2021

Preprint

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Background: Anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis (DM)-associated interstitial lung disease (ILD) is a life-threatening disease with a 6-month mortality as high as 50%. Evaluation of pulmonary high-resolution computed tomography (HRCT) is crucial in the prognostic prediction. This study aimed to develop a novel CT visual scoring method for anti-MDA5 positive DM-ILD.Methods: A prospective cohort of hospitalized patients with anti-MDA5 positive DM-ILD was analyzed, and was further divided into a derivation dataset and a validation dataset. The primary outcome was the six-month all-cause mortality since the time of admission. Three components of baseline pulmonary CT images, i.e., ground glass opacity (GGO), consolidation and fibrosis were semi-quantitatively calculated in different lung lobes. The multivariable COX proportional hazards model was used to identify independent prognostic risk factors and corresponding coefficients, based upon which a scoring model was constructed. In addition, an artificial intelligence (AI) algorithm-based analysis and an idiopathic pulmonary fibrosis (IPF)-based scoring were conducted as comparators. The prediction accuracy of different methods was measured and compared by Harrell concordance index (C-index). Results: Overall, 173 eligible patients were included. A novel GGO and consolidation-weighted CT visual scoring model for anti-MDA5 positive DM-ILD, namely ‘MDA5 score’, was developed with C-index values of 0.80 (95%CI 0.75-0.86) in the derivation dataset (n=116) and 0.84 (95%CI 0.71-0.97) in the validation dataset (n=57), respectively. While, the AI algorithm-based analysis, namely ‘AI score’, yielded C-index 0.78 (95%CI 0.72-0.84) for the derivation dataset and 0.77 (95%CI 0.64-0.90) for the validation dataset. ‘MDA5 score’ outperformed and IPF-based scoring (‘IPF score’) with respect to discrimination and clinical usefulness.Conclusions: The novel derived ‘MDA5 score’ may serve as an applicable prognostic predictor for anti-MDA5 positive DM-ILD and facilitate further clinical trial design. The AI based CT quantitative analysis from COVID-19 provided a promising solution for ILD evaluation.

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Noninvasive positive pressure ventilator deteriorates the outcome of pneumomediastinum in anti-MDA5 antibody-positive clinically amyopathic dermatomyositis

Cited by 17 publications

References 41 publications

Spontaneous Pneumomediastinum due to Anti-Melanoma Differentiation-Associated Protein 5 Requiring a Bilateral Lung Transplant

Spontaneous Pneumomediastinum due to Anti-Melanoma Differentiation-Associated Protein 5 Requiring a Bilateral Lung Transplant

A novel CT scoring method predicts the prognosis of interstitial lung disease associated with anti-MDA5 positive dermatomyositis

MDA5 score--A Novel Lung Computed Tomography Scoring Method for Anti-Melanoma Differentiation-associated Gene 5-positive Dermatomyositis

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