ArticleClinical rheumatology2019
Clinical features of patients with anti-melanoma differentiation-associated gene-5 antibody-positive dermatomyositis complicated by spontaneous pneumomediastinum.
Article in Clinical rheumatology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 43 citations in OpenAlex.
- Clinical characteristics and long-term prognosis of anti-MDA5-positive dermatomyositis: a comparative study across age groups.Orphanet journal of rare diseases · 2026Article
- Spontaneous pneumomediastinum and/or pneumothorax in anti-MDA5 dermatomyositis: a refined staging system.Respiratory research · 2025Article
- Different faces of anti-melanoma differentiation-associated gene 5 antibody-positive myositis.Reumatologia · 2025Article
- Case Report: Spontaneous pneumomediastinum in a patient with MDA5-positive dermatomyositis and severe pulmonary fibrosis.Frontiers in medicine · 2025Article
- Case Report: JAK inhibitors for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis with spontaneous pneumomediastinum.Frontiers in medicine · 2025Article
- Tension Pneumomediastinum in Anti-MDA5 Antibody-positive Dermatomyositis-associated Interstitial Lung Disease: A Case Report and Literature Review.Internal medicine (Tokyo, Japan) · 2024Review
- Anti-melanoma differentiation-associated gene-5 antibody-positive dermatomyositis with liver dysfunction: a warning sign of higher death risk.Clinical rheumatology · 2024Article
- Article
- Fever, Cutaneous Ulcers, and Arthritis Define the MDA5 Phenotype in Indian Patients with Idiopathic Inflammatory Myositis.Mediterranean journal of rheumatology · 2022Article
- Rapidly progressive interstitial lung disease in patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis: serial changes on HRCT.Emergency radiology · 2022Article
- Quantitative CT analysis of interstitial pneumonia in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis: a single center, retrospective study.Clinical rheumatology · 2022Article
- Spontaneous pneumomediastinum and cutaneous ulcers complicated in a patient with dermatomyositis and interstitial lung disease.Archives of rheumatology · 2022Article
- Rare complications of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis: Time to nip them in the bud.Frontiers in immunology · 2022Review
- A New Predictive Model for the Prognosis of MDA5Frontiers in medicine · 2022Article
- GAP Score and CA-153 Associated with One-Year Mortality in Anti-MDA-5 Antibody-Positive Patients: A Real-World Experience.Journal of clinical medicine · 2021Article
- A novel CT scoring method predicts the prognosis of interstitial lung disease associated with anti-MDA5 positive dermatomyositis.Scientific reports · 2021Article
- Adolescent-onset anti-MDA5 antibody-positive juvenile dermatomyositis with rapidly progressive interstitial lung disease and spontaneous pneumomediastinum: a case report and literature review.Pediatric rheumatology online journal · 2021Review
- Understanding and managing anti-MDA 5 dermatomyositis, including potential COVID-19 mimicry.Rheumatology international · 2021Review
- Interstitial Lung Disease in Anti-MDA5 Positive Dermatomyositis.Clinical reviews in allergy & immunology · 2021Review
- A Computed Tomography Radiomics-Based Prediction Model on Interstitial Lung Disease in Anti-MDA5-Positive Dermatomyositis.Frontiers in medicine · 2021Article
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Authors and funding
20 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundDermatomyositis (DM) with autoantibody against melanoma differentiation-associated gene-5 (MDA5) is characterized by elevated risk of rapidly progressive interstitial lung disease (RP-ILD) with a potentially fatal course. Pneumomediastinum (PNM) is a common pulmonary manifestation which accompanies ILD. However, the clinical features of the patients with anti-MDA5 antibody-positive DM who develop PNM remain unclear.
methodsWe retrospectively examined 31 patients with DM having anti-MDA5 antibody and compared the clinical features between patients with PNM (PMN(+)) (n = 11) and those without (PNM(-) (n = 20). In addition, we evaluated the treatment-related prognoses in PNM(+) group.
resultsCT score (total ground-glass opacity (GGO) score, P = 0.02; total fibrosis score, P = 0.02) before treatment, and mortality (P = 0.04) were significantly higher in PNM(+) group. The cumulative survival rate as assessed by Kaplan-Meier method was significantly lower for the PNM(+) group (P = 0.02). Among 11 PMN(+) patients, 9 patients (9/11, 81.8%) underwent intensive immunosuppression therapy for RP-ILD, and 5 patients (5/11, 45.5%) did not respond to it and died from the respiratory failure. At the time of diagnosis of PNM, nonsurvivors had worse liver function (ALT, P = 0.03; LDH, P = 0.01), worse respiratory status (A-aDO2, P = 0.01), and worse CT score (total GGO score, P < 0.01).
conclusionsA subgroup of patients with DM having anti-MDA5 antibody complicated by PNM as well as RP-ILD did respond to intensive immunosuppression therapy. Initial aggressive immunosuppressive therapy should be considered for these patients.Key Points• This study clearly demonstrate the presence of PNM was associated with elevated risk of death due to respiratory failure from RP-ILD among patients with DM having circulating anti-MDA5-antibody.•This study demonstrate evaluation of CT image may be helpful to find patients with better response to the intense immunosuppression therapy for the patients with DM having circulating anti-MDA5-antibody and PNM.
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