Evidence map›Paper›PMID 39915831›Full record

ArticleOrphanet journal of rare diseases2025

Clinical characteristics and prognosis of amyopathic dermatomyositis patients with interstitial lung disease: insights from a retrospective cohort.

Yanan Ying, Tingting Wu, Long Wang, Yun Zhang, Yiming Yu, Zaichun Deng, Qunli Ding

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In one paragraph

Article in Orphanet journal of rare diseases, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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1citing papers in PubMed
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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

7 authors.

Yanan YingDepartment of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo University, 59 Liuting Street, Haishu District, Ningbo, Zhejiang, China.
Tingting WuDepartment of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo University, 59 Liuting Street, Haishu District, Ningbo, Zhejiang, China. wutingting519128@163.com.ORCID http://orcid.org/0000-0002-3707-2425
Long WangRheumatology Department, The First Affiliated Hospital of Ningbo University, Ningbo, Zhejiang, China.
Yun ZhangDepartment of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo University, 59 Liuting Street, Haishu District, Ningbo, Zhejiang, China.
Yiming YuDepartment of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo University, 59 Liuting Street, Haishu District, Ningbo, Zhejiang, China.
Zaichun DengDepartment of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo University, 59 Liuting Street, Haishu District, Ningbo, Zhejiang, China.
Qunli DingDepartment of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo University, 59 Liuting Street, Haishu District, Ningbo, Zhejiang, China. dingqunli@nbu.edu.cn.

Funding

Natural Science Foundation of Ningbo 2021J243
6 · The paper itself

Abstract

introductionThe diagnosis of amyopathic dermatomyositis with interstitial lung disease (ADM-ILD) is challenging due to the lack of typical skin features and overlapping syndromes. We aimed to determine the characteristics and prognosis of patients with ADM-ILD to further guide their clinical management.

methodsA retrospective cohort study comprising 190 Chinese patients diagnosed with interstitial lung disease (ILD) was conducted. Patients were stratified into four groups using the Sontheimer criteria and predominant high-resolution computed tomography (HRCT) patterns. Demographic features, clinical presentation, laboratory parameters, duration of ILD, and follow-up data were analysed.

resultsThere were significant differences in the clinical parameters among the 190 patients with ILD in the amyopathic dermatomyositis (ADM, n = 69) and control (n = 121) groups. The ADM with nonspecific interstitial pneumonia (NSIP) group (n = 46) presented increased haemoglobin (125.93 ± 12.91 g/L, p = 0.005), creatine kinase-MB (15.19 ± 8.58 U/L, p < 0.001), and partial pressure of oxygen (93.08 ± 26.20 mmHg, p = 0.003) levels and decreased β2-microglobulin (2.61 ± 1.21 mg/L, p = 0.039) levels compared to the control-NSIP group (n = 92). The ADM with organizing pneumonia (OP) group (n = 23) had a greater percentage of females (7/16, p = 0.023) and higher alanine aminotransferase (30.30 ± 20.67 U/L, p = 0.039) and aspartate aminotransferase (53.35 ± 65.86 U/L, p = 0.003) levels than the control-OP group (n = 29). Both the ADM-NSIP and OP groups presented elevated lactate dehydrogenase (LDH) levels (290.61 ± 86.49 U/L, p = 0.009; 317.35 ± 181.32 U/L, p = 0.003, respectively) and increased anti-nuclear antibody (ANA) positivity rates (82.61%, p = 0.01; 73.91%, p < 0.001, respectively). Notably, 81.26% of patients with ADM-NSIP/OP had LDH levels above normal. The serum LDH levels could be used to distinguish patients with ADM-NSIP/OP (sensitivity: 73.91%, specificity: 82.64%). Survival was shorter among patients with ADM-OP than among control patients (p = 0.002). Cox multivariate analysis revealed that age (p = 0.002), smoking status (p = 0.011), anti-melanoma differentiation-associated gene 5 (MDA5) antibody (p = 0.017), and white blood cell count (p = 0.004) were independent predictors of shorter survival.

conclusionsElevated serum LDH levels in patients predominantly presenting with NSIP or OP patterns may indicate the presence of ADM-ILD. The identified prognostic factors underscore the importance of early detection and personalized management strategies for optimizing outcomes in patients with ADM-ILD.

Indexed as

DermatomyositisLung Diseases, InterstitialAdultAgedFemaleHumansMaleMiddle AgedPrognosisRetrospective StudiesTomography, X-Ray ComputedAmyopathic dermatomyositisInterstitial lung diseaseLactate dehydrogenaseNonspecific interstitial pneumoniaOrganizing pneumonia

Identifiers

PMID39915831
PMCPMC11804100

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