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Introduction <p>The 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.</p> Methods <p>A 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.</p> Results <p>There were significant differences in the clinical parameters among the 190 patients with ILD in the amyopathic dermatomyositis (ADM, <i>n</i> = 69) and control (<i>n</i> = 121) groups. The ADM with nonspecific interstitial pneumonia (NSIP) group (<i>n</i> = 46) presented increased haemoglobin (125.93 ± 12.91&#xa0;g/L, <i>p</i> = 0.005), creatine kinase-MB (15.19 ± 8.58 U/L, <i>p</i> &lt; 0.001), and partial pressure of oxygen (93.08 ± 26.20&#xa0;mmHg, <i>p</i> = 0.003) levels and decreased β2-microglobulin (2.61 ± 1.21&#xa0;mg/L,<i> p</i> = 0.039) levels compared to the control-NSIP group (<i>n</i> = 92). The ADM with organizing pneumonia (OP) group (<i>n</i> = 23) had a greater percentage of females (7/16, <i>p</i> = 0.023) and higher alanine aminotransferase (30.30 ± 20.67 U/L, <i>p</i> = 0.039) and aspartate aminotransferase (53.35 ± 65.86 U/L, <i>p</i> = 0.003) levels than the control-OP group (<i>n</i> = 29). Both the ADM-NSIP and OP groups presented elevated lactate dehydrogenase (LDH) levels (290.61 ± 86.49 U/L,<i> p</i> = 0.009; 317.35 ± 181.32 U/L, <i>p</i> = 0.003, respectively) and increased anti-nuclear antibody (ANA) positivity rates (82.61%, p = 0.01; 73.91%, <i>p</i> &lt; 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 (<i>p</i> = 0.002). Cox multivariate analysis revealed that age (<i>p</i> = 0.002), smoking status (<i>p</i> = 0.011), anti-melanoma differentiation-associated gene 5 (MDA5) antibody (<i>p</i> = 0.017), and white blood cell count (<i>p</i> = 0.004) were independent predictors of shorter survival.</p> Conclusions <p>Elevated 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.</p>

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

  • 期刊论文

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  • 发表日期：

    2025年2月6日

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

摘要

Introduction

The 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.

Methods

A 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.

Results

There 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.

Conclusions

Elevated 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.