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Idiopathic inflammatory myopathies: CT characteristics of interstitial lung disease and their association(s) with myositis-specific autoantibodies

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Abstract

Objectives

Interstitial lung disease (ILD), one of the most common extramuscular manifestations of idiopathic inflammatory myopathies (IIMs), carries a poor prognosis. Myositis-specific autoantibody (MSA)-positivity is a key finding for IIM diagnosis. We aimed to identify IIM-associated lung patterns, evaluate potential CT–ILD finding–MSA relationships, and assess intra- and interobserver reproducibility in a large IIM population.

Methods

All consecutive IIM patients (2003–2019) were included. Two chest radiologists retrospectively assessed all chest CT scans. Multiple correspondence and hierarchical cluster analyses of CT findings identified and characterized ILD-patient subgroups. Classification and regression-tree analyses highlighted CT-scan variables predicting three patterns. Three independent radiologists read CT scans twice to assign patients according to CT–ILD-pattern clusters.

Results

Among 257 IIM patients, 94 (36.6%) had ILDs; 87 (93%) of them were MSA-positive. ILD–IIM distribution was 54 (57%) ASyS, 21 (22%) DM, 15 (16%) IMNM, and 4 (4%) IBM. Cluster analysis identified three ILD-patient subgroups. Consolidation characterized cluster 1, with significantly (p < 0.05) more frequent anti-MDA5–autoantibody-positivity. Significantly more cluster-2 patients had a reticular pattern, without cysts and with few consolidations. All cluster-3 patients had cysts and anti-PL12 autoantibodies. Clusters 2 and 3 included significantly more ASyS patients. Intraobserver concordances to classify patients into those three clusters were good-to-excellent (Cohen κ 0.64–0.81), with good interobserver reliability (Fleiss’s κ 0.56).

Conclusion

Despite the observed IIM heterogeneity, CT-scan criteria enabled ILD assignment to the three clusters, which were associated with MSAs. Radiologist identification of those clusters could facilitate diagnostic screening and therapeutics.

Summary statement

Interstitial lung disease in patients with idiopathic inflammatory myopathy could be classified into three clusters according to CT-scan criteria, and these clusters were significantly associated with myositis-specific autoantibodies.

Key Points

• Cluster analysis discerned three homogeneous groups of interstitial lung disease (ILD) for which cysts, consolidations, and reticular pattern were discriminatory, and associated with myositis-specific autoantibodies.

• Like muscle- and extramuscular-specific phenotypes, myositis-specific autoantibodies are also associated with specific ILD patterns in patients with idiopathic inflammatory myopathies.

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Abbreviations

ASyS:

Anti-synthetase syndrome

CART:

Classification-and-regression tree

DM:

Dermatomyositis

IBM:

Inclusion body myositis

IIM:

Idiopathic inflammatory myopathy

ILD:

Interstitial lung disease

IMNM:

Immune-mediated necrotizing myopathy

MSA:

Myositis-specific antibody

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Funding

The authors state that this work has not received any funding.

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Authors and Affiliations

  1. Cardiovascular and Thoracic Imaging Unit, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University and Laboratoire d’Imagerie Biomédicale, INSERM, CNRS, Institute of Cardiometabolism and Nutrition, Sorbonne University, Paris, France

    Amandine Laporte, Nicoletta Pasi, Dan Toledano & Samia Boussouar

  2. MRSU 974, INSERM, Research Center in Myology, Sorbonne University, Paris, France

    Kubéraka Mariampillai, Yves Allenbach & Olivier Benveniste

  3. Department of Internal Medecine and Clinical Immunology, Referral Center for Rare Neuromuscular Diseases, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, Paris, France

    Yves Allenbach & Olivier Benveniste

  4. Department of Radiology, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, Paris, France

    Victoria Donciu

  5. Department of Public Health (INSERM UMR 1136) and Pharmaco-Epidemiology Center, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, Paris, France

    Benjamin Granger

  6. Referral Center for Neuro-Muscular Diseases, DHUi2B, Paris and Department of Clinical Research and Innovation, Foch Hospital, Suresnes, France

    Philippe A. Grenier

Authors
  1. Amandine Laporte
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  2. Kubéraka Mariampillai
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Corresponding author

Correspondence to Samia Boussouar.

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The scientific guarantor of this publication is Dr. Boussouar Samia.

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The authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article.

Statistics and biometry

Dr. Mariampillai Kubéraka kindly provided statistical advice for this manuscript.

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Written informed consent was obtained from all subjects (patients) in this study.

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Institutional Review Board approval was obtained.

Study subjects or cohorts overlap

Some study subjects or cohorts have been previously reported in “Development of a new classification system for idiopathic inflammatory myopathies based on clinical manifestations and myositis-specific autoantibodies.” JAMA Neurology 2018.

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• cross sectional study / diagnostic study / observational

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Laporte, A., Mariampillai, K., Allenbach, Y. et al. Idiopathic inflammatory myopathies: CT characteristics of interstitial lung disease and their association(s) with myositis-specific autoantibodies. Eur Radiol 32, 3480–3489 (2022). https://doi.org/10.1007/s00330-021-08411-w

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