Clinical characteristics of cancer-associated myositis complicated by interstitial lung disease: a large-scale multicentre cohort study.

Abstract

OBJECTIVE:

To clarify the incidence, risk factors, and impact of malignancy in patients with PM/DM-associated interstitial lung disease (ILD).

METHODS:

This study used data from 497 patients with PM/DM-associated ILD enrolled in a multicentre, retrospective and prospective cohort of incident cases. Cancer-associated myositis (CAM) was defined as malignancy diagnosed within 3 years before or after PM/DM diagnosis. Demographic and clinical information was recorded at the time of diagnosis, and data about the occurrence of mortality and malignancy was collected.

RESULTS:

CAM was identified in 32 patients with PM/DM-associated ILD (6.4%). Patients with CAM were older (64 vs 55 years, P < 0.001), presented with arthritis less frequently (24% vs 49%, P = 0.01), and showed a lower level of serum Krebs von den Lungen-6 (687 vs 820 IU/l, P = 0.03) than those without CAM. The distribution of myositis-specific autoantibodies, including anti-melanoma differentiation-associated gene 5, anti-aminoacyl tRNA synthetase, and anti-transcriptional intermediary factor 1-γ antibodies, did not differ between the groups. Survival analysis demonstrated that CAM patients had a poorer survival than non-CAM patients (P = 0.006), primarily due to excess deaths by concomitant malignancy, while mortality due to ILD-related respiratory failure was similar between the groups (P = 0.51).

CONCLUSION:

Concomitant malignancy can occur in patients with PM/DM-associated ILD, and has significant impact on mortality. Older age, lack of arthritis, and a lower level of serum Krebs von den Lungen-6 at diagnosis are predictors of concomitant malignancy.

• Authors: Yuko Kaneko, Takahiro Nunokawa, Yoshinori Taniguchi, Yukie Yamaguchi, Takahisa Gono, Kenichi Masui, Atsushi Kawakami, Yasushi Kawaguchi, Shinji Sato, Masataka Kuwana, JAMI investigators
• Journal: Rheumatology (Oxford, England) (Rheumatology (Oxford)) Vol. 59 Issue 1 Pg. 112-119 (01 01 2020) ISSN: 1462-0332 [Electronic] England
• PMID: 31382295 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
• Copyright: © The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].

Chemical References

• Autoantibodies
• MUC1 protein, human
• Mucin-1
• Amino Acyl-tRNA Synthetases

Topics

• Age Factors
• Amino Acyl-tRNA Synthetases (immunology)
• Autoantibodies (blood)
• Female
• Humans
• Incidence
• Japan (epidemiology)
• Lung Diseases, Interstitial (blood, etiology, mortality)
• Male
• Middle Aged
• Mucin-1 (blood)
• Myositis (blood, etiology, mortality)
• Neoplasms (blood, complications, mortality)
• Prospective Studies
• Retrospective Studies
• Risk Factors