Incidence and impact of interstitial lung disease and malignancy in patients with polymyositis, dermatomyositis, and clinically amyopathic dermatomyositis: a retrospective cohort study.

Satoshi Ikeda,Akihiro Ito,Tadashi Ishida,Akihiro Nishiyama,Takuya Takaiwa,Maki Noyama,Machiko Arita,Fumiaki Tokioka,Shohei Mishima,Kenjiro Furuta,Kenta Misaki,Toshihide Yokoyama,Hiroshige Yoshioka

doi:10.1186/s40064-015-1013-8

Satoshi Ikeda, Akihiro Ito + Show 11 more

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https://doi.org/10.1186/s40064-015-1013-8

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Journal: SpringerPlus	Publication Date: May 28, 2015
Citations: 68	License type: CC BY 4.0

Affiliation: Kurashiki Central Hospital

Abstract

The aims of this study were to retrospectively review Japanese consecutive cases of polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM), focusing on interstital lung disease (ILD) and malignancy, and to document any differences in the incidence, clinical features, and impact on prognosis among patients with PM, DM, and CADM.We retrospectively reviewed 62 consecutive patients diagnosed with PM, DM, and CADM according to Bohan and Peter’s criteria (PM/DM) and Sontheimer’s criteria and Gerami’s criteria (CADM), focusing on ILD and malignancy.ILD occurrence rates were 48 % (11/23) in patients with PM, 46 % (11/24) in DM, and 100 % (15/15) in CADM. Malignancy occurred during diagnosis or the observation period in 14 patients; 86 % were without ILD, and 64 % were DM without ILD. Multivariate logistic regression analysis showed that the risk of newly diagnosed malignancy was significantly lower in patients with ILD [odds ratio, 0.0688; 95 % confidence interval (CI), 0.00127–0.372; p = 0.00190] and significantly higher in patients with DM (odds ratio, 5.21; 95 % CI, 1.17–23.1; p = 0.0299) than in other patients. Patients with malignancies had shorter survival than those without malignancies; no clinically meaningful difference in survival was observed among the different myositis types and for presence of ILD. In CADM-ILD, 80 % fatal cases died from refractory ILD ≤90 days from the first visit; neither death nor recurrence occurred subsequently.In conclusion, a positive association between DM and malignancy and a negative association between ILD and malignancy were noted. In the present study, malignancy was a predictor of poor long-term prognosis, but ILD were not. ILD associated with CADM contributed greatly to poor short-term prognosis, but neither death nor recurrence occurred subsequently.

Highlights

Polymyositis (PM) and dermatomyositis (DM) are classified as idiopathic inflammatory myopathies characterized by proximal skeletal muscle weakness and muscle inflammation
The median time from the onset of symptoms to first visit or therapy initiation tended to be short in the patients with interstital lung disease (ILD), especially those in the CADMILD group
The Department of Respiratory Medicine was usually the first department in which the diagnosis of ILD was made in the PM with ILD (PM-ILD) and clinically amyopathic dermatomyositis (CADM)-ILD groups

Summary

Introduction

Polymyositis (PM) and dermatomyositis (DM) are classified as idiopathic inflammatory myopathies characterized by proximal skeletal muscle weakness and muscle inflammation. Unlike PM, DM is associated with a variety of characteristic rashes, such as the heliotrope. Interstitial lung disease (ILD) is an important complication in patients with PM, DM, and CADM. The occurrence of ILD has varied widely from 20 to 80 % among case series of patients with PM and DM (Marie et al 2002; Love et al 1991; Fathi et al 2008; Connors et al 2010). The clinicopathological features and prognosis of ILD vary depending on the type of myositis and. Ikeda et al SpringerPlus (2015) 4:240 myositis-specific antibodies (Fujisawa et al 2005). ILD associated with CADM is often refractory and rapidly progressive (Douglas et al 2001). The ratio of rapidly progressive ILD to the total population of CADM-ILD has been reported to be higher in eastern Asia than in Europe and the US. The presence of antiaminoacyl tRNA synthetase (anti-ARS) antibodies, such as anti Jo-1 antibody, is associated with chronic ILD, anti-MDA5 antibodies are associated with acute/subacute ILD (Sato et al 2012, 2013)

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