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Predictive factors for long-term outcome in polymyositis/dermatomyositis-associated interstitial lung diseases.
Respir Investig 2017; 55(2):130-137RI

Abstract

BACKGROUND

Interstitial lung disease (ILD) is strongly associated with polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM). It is also related to mortality. Previous studies have highlighted that the acute form of PM/DM/CADM-associated ILD (PM/DM/CADM-ILD) has a poor short-term prognosis. However, little is known about the long-term clinical features of patients with PM/DM/CADM-ILD. The aim of the present study is to clarify the clinical characteristics and the predictive factors for long-term outcomes in patients with PM/DM/CADM-ILD.

METHODS

Thirty-four patients with PM/DM/CADM-ILD who were followed up for more than 12 months were analyzed retrospectively. The patients were classified as "stable" or "deterioration" according to respiratory symptoms, serial changes in forced vital capacity (FVC) or arterial oxygen pressure, and radiologic findings during the follow-up period.

RESULTS

Twenty-six patients (76%) were in the stable group and eight patients (24%) were in the deterioration group. Home oxygen therapy was performed in six cases in the deterioration group because of chronic respiratory failure due to progression of ILD. The deterioration group, in comparison to the stable group, had a significantly lower %FVC and a higher positive rate for the anti-PL-7 antibody. Multivariate logistic regression analysis revealed that a positive anti-PL-7 antibody test and a lower %FVC were independently associated with deterioration during long-term follow-up.

CONCLUSIONS

Patients with PM/DM/CADM-ILD are at risk for chronic respiratory failure due to the deterioration of ILD during long-term follow-up. The presence of anti-PL-7 antibody and a lower %FVC at initial diagnosis may predict long-term deterioration in patients with PM/DM/CADM-ILD.

Authors+Show Affiliations

Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu 431-3192, Japan. Electronic address: fujisawa@hama-med.ac.jp.Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu 431-3192, Japan. Electronic address: hozumi@hama-med.ac.jp.Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu 431-3192, Japan. Electronic address: masato.k@hama-med.ac.jp.Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu 431-3192, Japan. Electronic address: norieno@hama-med.ac.jp.Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu 431-3192, Japan. Electronic address: nakayuta@hama-med.ac.jp.Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu 431-3192, Japan. Electronic address: inui@hama-med.ac.jp.Department of Rheumatology and Clinical Immunology, Kyoto University Hospital, 54 Shogoin-kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan. Electronic address: ranran@kuhp.kyoto-u.ac.jp.Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, 54 Shogoin-kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan. Electronic address: imuray@kuhp.kyoto-u.ac.jp.Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, 54 Shogoin-kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan. Electronic address: mimorit@kuhp.kyoto-u.ac.jp.Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu 431-3192, Japan. Electronic address: suda@hama-med.ac.jp.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

28274528

Citation

Fujisawa, Tomoyuki, et al. "Predictive Factors for Long-term Outcome in Polymyositis/dermatomyositis-associated Interstitial Lung Diseases." Respiratory Investigation, vol. 55, no. 2, 2017, pp. 130-137.
Fujisawa T, Hozumi H, Kono M, et al. Predictive factors for long-term outcome in polymyositis/dermatomyositis-associated interstitial lung diseases. Respir Investig. 2017;55(2):130-137.
Fujisawa, T., Hozumi, H., Kono, M., Enomoto, N., Nakamura, Y., Inui, N., ... Suda, T. (2017). Predictive factors for long-term outcome in polymyositis/dermatomyositis-associated interstitial lung diseases. Respiratory Investigation, 55(2), pp. 130-137. doi:10.1016/j.resinv.2016.09.006.
Fujisawa T, et al. Predictive Factors for Long-term Outcome in Polymyositis/dermatomyositis-associated Interstitial Lung Diseases. Respir Investig. 2017;55(2):130-137. PubMed PMID: 28274528.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Predictive factors for long-term outcome in polymyositis/dermatomyositis-associated interstitial lung diseases. AU - Fujisawa,Tomoyuki, AU - Hozumi,Hironao, AU - Kono,Masato, AU - Enomoto,Noriyuki, AU - Nakamura,Yutaro, AU - Inui,Naoki, AU - Nakashima,Ran, AU - Imura,Yoshitaka, AU - Mimori,Tsuneyo, AU - Suda,Takafumi, Y1 - 2017/02/08/ PY - 2016/05/06/received PY - 2016/09/18/revised PY - 2016/09/26/accepted PY - 2017/3/10/entrez PY - 2017/3/10/pubmed PY - 2017/9/20/medline KW - Anti-PL-7 antibody KW - Dermatomyositis KW - Interstitial lung disease KW - Outcome KW - Polymyositis SP - 130 EP - 137 JF - Respiratory investigation JO - Respir Investig VL - 55 IS - 2 N2 - BACKGROUND: Interstitial lung disease (ILD) is strongly associated with polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM). It is also related to mortality. Previous studies have highlighted that the acute form of PM/DM/CADM-associated ILD (PM/DM/CADM-ILD) has a poor short-term prognosis. However, little is known about the long-term clinical features of patients with PM/DM/CADM-ILD. The aim of the present study is to clarify the clinical characteristics and the predictive factors for long-term outcomes in patients with PM/DM/CADM-ILD. METHODS: Thirty-four patients with PM/DM/CADM-ILD who were followed up for more than 12 months were analyzed retrospectively. The patients were classified as "stable" or "deterioration" according to respiratory symptoms, serial changes in forced vital capacity (FVC) or arterial oxygen pressure, and radiologic findings during the follow-up period. RESULTS: Twenty-six patients (76%) were in the stable group and eight patients (24%) were in the deterioration group. Home oxygen therapy was performed in six cases in the deterioration group because of chronic respiratory failure due to progression of ILD. The deterioration group, in comparison to the stable group, had a significantly lower %FVC and a higher positive rate for the anti-PL-7 antibody. Multivariate logistic regression analysis revealed that a positive anti-PL-7 antibody test and a lower %FVC were independently associated with deterioration during long-term follow-up. CONCLUSIONS: Patients with PM/DM/CADM-ILD are at risk for chronic respiratory failure due to the deterioration of ILD during long-term follow-up. The presence of anti-PL-7 antibody and a lower %FVC at initial diagnosis may predict long-term deterioration in patients with PM/DM/CADM-ILD. SN - 2212-5353 UR - https://www.unboundmedicine.com/medline/citation/28274528/Predictive_factors_for_long_term_outcome_in_polymyositis/dermatomyositis_associated_interstitial_lung_diseases_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S2212-5345(16)30139-3 DB - PRIME DP - Unbound Medicine ER -