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Clinical features of patients with anti-melanoma differentiation-associated gene-5 antibody-positive dermatomyositis complicated by spontaneous pneumomediastinum

  • Koichi YamaguchiEmail author
  • Aya Yamaguchi
  • Miki Itai
  • Chiharu Kashiwagi
  • Kazutaka Takehara
  • Shuhei Aoki
  • Yuri Sawada
  • Kohei Taguchi
  • Kazue Umetsu
  • Kazuma Oshima
  • Megumi Uchida
  • Masao Takemura
  • Kenichiro Hara
  • Sei-ichiro Motegi
  • Yoshinao Muro
  • Masao Nakasatomi
  • Toru Sakairi
  • Keiju Hiromura
  • Masahiko Kurabayashi
  • Toshitaka Maeno
Original Article
  • 2 Downloads

Abstract

Background

Dermatomyositis (DM) with autoantibody against melanoma differentiation-associated gene-5 (MDA5) is characterized by elevated risk of rapidly progressive interstitial lung disease (RP-ILD) with a potentially fatal course. Pneumomediastinum (PNM) is a common pulmonary manifestation which accompanies ILD. However, the clinical features of the patients with anti-MDA5 antibody-positive DM who develop PNM remain unclear.

Methods

We retrospectively examined 31 patients with DM having anti-MDA5 antibody and compared the clinical features between patients with PNM (PMN(+)) (n = 11) and those without (PNM(−) (n = 20). In addition, we evaluated the treatment-related prognoses in PNM(+) group.

Results

CT score (total ground-glass opacity (GGO) score, P = 0.02; total fibrosis score, P = 0.02) before treatment, and mortality (P = 0.04) were significantly higher in PNM(+) group. The cumulative survival rate as assessed by Kaplan–Meier method was significantly lower for the PNM(+) group (P = 0.02). Among 11 PMN(+) patients, 9 patients (9/11, 81.8%) underwent intensive immunosuppression therapy for RP-ILD, and 5 patients (5/11, 45.5%) did not respond to it and died from the respiratory failure. At the time of diagnosis of PNM, nonsurvivors had worse liver function (ALT, P = 0.03; LDH, P = 0.01), worse respiratory status (A-aDO2, P = 0.01), and worse CT score (total GGO score, P < 0.01).

Conclusions

A subgroup of patients with DM having anti-MDA5 antibody complicated by PNM as well as RP-ILD did respond to intensive immunosuppression therapy. Initial aggressive immunosuppressive therapy should be considered for these patients.

Key Points

This study clearly demonstrate the presence of PNM was associated with elevated risk of death due to respiratory failure from RP-ILD among patients with DM having circulating anti-MDA5-antibody.

This study demonstrate evaluation of CT image may be helpful to find patients with better response to the intense immunosuppression therapy for the patients with DM having circulating anti-MDA5-antibody and PNM.

Keywords

Anti-melanoma differentiation-associated gene 5 antibodies Dermatomyositis Pneumomediastinum Rapidly progressive interstitial lung disease 

Notes

Compliance with ethical standards

This study was approved by institutional review board of Gunma University. This study was conducted by the principles of the Declaration ofHelsinki.

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  • Koichi Yamaguchi
    • 1
    Email author
  • Aya Yamaguchi
    • 1
  • Miki Itai
    • 1
  • Chiharu Kashiwagi
    • 1
  • Kazutaka Takehara
    • 1
  • Shuhei Aoki
    • 1
  • Yuri Sawada
    • 1
  • Kohei Taguchi
    • 1
  • Kazue Umetsu
    • 1
  • Kazuma Oshima
    • 1
  • Megumi Uchida
    • 1
  • Masao Takemura
    • 1
  • Kenichiro Hara
    • 1
  • Sei-ichiro Motegi
    • 2
  • Yoshinao Muro
    • 3
  • Masao Nakasatomi
    • 4
  • Toru Sakairi
    • 4
  • Keiju Hiromura
    • 4
  • Masahiko Kurabayashi
    • 5
  • Toshitaka Maeno
    • 1
  1. 1.Department of Allergy and Respiratory MedicineGunma University Graduate School of MedicineMaebashiJapan
  2. 2.Department of DermatologyGunma University Graduate School of MedicineMaebashiJapan
  3. 3.Department of DermatologyNagoya University Graduate School of MedicineNagoyaJapan
  4. 4.Department of Nephrology and RheumatologyGunma University Graduate School of MedicineMaebashiJapan
  5. 5.Department of Cardiovascular MedicineGunma University Graduate School of MedicineMaebashiJapan

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