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Predictors of mortality for dermatomyositis patients positive with anti-melanoma differentiation-related gene 5 and optimal treatment


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11

 

  1. Department of Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  2. Department of Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  3. Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital, Shanghai, China.
  4. Department of Gynaecology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  5. Department of Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  6. Department of Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  7. Department of Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  8. Department of Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  9. Department of Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  10. Department of Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China. shengyun19690505@163.com
  11. Department of Rheumatology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China. llj1503609@126.com

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PMID: 37199165 [PubMed]

Received: 29/10/2022
Accepted : 03/03/2023
In Press: 15/05/2023

Abstract

OBJECTIVES:
To explore the risk factors of early death in dermatomyositis patients positive with anti-melanoma differentiation-related gene 5 antibody (anti-MDA5-DM). To explore the optimal treatment regimen for patients with anti-MDA5-DM.
METHODS:
Patients with newly onset anti-MDA5-DM from June 2018 to October 2021 in our centre were retrospectively reviewed for 6 months. Patients were divided into five groups based on initial treatments. The major outcome was mortality in 6 months. Secondary outcomes included remission and severe infection.
RESULTS:
A total of 214 patients were included in the study. During 6 month follow-up, 63 patients (30.14%) died, 112 patients (53.59%) achieved remission, 52 patients (24.88%) experienced serious infection and 5 patients (2.34%) were lost. Independent risk factors of mortality in the first 6 months after diagnosis were as follows: age> 53 years, skin ulcer, peripheral blood lymphocyte count (LYMP)≤ 0.6×109/L, lactate dehydrogenase (LDH) > 500 U/L, C reactive protein (CRP) > 5mg/L, anti-Ro52 antibody and ground-glass opacity (GGO) score> 2. On the contrary, prophylactic use of the compound sulfamethoxazole (SMZ Co) was independent protective factor. The five-category treatment was not an independent influencing factor of early death, but subgroup analysis found that patients with rapidly progressive interstitial lung disease (RPILD) responded better to a triple combination of high-dose glucocorticoids (GC), calcineurin inhibitors (CNI) and cyclophosphamide (CYC) or a triple combibation of GC, CNI and tofacitinib (TOF).
CONCLUSIONS:
Advanced age, skin ulcer, lymphopenia, anti-Ro52 antibody and higher levels of LDH, CRP and GGO score increase the risk of early death for MDA5-DM, while prophylactic use of SMZ Co is protective. Aggressive therapy with combined immunosuppressants may improve the short-term prognosis of anti-MDA5-DM with RPILD.

DOI: https://doi.org/10.55563/clinexprheumatol/1vz1p2

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