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Anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis exhibit three clinical phenotypes with different prognoses


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

 

  1. Department of Rheumatology and Immunology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  2. Department of Urology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  3. Department of Rheumatology and Immunology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  4. Department of Rheumatology and Immunology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  5. Department of Rheumatology and Immunology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  6. Department of Rheumatology and Immunology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  7. Department of Rheumatology and Immunology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
  8. Department of Rheumatology and Immunology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China. shengyun@medmail.com.cn
  9. Department of Rheumatology and Immunology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China. tfli@zzu.edu.cn

CER15110
2022 Vol.40, N°2
PI 0304, PF 0308
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PMID: 35084311 [PubMed]

Received: 27/08/2021
Accepted : 22/11/2021
In Press: 14/01/2022
Published: 25/02/2022

Abstract

OBJECTIVES:
We aimed to identify different subtypes of dermatomyositis (DM) patients positive with anti-melanoma differentiation-associated gene 5 antibody (DM-MDA5+) for customised treatments to improve the outcomes.
METHODS:
Among 96 DM-MDA5+ patients, subgroups with similar phenotypes were delineated using hierarchical clustering analysis of the clinico-biological characteristics. Classification and regression trees were used to build a classification model and survival analysis was used to evaluate the prognoses of subgroups.
RESULTS:
Three subgroups were identified among 96 DM-MDA5+ patients, and patients in different subgroups had highly heterogenic manifestations and outcomes. Cluster 1 patients were referred to as mild group of rheumatologic patterns with good prognosis. Cluster 2 patients were referred to as young typical DM group with good prognosis. Cluster 3 patients were referred to as elderly rapidly progressive interstitial lung disease (RPILD) group with poor prognosis. A predictive model to classify patients was established, and three critical factors were found, including age, serum ferritin and myalgia.
CONCLUSIONS:
DM-MDA5+ patients have a poor short-term prognosis. Three clinical phenotypes with different prognoses were identified in DM-MDA5+ patients.

DOI: https://doi.org/10.55563/clinexprheumatol/df2oc3

Rheumatology Article