Anti-MDA5 antibody positive dermatomyositis, early typing may reduce mortality!

*For medical professional reading reference only

The world's largest cohort of anti-MDA5 antibody-positive dermatomyositis patients On July 18, 2022, Professor Tan Wenfeng's research group from the Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University (Jiangsu Provincial People's Hospital) published a topic on Arthritis & Rheumatology online.

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Background: Anti-MDA5 antibody-positive dermatomyositis (anti-MDA5 ⁺ ) is prone to be associated with rapidly progressive pulmonary interstitial disease (RP-ILD)

Early warning is important

Q

Medical field: DM is a systemic inflammatory disease with muscle and skin damage as the main manifestation

Professor Tan Wenfeng: Anti-MDA5 ⁺ DM will show pulmonary phagocytosis, which can easily lead to severe lung injury.

Therefore, if early identification can be achieved, early attention and treatment can be given, thereby reducing the incidence of RP-ILD and improving the prognosis

The largest international cohort

Q

Medical community: Anti-MDA5 ⁺ DM has different clinical features in different ethnic groups, and this study established the largest anti-MDA5 ⁺ DM cohort reported in the literature, the first to describe the anti-MDA5 ⁺ DM classification of Asian patients in a large cohort, how do you Looking at this "biggest" and "first time", can you share your feelings?

Professor Tan Wenfeng: Compared with other rheumatism and even internal diseases, anti-MDA5 ⁺ DM is a rare disease, so cases are scattered in various centers, and it has many phenotypes, so a large sample of data is needed to Help us clarify its entire characteristics, development process, and prognosis of adverse events

Q

Medical community: The study was completed with the joint efforts of several rheumatology centers in Jiangsu Province.

Professor Tan Wenfeng: I think one is coordination
.

Reconciling so many centers is a difficult and very worrying issue
.
The second is to ensure the quality of research
.

Specimen collection, data collection, various indicators, especially subjective indicators, are interpreted differently by each center, so ensuring consistency is a very challenging issue
.
Third, data integrity
.

There may be a lot of missing data because of the different patient profiles in each center
.

Early identification of RP-ILD with a view to improving patient outcomes

Q

Medical community: This study identified 3 different clinical patterns and outcomes by stratifying the risk of RP-ILD in patients with different clinical subtypes of anti-MDA5 ⁺ DM.
Could you please briefly describe the situation of these 3 subgroups , and what clinical implications does this have?

Professor Tan Wenfeng: Through machine learning and unsupervised cluster analysis, anti-MDA5 ⁺ dermatomyositis patients were divided into three clinical subtypes of low, intermediate and high RP-ILD risk groups, revealing the clinical characteristics of different subtypes
.
The mortality risk of RP-ILD was different among the three subtypes, with type 1 being the lowest (low risk group), type 2 intermediate (intermediate risk group), and type 3 (high risk group) being the highest
.

• From the perspective of clinical features, type 1 has typical rash and muscle weakness, that is, classic DM
  .
  
  The second is that the inflammatory indicators are not high, and the levels of ESR, CRP, LDH, ALT and AST are the lowest
  .
  
  Therefore, in clinical practice, if a patient is found to have rash and muscle weakness as the main manifestations, and the inflammatory index is not high, we can classify it as one type
  .
  
  

• As for type II, it has rashes and muscle weakness, but it is slightly lighter than type I.
  It is not its main manifestation.
  It is more of arthritis as an important feature, and the inflammatory index is moderately increased
  .
  
  

• Type 3 is much like classic amyopathic DM, with minimal to no rash and no muscle weakness, but with high levels of inflammation
  .
  
  At the same time, anti-Ro52 antibody positivity and high titer anti-MDA5 antibody positivity often coexist in this type, and the co-positivity of both antibodies is highly correlated with the severity and prognosis of RP-ILD
  .
  

We made a decision tree and generated eight parameters to help distinguish the three subtypes, namely age > 50 years, disease duration < 3 months, muscle weakness, arthritis, CRP level, CK level, anti-Ro52 Antibody titers and anti-MDA5 antibody titers were used to further clarify the different typing characteristics
.
I think the significance of this research is:
early assessment and early warning
.

When we see that the patient is mainly manifested by rash, we can be a little more relieved
.

But if the rash and muscle weakness are mild, but inflammation levels are high, there is a high risk of developing RP-ILD
.
Among the eight parameters mentioned above, the duration of the disease is very important
.

From the data analysis point of view, there is a time window for the occurrence and development of RP-ILD.
The first three months were the hardest hit area, followed by the first six months
.

That is to say, 90% of RP-ILD deaths occurred in the first six months, especially the first three months.
This finding is very important
.

references:

[1]Xu L, You H, Wang L, Lv C, Yuan F, Li J, Wu M, Da Z, Wei H, Yan W, Zhou L, Yin S, Zhou D, Wu J, Lu Y, Su D , Liu Z, Liu L, Ma L, Xu X, Zang Y, Liu H, Ren T, Wang F, Du Y, Xue J, Zhang M, Tan W.
Identification of three different phenotypes in anti-MDA5 antibody-positive dermatomyositis patients: implications for rapidly progressive interstitial lung disease prediction.
Arthritis Rheumatol.
2022 Jul 18.
doi: 10.
1002/art.
42308.
Epub ahead of print.
PMID: 35849805.

Expert Profile

Professor Tan Wenfeng

• Chief Physician, Doctoral Supervisor

• MD, Postdoctoral Fellow, University of California, Los Angeles

• Deputy Director of the Department of Rheumatology and Immunology, The First Affiliated Hospital of Nanjing Medical University (in charge of the work)

• Chairman-designate of Jiangsu Rheumatology Association

• Chairman of Jiangsu Association of Integrated Chinese and Western Rheumatology

• The second-level training object of 333 high-level talents in Jiangsu Province

• Presided over 5 projects of the National Natural Science Foundation of China

• Published more than 30 SCI papers in journals including "Arthritis Rheum"

Source of this article: Rheumatism Immunity Channel of the Medical Community
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