Neurology: Dermatomyositis: The underlying pathobiological mechanisms of the potential subtypes of different antibody classifications are different

The discovery of dermatomyositis-specific antibodies (DMSA) transformed the clinically oriented dermatomyositis (DM) classification standard (mainly based on the presence of skin lesions and muscle weakness described by Bohan and Peter in 1975) into the European Neuromuscular Center (ENMC) The clinical serological pathology standard proposed in 2018 (ENMC-DM in 2018)
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The discovery of dermatomyositis-specific antibodies (DMSA) transformed the clinically oriented dermatomyositis (DM) classification standard (mainly based on the presence of skin lesions and muscle weakness described by Bohan and Peter in 1975) into the European Neuromuscular Center (ENMC) The clinical serological pathology standard proposed in 2018 (ENMC-DM in 2018)
.

In the 2018 ENMC-DM consensus , the following 5 DMSAs were included as serological standards: anti-transcription intermediary factor 1-γ (TIF1-γ), anti-complex nucleosome restructuring histone deacetylase (Mi-2) , Anti-melanoma differentiation gene 5 (MDA5), anti-nuclear matrix protein 2 (NXP-2) and anti-small ubiquitin-like modification activating enzyme (SAE)

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In the 2018 ENMC-DM consensus , the following 5 DMSAs were included as serological standards: anti-transcription intermediary factor 1-γ (TIF1-γ), anti-complex nucleosome restructuring histone deacetylase (Mi-2) , Anti-melanoma differentiation gene 5 (MDA5), anti-nuclear matrix protein 2 (NXP-2) and anti-small ubiquitin-like modification activating enzyme (SAE)

Interestingly, DMSA-related clinical phenotypes have been characterized, including: anti-TIF1-γ DM with diabetic skin lesions, dysphagia, and malignant tumors; anti-Mi-2 DM with high creatine kinase (CK), myalgia, muscle Weakness ; anti-MDA5 DM with manipulators and interstitial lung disease (ILD), but low CK levels and less muscle involvement; anti-NXP-2 DM with muscle weakness but less skin involvement

However, most of these phenotypes were limited to small-scale studies and were not included in the 2018 ENMC-DM

The researchers conducted a retrospective pathological review of the muscle biopsy of patients with dermatomyositis diagnosed in the tertiary muscle disease laboratory from January 2009 to December 2020

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The researchers conducted a retrospective pathological review of the muscle biopsy of patients with dermatomyositis diagnosed in the tertiary muscle disease laboratory from January 2009 to December 2020

• A total of 256 patients were enrolled
  
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  The study described the comprehensive serology-pathology correlation of diabetes, mainly using MXA expression as the inclusion criteria
  
  Literature source: Tanboon J, Inoue M, Saito Y, et al.
  Dermatomyositis: Muscle Pathology According to Antibody Subtypes [published online ahead of print, 2021 Dec 6].
  Neurology.
  2021;10.
  1212/WNL.
  0000000000013176.
  doi:10.
  1212/WNL .
  0000000000013176 Tanboon J, Inoue M, Saito Y, et al.
  Dermatomyositis: Muscle Pathology According to Antibody Subtypes [published online ahead of print, 2021 Dec 6].
  Neurology.
  2021;10.
  1212/WNL.
  0000000000013176.
  doi:10.
  1212/WNL.
  0000000000013176 Leave a message here