Systemic sclerosis & Raynaud's

Autoantibodies to nuclear valosin-containing protein-like protein: systemic sclerosis-specific antibodies revealed by in vitro human proteome

Rheumatology (Oxford)Published Mechanistic study

Authors' conclusion

Anti-NVL Ab is an SSc-specific autoantibody associated with a unique combination of clinical features, including limited skin sclerosis and lack of lung involvement.

Study context

Population
Systemic sclerosis (SSc) patients with nucleolar patterned ANA of unknown specific antibodies (Abs); controls: SSc with known SSc-specific autoantibodies, other connective tissue diseases, healthy subjects
Comparison
Anti-NVL Ab-positive SSc vs SSc with NUC-ANAs other than anti-NVL Abs
Outcome
Identification of undescribed SSc-specific autoantibodies targeting nucleolar antigens and their clinical significance
Methodological detailsproteome-wide autoantibody screening (PWAS), wet protein arrays from in vitro human proteome, retrospective review of electronic medical records
  • Proteome-wide autoantibody screening (PWAS)
  • Wet protein arrays from in vitro human proteome
  • Retrospective review of electronic medical records

Main finding

As reported in the abstract

  • PWAS identified three candidate autoantibodies, including anti-NVL Ab
  • Only anti-NVL Abs exclusively detected in SSc vs disease controls
  • Anti-NVL Ab detection reproduced by immunoblotting and immunoprecipitation
  • Indirect immunofluorescence: homogeneous nucleolar patterns

Further findings

Clinical features: diffuse skin sclerosis and ILD

Significantly low prevalence of diffuse skin sclerosis and ILD in anti-NVL Ab-positive cases vs SSc with other NUC-ANAs

Original publication

Autoantibodies to nuclear valosin-containing protein-like protein: systemic sclerosis-specific antibodies revealed by in vitro human proteome. Rheumatology (Oxford). Published 30 January 2024. doi:10.1093/rheumatology/keae063

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