Lupus & antiphospholipid syndrome

Anti-fibroblast and anti-endothelial cell autoantibodies in pulmonary arterial hypertension (PAH) in patients with connective tissue diseases (CTD)

Rheumatology (Oxford)Published Cross-sectional study

Authors' conclusion

Anti-ANXA5 IgG autoantibody reactivity might represent a predictive biomarker for PAH associated with MCTD and SLE.

Study context

Population
Patients with CTD excluding SSc (SLE, MCTD, primary SS), French multicentre auto-HTAP study
Participants
86 patients
Exposure
CTD patients with PAH vs without PAH
  • PAH+n = 32
  • PAH-n = 54reference
Outcome
Autoantibodies (AFA, AECA) discriminating for PAH associated with SLE, MCTD and primary SS, and their target antigens
Methodological detailsmulticentre, proteomics, 1D and 2D immunoblots, ELISA confirmation
  • Multicentre
  • Proteomics
  • 1D and 2D immunoblots
  • ELISA confirmation

Main finding

As reported in the abstract

Anti-ANXA5 IgG reactivity

PAH+ IgG recognised fibroblast protein spots not recognised by PAH- IgG, including Annexin A5 (ANXA5)

39

PAH+ IgG recognised endothelial cell protein spots not recognised by PAH- IgG

34

Observational data: associations, not effects.

Further findings

IgG anti-fibroblast (AFA) and AECA reactivity in 1D immunoblots, PAH+

PAH+ with IgG AFA reactivity
56.2%
PAH+ with IgG AECA reactivity
40.6%

Protein spots recognised by serum IgG pools in 2D immunoblots (fibroblasts / endothelial cells)

SLE (n = 14), fibroblasts
273 ± 79
MCTD (n = 10), fibroblasts
205 ± 77
SS (n = 9), fibroblasts
109 ± 11
Healthy controls, fibroblasts
109
SLE, endothelial cells
189 ± 48
MCTD, endothelial cells
146 ± 30
SS, endothelial cells
88 ± 33
Healthy controls, endothelial cells
190

Original publication

Anti-fibroblast and anti-endothelial cell autoantibodies in pulmonary arterial hypertension (PAH) in patients with connective tissue diseases (CTD). Rheumatology (Oxford). Published 7 February 2025. doi:10.1093/rheumatology/keaf075

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