Prevalence of Auto-Antibodies Associated to Pulmonary Arterial Hypertension in Scleroderma - A Review

Autoimmun Rev. 2018 Dec;17(12):1186-1201. doi: 10.1016/j.autrev.2018.06.009. Epub 2018 Oct 12.


The prevalence of auto-antibodies associated to pulmonary arterial hypertension in scleroderma patients was reviewed, based on reports cited in two major scientific databases. Data were collected on the following types of antibodies: antinuclear, anti-double-stranded DNA, anticentromere, anti-CENP-A, anti-CENP-B, anti-bicaudal D2, anti-nucleolar, anti-Scl-70 (anti-topoisomerase I), anti-topoisomerase II α, anti-RNP, anti-U1RNP, anti-U3RNP, anti-RNA polymerase III, anti-Th/To, anti-histone, antiphospholipid, anti-PmScl, anti-Sm, anti SSA (anti-Ro),anti SSB (La), anti-Ro52 (TRIM 21), anti-Ku, anti-B23, anti-RuvBL1, anti-RuvBL2, anti-fibrin bound tissue plasminogen activator, anti-endothelial cell, anti-phosphatidylserine-prothrombin complex, anti-endothelin-1 type A receptor, anti-angiotensin II type 1 receptor, anti‑carbonic anhydrase II, anti-fibroblast, anti-cyclic citrullinated peptide, anti-4-sulfated N-Acetyl-lactosamine, class I and II anti-human leukocyte antigen. Auto-antibodies were shown by different authors to be associated to this condition, with different prevalence values for each type of auto-antibody. Antinuclear antibodies, anti-centromere antibodies, antiphospholipid antibodies, anti-U3 RNP antibodies and anti-Th/To antibodies would appear to show a particularly important prevalence in scleroderma patients with pulmonary hypertension, appearing in about 8/10 (antinuclear), 1/ 2 (anti-centromere, anti-phospholipid), and 1/4 (anti-U3RNP, anti-Th/To) of patients. The available evidence points in the direction of a strong association between auto-immune mechanisms and pulmonary hypertension in the setting of scleroderma.

Keywords: Auto-antibodies; Pulmonary hypertension; Scleroderma; Systemic sclerosis.

Publication types

  • Review

MeSH terms

  • Autoantibodies / blood*
  • Autoantibodies / immunology*
  • Familial Primary Pulmonary Hypertension / blood
  • Familial Primary Pulmonary Hypertension / epidemiology*
  • Familial Primary Pulmonary Hypertension / immunology*
  • Humans
  • Prevalence
  • Scleroderma, Systemic / physiopathology*


  • Autoantibodies