Anti-U11/U12 Antibodies as a Rare but Important Biomarker in Patients with Systemic Sclerosis : A Narrative Review

Marvin J. Fritzler, Chelsea Bentow, Lorenzo Beretta, Boaz Palterer, Janire Perurena-Prieto, María Teresa Sanz-Martínez, Alfredo Guillén-Del-Castillo, Ana Marín-Sánchez, Vicent Fonollosa Pla, Eduardo Luis Callejas Moraga, Carmen Pilar Simeón-Aznar, Michael Mahler

Research output: Contribution to journalArticleResearchpeer-review

5 Citations (Scopus)

Abstract

Anti-nuclear (ANA) are present in approximately 90% of systemic sclerosis (SSc) patients and are key biomarkers in supporting the diagnosis and determining the prognosis of this disease. In addition to the classification criteria autoantibodies for SSc [i.e., anti-centromere, anti-topoisomerase I (Scl-70), anti-RNA polymerase III], other autoantibodies have been associated with important SSc phenotypes. Among them, anti-U11/U12 ribonucleoprotein (RNP) antibodies, also known as anti-RNPC-3, were first reported in a patient with SSc, but very little is known about their association and clinical utility. The U11/U12 RNP macromolecular complex consists of several proteins involved in alternative mRNA splicing. More recent studies demonstrated associations of anti-anti-U11/U12 antibodies with SSc and severe pulmonary fibrosis as well as with moderate to severe gastrointestinal dysmotility. Lastly, anti-U11/U12 autoantibodies have been strongly associated with malignancy in SSc patients. Here, we aimed to summarize the knowledge of anti-U11/U12/RNPC-3 antibodies in SSc, including their seroclinical associations in a narrative literature review.
Original languageEnglish
JournalDiagnostics
Volume13
DOIs
Publication statusPublished - 2023

Keywords

  • Autoantibodies
  • Interstitial lung disease
  • SSc
  • Systemic sclerosis
  • U11/U12

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