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

Other authors

Institut Català de la Salut

[Fritzler MJ] Cumming School of Medicine, University of Calgary, Calgary, AB, Canada. [Bentow C] Research and Development, Werfen, Autoimmunity Headquarters and Technology Center, San Diego, CA, USA. [Beretta L] Scleroderma Unit and (Referral) Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milan, Milano, Italy. [Palterer B] Department of Experimental and Clinical Medicine, University of Florence, Firenze, Italy. [Perurena-Prieto J, Sanz-Martínez MT, Marín A] Servei d’Immunologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. [Guillen-Del-Castillo A, Fonollosa-Pla V, Simeón-Aznar CP] Àrea de Malalties Autoimmunes Sistèmiques, Unitat d’Inflamació i Autoimmunitat, Servei de Medicina Interna, Vall d’Hebron Hospital Universitari, Barcelona, Spain

Vall d'Hebron Barcelona Hospital Campus

Publication date

2023-05-04T11:47:45Z

2023-05-04T11:47:45Z

2023-03-27



Abstract

Autoantibodies; Interstitial lung disease; Systemic sclerosis


Autoanticuerpos; Enfermedad pulmonar intersticial; Esclerosis sistémica


Autoanticossos; Malaltia pulmonar intersticial; Esclerosi sistèmica


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.

Document Type

Article


Published version

Language

English

Publisher

MDPI

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https://doi.org/10.3390/diagnostics13071257

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Rights

Attribution 4.0 International

http://creativecommons.org/licenses/by/4.0/

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