| Autor: |
Fritzler MJ; Cumming School of Medicine, University of Calgary, Calgary, AB T2N 4N1, Canada., Bentow C; Research and Development, Werfen, Autoimmunity Headquarters and Technology Center, San Diego, CA 92131-1638, USA., Beretta L; Scleroderma Unit and (Referral) Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milan, 20122 Milano, Italy., Palterer B; Department of Experimental and Clinical Medicine, University of Florence, 50121 Firenze, Italy., Perurena-Prieto J; Department of Immunology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, 08193 Barcelona, Spain., Sanz-Martínez MT; Department of Immunology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, 08193 Barcelona, Spain., Guillen-Del-Castillo A; Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Universitari Vall d'Hebron, 08035 Barcelona, Spain., Marín A; Department of Immunology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, 08193 Barcelona, Spain., Fonollosa-Pla V; Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Universitari Vall d'Hebron, 08035 Barcelona, Spain., Callejas-Moraga E; Department of Internal Medicine, Hospital Público de Monforte, 27400 Lugo, Spain., Simeón-Aznar CP; Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Universitari Vall d'Hebron, 08035 Barcelona, Spain., Mahler M; Research and Development, Werfen, Autoimmunity Headquarters and Technology Center, San Diego, CA 92131-1638, USA. |
| Abstrakt: |
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. |
