TY - JOUR
T1 - Anti-U11/U12 Antibodies as a Rare but Important Biomarker in Patients with Systemic Sclerosis :
T2 - A Narrative Review
AU - Fritzler, Marvin J.
AU - Bentow, Chelsea
AU - Beretta, Lorenzo
AU - Palterer, Boaz
AU - Perurena-Prieto, Janire
AU - Sanz-Martínez, María Teresa
AU - Guillén-Del-Castillo, Alfredo
AU - Marín-Sánchez, Ana
AU - Fonollosa Pla, Vicent
AU - Callejas Moraga, Eduardo Luis
AU - Simeón-Aznar, Carmen Pilar
AU - Mahler, Michael
PY - 2023
Y1 - 2023
N2 - 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.
AB - 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.
KW - Autoantibodies
KW - Interstitial lung disease
KW - SSc
KW - Systemic sclerosis
KW - U11/U12
U2 - 10.3390/diagnostics13071257
DO - 10.3390/diagnostics13071257
M3 - Article
C2 - 37046475
SN - 2075-4418
VL - 13
JO - Diagnostics
JF - Diagnostics
ER -