Quantification of Antifibrillarin (anti-U3 RNP) Antibodies: A New Insight for Patients with Systemic Sclerosis
2021
Benyamine, Audrey | Bertin, Daniel | Resseguier, Noémie | Heim, Xavier | Bermudez, Julien | Launay, David | Dubucquoi, Sylvain | Hij, Adrian | Farge, Dominique | Lescoat, Alain | Bahon-Riedinger, Isabelle | Benmostefa, Nouria | Mouthon, Luc | Harlé, Jean-Robert | Kaplanski, Gilles | Rossi, Pascal | Bardin, Nathalie | Granel, Brigitte | Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research (C2VN) ; Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE) | Hôpital Nord [CHU - APHM] | Hôpital de la Conception [CHU - APHM] (LA CONCEPTION) | Hôpital de la Timone [CHU - APHM] (TIMONE) | Aix Marseille Université (AMU) | Institute for Translational Research in Inflammation - U 1286 (INFINITE) ; Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [CHU Lille] (CHRU Lille) | Centre Hospitalier Régional Universitaire [CHU Lille] (CHRU Lille) | Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO) | Hopital Saint-Louis [AP-HP] (AP-HP) ; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP) | Center of reference for rare systemic autoimmune diseases (FAI2R) | Recherche clinique appliquée à l'hématologie ((EA_3518)) ; Université Paris Diderot - Paris 7 (UPD7) | McGill University = Université McGill [Montréal, Canada] | Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou] | Hôpital Cochin [AP-HP] ; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
International audience
Show more [+] Less [-]English. Background: The detection of additional autoantibodies is of great concern in systemic sclerosis (SSc) when those included in the ACR/EULAR classification are negative. In this context, the interest of antifibrillarin (anti-U3RNP) autoantibodies (AFAs) in the routine evaluation of SSc remains unclear. We aimed to assess the relevance of AFAs and their clinical association in SSc patients. Methods: In a multicenter observational retrospective study, we collected immunological and clinical data associated with AFA positivity in SSc (n = 42) and non-SSc patients (n = 13). Patients with SSc negative for AFAs (n = 83) were considered as a control group. AFAs were detected by indirect immunofluorescence (IIF) using HEp-2 cells, EliA or immunoblot techniques. Results: We confirmed a typical nuclear IIF pattern and showed that AFAs are mostly exclusive towards SSc conventional autoantibodies. Although also observed in non-SSc patients, high levels of AFAs with the ELiA technique allowed the diagnosis of SSc. Compared to AFA-negative SSc patients, AFA-positive SSc patients more frequently exhibited visceral involvements. They more frequently suffered from the diffuse cutaneous form and had a higher global severity of the disease. Conclusions: We demonstrate the usefulness of quantifying AFAs in the immunological exploration of SSc, especially when patients are seronegative for SSc conventional autoantibodies and display a typical IIF pattern. AFAs might constitute an interesting marker of SSc severity.
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