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
Internal Medicine Department, North Hospital of Marseilles, Public Assistance Hospital of Marseilles (AP-HM), 13015 Marseilles, France.
Aix Marseilles University (AMU), INSERM, INRA, C2VN, 13005 Marseilles, France.
Diagnostics (Basel). 2021 Jun 9;11(6):1064. doi: 10.3390/diagnostics11061064.
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.
In a multicenter observational retrospective study, we collected immunological and clinical data associated with AFA positivity in SSc ( = 42) and non-SSc patients ( = 13). Patients with SSc negative for AFAs ( = 83) were considered as a control group. AFAs were detected by indirect immunofluorescence (IIF) using HEp-2 cells, EliA or immunoblot techniques.
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.
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.
当美国风湿病学会（ACR）/欧洲抗风湿病联盟（EULAR）分类中包含的自身抗体检测为阴性时，系统性硬化症（SSc）中其他自身抗体的检测备受关注。在此背景下，抗纤维核蛋白（抗U3RNP）自身抗体（AFAs）在SSc常规评估中的意义仍不明确。我们旨在评估AFAs在SSc患者中的相关性及其临床关联。
在一项多中心观察性回顾性研究中，我们收集了SSc患者（n = 42）和非SSc患者（n = 13）中与AFA阳性相关的免疫学和临床数据。将AFAs检测为阴性的SSc患者（n = 83）作为对照组。使用HEp-2细胞通过间接免疫荧光法（IIF）、酶联免疫吸附测定（EliA）或免疫印迹技术检测AFAs。
我们证实了一种典型的核IIF模式，并表明AFAs大多与SSc传统自身抗体互斥。尽管在非SSc患者中也观察到，但EliA技术检测到的高水平AFAs有助于SSc的诊断。与AFA阴性的SSc患者相比，AFA阳性的SSc患者更常出现内脏受累。他们更常患弥漫性皮肤型，疾病的整体严重程度更高。
我们证明了在SSc的免疫学检查中量化AFAs的有用性，特别是当患者的SSc传统自身抗体血清学阴性且显示典型IIF模式时。AFAs可能构成SSc严重程度的一个有趣标志物。
