干燥综合征与系统性红斑狼疮重叠综合征作为两种自身免疫性疾病交叉点上的独特实体：来自意大利这两种疾病的两个参考中心的临床特征

Sjögren's disease and systemic lupus erythematosus overlap syndrome as distinct entity at crossroads of two autoimmune disorders: clinical characterisation from two Italian reference centres for both the diseases.

作者信息

De Marchi Ginevra, Nano Alessia, Fulvio Giovanni, Manfrè Valeria, Navarro Garcia Inmaculada Conception, Zabotti Alen, Treppo Elena, Baldini Chiara, Quartuccio Luca, De Vita Salvatore

机构信息

Division of Rheumatology, Azienda Sanitaria Universitaria del Friuli Centrale, Santa Maria della Misericordia Hospital, Udine, Italy.

Division of Rheumatology, Azienda Sanitaria Universitaria del Friuli Centrale, Santa Maria della Misericordia Hospital, Udine; and Department of Medicine (DMED), University of Udine, Italy.

出版信息

Clin Exp Rheumatol. 2024 Dec;42(12):2453-2458. doi: 10.55563/clinexprheumatol/7v6qht. Epub 2024 Dec 10.

DOI:10.55563/clinexprheumatol/7v6qht

PMID:39656592

Abstract

OBJECTIVES

To characterise the overlap syndrome between Sjögren's disease (SjD) and systemic lupus erythematosus (SLE).

METHODS

Consecutive patients clinically defined as affected by SjD and SLE overlap syndrome (SjD-SLE), belonging to two Italian rheumatology centres were classified following the application of both the SjD and SLE classification criteria. Clinical, functional, ultrasound and histological data were compared with patients suffering from only SjD or SLE.

RESULTS

Compared to SjD controls, SjD-SLE patients were younger at onset (p<0.0001). Schirmer's test and parotid swelling were comparable between the two groups, while unstimulated sialometry was more impaired in the SjD controls (p=0.0001). SjD-SLE cases showed increased joint (p=0.009), mucocutaneous (p<0.0001), renal (p=0.001) involvement, and serositis (p<0.0001). Ultrasound changes in the major salivary glands were prevalent in SjD controls, while the histological findings of the minor salivary glands were similar. Furthermore, SjD-SLE cases presented a higher prevalence of anti-SSA (p<0.0001) and lower presence of rheumatoid factor (p=0.008) and serum cryoglobulins (p=0.035). Compared to SLE controls, SjD-SLE were older (p=0.044). The frequency of extra-glandular manifestations of SjD-SLE was similar compared to SLE, including renal involvement. SjD-SLE patients showed higher prevalence of anti-SSA and anti-SSB (p<0.0001), C4 reduction (p=0.011), and leukopenia (p=0.025).

CONCLUSIONS

Our data further highlight the limitations of the application of the current classification criteria in overlap syndrome, since they are primarily based on clinical manifestations and common autoantibodies. Molecular signatures may explain clinical similarities and differences among systemic autoimmune diseases, and they may be particularly helpful in overlap syndromes.

摘要

目的

描述干燥综合征（SjD）与系统性红斑狼疮（SLE）重叠综合征的特征。

方法

连续纳入两个意大利风湿病中心临床诊断为患有SjD和SLE重叠综合征（SjD-SLE）的患者，应用SjD和SLE分类标准进行分类。将临床、功能、超声和组织学数据与仅患有SjD或SLE的患者进行比较。

结果

与SjD对照组相比，SjD-SLE患者发病年龄更小（p<0.0001）。两组间Schirmer试验和腮腺肿大情况相当，而SjD对照组的非刺激性唾液流量测定受损更严重（p=0.0001）。SjD-SLE患者的关节受累（p=0.009）、黏膜皮肤受累（p<0.0001）、肾脏受累（p=0.001）和浆膜炎（p<0.0001）更为常见。主要唾液腺的超声改变在SjD对照组中更为普遍，而小唾液腺的组织学表现相似。此外，SjD-SLE患者抗SSA的患病率更高（p<0.0001），类风湿因子（p=0.008）和血清冷球蛋白的存在率更低（p=0.035）。与SLE对照组相比，SjD-SLE患者年龄更大（p=0.044）。SjD-SLE患者的腺外表现频率与SLE相似，包括肾脏受累。SjD-SLE患者抗SSA和抗SSB的患病率更高（p<0.0001），C4降低（p=0.011），白细胞减少（p=0.025）。