Anti-SSA/SSB-negative primary Sjögren's syndrome showing different clinical phenotypes: a retrospective study of 934 cases

Chen, Jiaqi; He, Qian; Yang, Jianying; Wu, TzuHua; Huang, Ziwei; Zhang, Yan; Liao, Jiahe; Zhang, Lining; Yu, Xinbo; Yao, Chuanhui; Luo, Jing; Tao, Qingwen

doi:10.1186/s42358-023-00304-4

Acessibilidade / Reportar erro

Brasil

Español English

sumário « anterior atual seguinte »

Sumário

Research • Adv. rheumatol. 63 • 2023 • https://doi.org/10.1186/s42358-023-00304-4 copy

Anti-SSA/SSB-negative primary Sjögren's syndrome showing different clinical phenotypes: a retrospective study of 934 cases

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Background

Currently, only a few studies have described the general characteristics of patients with primary Sjögren's syndrome (pSS) who tested negatives for anti-SSA and anti-SSB antibodies. We aimed to further investigate the clinical characteristics of these patients in a large sample.

Methods

Data from patients with pSS who were treated at a tertiary hospital in China between 2013 and 2022 were retrospectively analyzed. Clinical characteristics of the patients were compared between those with and without anti-SSA and anti-SSB antibody negativity. Factors associated with anti-SSA and anti-SSB negativity were identified by logistic regression analysis.

Results

Overall, 934 patients with pSS were included in this study, among whom 299 (32.0%) tested negative for anti-SSA and anti-SSB antibodies. Compared with patients testing positive for anti-SSA or anti-SSB antibodies, that testing negative for the two antibodies had a lower proportion of females (75.3% vs. 90.6%, p < 0.001) and thrombocytopenia (6.7% vs. 13.6%, p = 0.002), but a higher proportion of abnormal Schirmer I tests (96.0% vs. 89.1%, p = 0.001) and interstitial lung disease (ILD) (59.2% vs. 28.8%, p = 0.001). Anti-SSA and anti-SSB negativity was positively associated with male sex (odds ratio [OR] = 1.86, 95% confidence interval [CI]: 1.05, 3.31), abnormal Schirmer I tests (OR = 2.85, 95% CI: 1.24, 6.53), and ILD (OR = 2.54, 95% CI: 1.67, 3.85). However, it was negatively related to thrombocytopenia (OR = 0.47, 95% CI: 0.24, 0.95).

Conclusion

Approximately one third of pSS patients had anti-SSA and anti-SSB negativity. pSS patients testing negative for anti-SSA and anti-SSB showed a higher risk of abnormal Schirmer I tests and ILD, but a lower risk of thrombocytopenia.

Keywords
Sjögren's syndrome; Anti-SSA; Anti-SSB; Clinical manifestation; Interstitial lung disease

Variables	Total N = 934	Patients with anti-SSA and anti-SSB negativity N = 299	Patients with anti-SSA and/or anti-SSB positivity N = 635	p-value ^* * Continuous variables are expressed as means ± standard deviation or medians [interquartile range (IQR)] and compared with Student's t-test or Wilcoxon's rank test; categorical variables are expressed as n (%) and compared with χ2 tests
Age (years), Mean ± SD	57.0 ± 12.6	61.7 ± 10.2	54.8 ± 13.1	< 0.001
Sex (female), n (%)	800 (85.7)	225 (75.3)	575 (90.6)	< 0.001
Disease duration (months), M (IQR)	925/36 (10-96)	24 (6-60)	48 (12-108)	< 0.001
Nationality, Han Chinese, n (%)	883 (94.5)	283 (94.6)	600 (94.5)	0.920
Others Chinese, n (%)	51 (5.5)	16 (5.4)	35 (5.5)
Dry mouth, n (%)	799 (85.5)	262 (87.6)	537 (84.6)	0.215
Dry eyes, n (%)	739 (79.1)	240 (80.3)	499 (78.6)	0.554
Fatigue, n (%)	466 (49.9)	148 (49.5)	318 (50.1)	0.869
Arthralgia, n (%)	361/925 (39.0)	102 (34.5)	259 (41.2)	0.051
Dental caries, n (%)	339/879 (38.6)	100 (36.1)	239 (39.7)	0.308
Palpable purpura, n (%)	60/926 (6.5)	14.12 (4.1)	48 (7.6)	0.045
Parotid enlargement, n (%)	131/932 (14.1)	35 (11.7)	96 (15.2)	0.156
Lymphadenopathy, n (%)	109/929 (11.7)	31 (10.5)	78 (12.3)	0.429
Schirmer I test ≤ 5 mm/5 min, n (%)	853 (91.3)	287 (96.0)	566 (89.1)	0.001
Interstitial lung disease, n (%)	360 (38.5)	177 (59.2)	183 (28.8)	< 0.001
Focus score on MSGB, M(IQR)	517/2 (1-4)	2 (1-4)	3 (1-4)	0.351

Variables	Total N = 934	Patients with anti-SSA and anti-SSB negativity N = 299	Patients with anti-SSA and/ or anti-SSB positivity N = 635	p-value ^* * Categorical variables are expressed as n (%) and compared with χ2 tests
Autoantibodies, n (%)
ANA titres > 1:320	258(27.6)	56 (18.7)	202 (31.8)	< 0.001
Positive anti-Ro52	533 (57.1)	71 (23.7)	462 (72.8)	< 0.001
Positive anti-CENP-B	69 (7.4)	38 (12.7)	31 (4.9)	< 0.001
Positive anti-RNP	66 (7.1)	20 (6.7)	46 (7.2)	0.757
Positive AMA-M2	81 (8.7)	29 (9.7)	52 (8.2)	0.444
Positive RF (> 20 lU/mL)	380/864(44.0)	62 (22.2)	318 (54.4)	< 0.001
Hematological characteristics, n (%)
Leucopenia (< 4 x 10⁹/L)	243/924 (26.3)	40 (13.4)	203 (32.4)	< 0.001
Neutropenia (< 1.5 x 10⁹/L)	75/924 (8.1)	11 (3.7)	64 (10.2)	0.001
Lymphopenia (< 0.8 x 10⁹/L)	71/924 (7.7)	17 (5.7)	54 (8.6)	0.119
Anemia (< 110 g/L)	179/924 (19.4)	32 (10.7)	147 (23.5)	< 0.001
Thrombocytopenia (100 x 10⁹/L)	105/924 (11.4)	20 (6.7)	85 (13.6)	0.002
Hyper-IgA (> 3.78 g/L)	247/917 (26.9)	62 (20.9)	185 (29.8)	0.004
Hyper-IgG (> 16.2 g/L)	416/917 (45.4)	84 (28.3)	332 (53.5)	< 0.001
Hyper-IgM (> 2.63 g/L)	72/917 (7.9)	29 (9.8)	43 (6.9)	0.136
Low C3 (< 0.7 g/L)	196/912 (21.5)	47 (15.9)	149 (24.1)	0.005
Low C4 (< 0.16 g/L)	311/912 (34.1)	75 (25.4)	236 (38.2)	< 0.001
Elevated ESR (> 20 mm/h)	430/868 (49.5)	113 (39.5)	317 (54.4)	< 0.001

Variables	OR	95%CI	P-value ^* * Multivariate logistic regression analysis
Age (years)	1.03	1.01, 1.05	0.002
Sex (male)	1.86	1.05, 3.31	0.034
Disease duration (months)	1.00	0.99, 1.00	0.018
Nationality (Han)	1.18	0.47, 2.98	0.727
Arthralgia	1.37	0.90, 2.10	0.143
Palpable purpura	0.53	0.22, 1.28	0.160
Schirmer I test < 5 mm/5 min	2.85	1.24, 6.53	0.013
ANA titers > 1:320	0.76	0.46, 1.25	0.278
Positive anti-Ro52	0.13	0.09, 0.19	< 0.001
Positive anti-CENP-B	4.17	2.04, 8.53	< 0.001
Positive RF (> 20 lU/mL)	0.40	0.25, 0.63	< 0.001
Leucopenia (< 4 x 10⁹/L)	0.98	0.45, 2.14	0.953
Neutropenia (< 1.5 x 10⁹/L)	0.61	0.25, 1.48	0.274
Anemia (< 110 g/L)	0.68	0.38, 1.19	0.171
Thrombocytopenia (100 x 10⁹/L)	0.47	0.24, 0.96	0.037
Hyper-IgA (> 3.78 g/L)	0.71	0.43, 1.17	0.181
Hyper-IgG (> 16.2 g/L)	0.78	0.49, 1.26	0.315
Low C3 (< 0.7 g/L)	1.02	0.60, 1.75	0.935
Low C4 (< 0.16 g/L)	0.83	0.53, 1.30	0.408
Elevated ESR (> 20 mm/h)	0.87	0.54, 1.41	0.564
Interstitial lung disease	2.54	1.67, 3.85	< 0.001

Sociedade Brasileira de Reumatologia Av. Brigadeiro Luís Antônio, 2466, Jardim Paulista, 01402-000 - São Paulo, SP, Tel.: +551132897165 - São Paulo - SP - Brazil
E-mail: rbreumatol@terra.com.br

Acompanhe os números deste periódico no seu leitor de RSS

[1] *Correspondence: Jing Luo luojinggg@sina.com Qingwen Tao taoqg1@sina.com