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Table 2 Imunologic and hematologic characteristics of pSS patients with and without anti-SSA and anti-SSB antibody negativity

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

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*

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 IU/mL)

380/864(44.0)

62 (22.2)

318 (54.4)

< 0.001

Hematological characteristics, n (%)

Leucopenia (< 4 × 109/L)

243/924 (26.3)

40 (13.4)

203 (32.4)

< 0.001

Neutropenia (< 1.5 × 109/L)

75/924 (8.1)

11 (3.7)

64 (10.2)

0.001

Lymphopenia (< 0.8 × 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 × 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

  1. * Categorical variables are expressed as n (%) and compared with χ2 tests
  2. ANA, antinuclear antibodies; anti-CENP-B, anti-centromere protein B; anti-RNP, anti-ribonucleoprotein; AMA-M2, anti-mitochondrial M2 antibody; RF, rheumatoid factor; IgG, immunoglobulin G; IgA, immunoglobulin A; IgM, immunoglobulin M; C3, complement 3; C4, complement 4; ESR, erythrocyte sedimentation rate