Table 1

Characteristics of the 21 patients with primary Sjögren’s syndrome with anti-RNP antibodies at inclusion, as compared with other patients with primary Sjögren’s syndrome from the Paris-Sud cohort

CharacteristicsPrimary Sjögren with anti-RNP n=21Primary Sjögren without anti-RNP (Paris-Sud cohort), n=446P value
Number of women/men (ratio)18/3 (6)426/20 (21.3)0.0958
Classification
 Subjective xerostomia or xerophthalmia21 (100.0)442/446 (99.1)1
Objective xerostomia or xerophthalmia15/16(93.8)418/446(93.7)1
Lymphocytic sialadenitis (focus score ≥1)14(66.7)365/427(85.5)0.0293
 Median Chisholm score4.0 (2.0–4.0)3.0 (3.0–4.0)0.8503
Positive anti-SSA antibodies19(90.5)298/444(67.1)0.0288
 Positive anti-SSB antibodies5 (23.8)166/442 (37.6)0.2022
 Positive ACR/EULAR 2016 SGS criteria21 (100.0)446 (100.0)
 Positive anti-DNA antibodies0 (0.0)0 (0.0)
 Positive anti-Sm antibodies6 (28.6)0 (0.0)
 Positive anti-RNP antibodies21 (100.0)0 (0.0)
 Positive MCTD criteria at inclusion3 (14.3)0 (0.0)
 Sharp et al0 (0.0)0 (0.0)
 Kasukawa and Sharp3 (14.3)0 (0.0)
 Alarcon-Segovia and Villareal2 (9.5)0 (0.0)
Age at onset of Sjögren’s symptoms41.0 (28.048.0)500 (36.062.0)0.0123
ESSDAI at inclusion8.0(5.017.0)3.0 (1.06.0)*<0.0001
Systemic manifestations
Constitutional symptoms3(14.3)2/361(0.01)0.0013
 Parotid gland involvement6 (28.6)170/438 (38.8)0.3457
 Joint involvement18 (85.7)335/444 (75.4)0.4329
 Arthralgia18 (85.7)320/441 (72.6)
 Non-erosive arthritis4 (19.0)48/438 (11.0)
 Myalgia5 (23.8)132/438 (30.1)0.5359
Myositis4(19.0)10/436(2.3)0.0025
Pulmonary interstitial lung disease4(19.0)25/437(5.7)0.0366
 NSIP with altered lung diffusion, normal TLC2 (9.5)
 USIP with altered lung diffusion, decreased TLC1 (4.8)
 Ground glass opacities and bronchectasis1 (4.8)
 Cutaneous involvement4 (19.0)161/442 (36.4)0.1042
 Isolated sclerodactyly1 (4.8)NA
 Vascular purpura2 (9.5)17/436 (3.9)
 Histology showing leucocytic vasculitis1 (4.8)NA
 Raynaud phenomenon11 (52.4)145/437 (33.2)0.0698
 Peripheral nervous system involvement2 (9.5)13/419 (3.1)0.1567
 Sensitive polyneuropathy—EMG confirmed1 (4.8)NA
 Trigeminal neuralgia1 (4.8)NA
 Central nervous system involvement0 (0.0)5/433 (1.2)1
 Renal involvement0 (0.0)1/124 (0.8)1
 Lymphoma1 (4.8)16/443 (3.6)0.5514
Treatment
 Corticosteroids11 (52.4)149/438 (34.0)0.0845
 NSAIDs4 (19.0)167/432 (38.7)0.1044
 Hydroxychloroquine11 (52.4)142/436 (32.6)0.0602
 Methotrexate3 (14.3)28/434 (6.5)0.1650
 Cyclophosphamide3 (14.3)NA
 Rituximab3 (14.3)NA
 Azathioprine2 (9.5)NA
 Mycophenolate mofetil1 (4.8)NA
  • Results are presented as number (%), or median (IQR).

  • Bold values are statistically significant.

  • *Data available for 129 patients.

  • ACR, American College of Rheumatology; EMG, electromyogram; ESSDAI, EULAR Sjögren’s syndrome disease activity index; MCTD, mixed connective tissue disease; NA, not available; NSAIDs, non-steroidal anti-inflammatory drugs; NSIP, non-specific interstitial pneumonia; TLC, total lung capacity; USIP, usual interstitial pneumonia.