Table 2 Key features of patients with Sjögren and an associated systemic autoimmune disease

From: 2023 International Rome consensus for the nomenclature of Sjögren disease

Feature

Primary Sjögren

Sjögren plus systemic autoimmune disease

SLE

SSc

RA

SpA

Sarcoidosis

IIM

AAV

IgG4-RD

Number of studies

NA

13

4

24

3

5

3

2

1

Number of European studies

NA

7

4

9

3

3

3

2

1

Prevalence of Sjögren in ADa

NA

606/4,133 (14.7)

222/1,511 (14.7)

361/3,681 (9.8)

20/111 (18.0)

50/2,974 (1.7)

34/99 (34.3)

No data

No data

Prevalence of AD in Sjögrenb

NA

56/670 (8.4)

No data

68/994 (6.8)

23/148 (15.5)

No data

21/1715 (1.2)

No data

10/133 (7.5)

Total patients with Sjögren plus AD

NA

726

222

673

91

88

55

66

10

Women, n/N (%)

14,308 (93.4)

637/652 (97.6)

349/441 (79.1)

466/505 (92.3)

66/91 (72.5)

48/88 (54.5)

50/55 (90.9)

55/66 (83.3)

9/10 (90.0)

Mean age at Sjögren diagnosis, years (range)

51.7 (NA)

42.4 (31–51.8)

52

55.1 (48–63.4)

45.7 (44–47)

50.4 (47–52.4)

54.3 (53–55.5)

61.9 (60–63.9)

No data

Dry mouth, n/N (%)

13,939 (91.0)

181/222 (81.5)

85/85 (100)

380/474 (80.2)

69/78 (88.4)

36/38 (94.7)

No data

22/22 (100)

10/10 (100)

Dry eyes, n/N (%)

14,059 (91.8)

175/222 (78.8)

85/85 (100)

395/475 (83.2)

67/78 (85.8)

33/34 (97.0)

No data

22/22 (100)

8/10 (80.0)

Abnormal Schirmer’s test, n/N (%)

10,037/12,709 (79.0)

137/181 (75.6)

19/19 (100)

225/331 (68.0)

No data

4/5 (80.0)

26/34 (76.5)

No data

No data

Positive corneal staining, n/N (%)

4,103/5,931 (69.2)

53/76 (69.7)

No data

158/256 (61.7)

No data

5/5 (100)

No data

No data

No data

Abnormal ocular tests, n/N (%)

11,495/13,569 (84.7)

78/90 (86.6)

24/28 (85.7)

152/207 (73.4)

No data

5/5 (100)

No data

No data

No data

Abnormal salivary flow test, n/N (%)

6,251/8,418 (74.3)

108/122 (88.5)

No data

168/220 (76.4)

12/13 (92.3)

No data

3/7 (42.9)

No data

No data

Abnormal parotid scintigraphy, n/N (%)

2,396/2,922 (82.0)

35/50 (70.0)

No data

97/122 (79.5)

ND

16/25 (64)

No data

No data

No data

Salivary biopsy grade 3–4, n/N (%)

8,974/10,379 (86.5)

67/77 (87.0)

58/59 (98.3)

120/158 (75.9)

77/91 (84.6)

34/37 (91.8)

27/30 (90.0)

56/66 (84.8)

No data

ANA, n/N (%)

11,383/15,180 (75.0)

98/127 (77.1)

53/59 (89.8)

227/300 (75.7)

33/43 (76.7)

18/26 (69.2)

22/28 (78.6)

18/22 (81.8)

6/10 (60)

RF, n/N (%)

6,265/15,056 (41.6)

124/334 (37.1)

56/85 (65.9)

441/518 (85.1)

13/78 (16.6)

11/26 (42.3)

No data

29/66 (43.9)

No data

Anti-SSA antibodies, n/N (%)

12,191/14,644 (83.2)

352/611 (57.6)

27/85 (31.7)

213/358 (59.5)

16/43 (37.2)

14/26 (53.8)

32/55 (58.2)

42/66 (63.6)

4/10 (40)

Anti-SSB antibodies, n/N (%)

6,090/1,3212 (46.1)

210/611 (34.3)

9/66 (13.6)

10/36 (27.8)

1/7 (14.2)

9/26 (34.6)

9/38 (23.7)

18/66 (27.2)

2/10 (20)

Low C3, n/N (%)

1,691/12,646 (13.4)

199/434 (45.8)

7/26 (26.9)

No data

2/23 (8.6)

No data

No data

No data

No data

Low C4, n/N (%)

1,685/12,615 (13.4)

203/434 (46.7)

3/26 (11.5)

No data

2/23 (8.6)

No data

No data

No data

2/10 (20)

  1. The table summarizes the key features of 1,931 patients with Sjögren disease (SjD) and an associated systemic autoimmune disease, from 55 individual studies identified in a systematic literature review27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81. Values in the ‘primary Sjögren’ column are derived from the Sjögren Big Data Consortium cohort26. Where data are expressed as ‘n/N’, n refers to the number of patients who have the evaluated feature, and N refers to the number of patients for whom information on that feature is available in the manuscript. aStudies focused on patients diagnosed with a specific autoimmune disease within which the occurrence of SjD was assessed. bStudies focused on patients with SjD within which the frequency of a concomitant autoimmune disease was assessed.AAV, anti-neutrophil cytoplasmic antibody-associated vasculitis; AD, autoimmune disease; ANA, antinuclear antibodies; IgG4-RD, IgG4-related disease; NA, not applicable; RA, rheumatoid arthritis; RF, rheumatoid factor; SSc, systemic sclerosis; SLE, systemic lupus erythematosus; SpA, spondyloarthritis.