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) |