Table 2.
Subjective ocular and oral symptoms, objective signs of impaired tear secretion, salivary gland inflammation and hypofunction, serum autoantibodies, and fulfillment of classification criteria in all included patients (n = 67) with juvenile Sjögren's syndromea
| No. | SGUS– | SGUS+ | P b | Benjamin‐Hochberg adjustment | |
|---|---|---|---|---|---|
| Ocular symptoms (I) | 42/67 (63)c | 23/26 (88) | 19/41 (46) | 0.001 | Significant |
| Oral symptoms (II) | 53/66 (80) | 18/26 (69) | 30/40 (75) | 0.607 | NS |
| Salivary gland enlargement | 37/58 (64)d | 12/24 (50) | 25/34 (74) | 0.066 | NS |
| Ocular signs (III) | 33/65 (51)c | 16/24 (67) | 17/41 (41) | 0.05 | NS |
| Schirmer I test (ever) | 27/64 (42)e | 13/25 (52) | 14/39 (36) | 0.203 | NS |
| Ocular staining score | 20/37 (54) | 12/18 (67) | 8/19 (42) | 0.134 | NS |
| Focus score ≥1 (IV) | 28/34 (82) | 7/8 (88) | 21/26 (81) | 0.662 | NS |
| Focus score, mean ± SD |
1.7 ± 1.7 (n = 20) |
1.0 ± 0 | 1.9 ± 1.9 | 0.368 | NS |
| Salivary gland involvement (V) | 41/61 (67)f | 21/26 (81) | 20/35 (57) | 0.052 | NS |
| UWS ≤1.5 ml/minute | 20/60 (33)e | 9/26 (35) | 11/34 (32) | 0.854 | NS |
| UWS, mean ± SD ml/15 minutes |
4.3 ± 4.6 (n = 60) |
5.6 ± 5.8 | 3.3 ± 3.2 | 0.049 | NS |
| Sialography | 1/3 (33)g | 1/2 (50) | 0/1 (0) | 0.386 | NS |
| Sialo‐scintigraphy | 29/31 (9)h | 20/22 (91) | 9/9 (100) | 0.350 | NS |
| SGUS | 41/67 (61)i | – | – | – | – |
| Autoantibodies (VI) | 50/67 (75)c | 14/26 (54) | 36/41 (88) | 0.002 | Significant |
| ANA | 62/67 (93)d | 23/26 (88) | 39/41 (9) | 0.312 | NS |
| Anti‐Ro/SSA | 50/67 (75)j | 14/26 (54) | 36/41 (88) | 0.002 | Significant |
| Anti‐La/SSB | 27/67 (40)i | 4/26 (15) | 23/41 (56) | 0.001 | Significant |
| Anti‐Ro/SSA and anti‐La/SSB | 27/67 (40)i | 4/26 (15) | 23/41 (56) | 0.001 | Significant |
| Anti‐Ro/SSA or anti‐La/SSB | 50/67 (75)j | 14/26 (54) | 36/41 (88) | 0.002 | Significant |
| Rheumatoid factor | 29/64 (45)c | 5/25 (20) | 24/39 (62) | 0.001 | Significant |
| AECG criteria | 35/67 (52) | 14/26 (54) | 21/41 (51) | 0.834 | NS |
| ACR/EULAR criteria | 39/67 (58) | 15/26 (58) | 24/41 (59) | 0.946 | NS |
| ACR/EULAR and AECG criteria | 32/67 (48) | 13/26 (50) | 19/41 (46) | 0.770 | NS |
| ACR/EULAR or AECG criteria | 42/67 (63) | 16/26 (62) | 26/41 (63) | 0.877 | NS |
Values are the number of patients with positive findings/patients with available information (%), unless indicated otherwise. Roman numerals indicate the corresponding items of the American‐European Consensus Group (AECG) criteria. Regional differences refer to patients included in Brazil compared to patients included in Europe/US. SGUS = salivary gland ultrasonography; NS = nonsignificant; UWS = unstimulated whole saliva flow; ANA = antinuclear antibody; ACR = American College of Rheumatology; EULAR = European League Against Rheumatism.
P values are for the comparison of SGUS+ and SGUS– patients.
Regional differences.
Eight patients with systemic lupus erythematosus (SLE)/mixed connective tissue disease (MCTD).
Four patients with SLE/MCTD.
Six patients with SLE/MCTD, and 5 patients with SLE/MCTD, with regional differences.
One patient with SLE/MCTD, with regional differences.
Four patients with SLE/MCTD, with regional differences.
Three patients with SLE/MCTD, with regional differences.
Seven patients with SLE/MCTD, with regional differences.