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. 2019 Dec 10;72(1):78–87. doi: 10.1002/acr.23839

Table 3.

Extraglandular manifestations in patients with juvenile Sjögren's syndromea

No. SGUS– SGUS+ P b Benjamin‐Hochberg adjustment
Extraglandular manifestations 56/62 (90)c 24/25 (96) 32/37 (86) 0.214 NS
Constitutional 24/59 (41)d 12/25 (48) 12/34 (35) 0.326 NS
Cutaneous 17/59 (29)e 8/25 (32) 9/34 (26) 0.643 NS
Lymphadenopathy 35/59 (59)f 17/25 (68) 18/34 (53) 0.245 NS
Articular 34/59 (58)g 17/25 (68) 17/34 (50) 0.167 NS
Muscular 5/59 (8) 1/25 (4) 4/34 (12) 0.290 NS
Pulmonary 1/59 (2) 1/25 (4) 0/34 (0) 0.240 NS
Renal 3/59 (5)h 1/25 (4) 2/34 (6) 0.745 NS
Peripheral nervous system 1/59 (2) 1/25 (4) 0/34 (0) 0.240 NS
Central nervous system 3/59 (5) 3/25 (12) 0/34 (0) 0.038 NS
Hematologic domain 16/58 (28)e 6/24 (25) 10/34 (29) 0.711 NS
Biologic domain 21/58 (36)i 9/24 (38) 12/34 (35) 0.863 NS
ESSPRI score, mean ± SD

3.5 ± 2.5

(n = 65)

3.2 ± 2.8 3.7 ± 2.3 0.368 NS
ESSPRI dryness, mean ± SD

3.4 ± 3.0

(n = 65)

3.4 ± 2.9 3.5 ± 3.1 0.919 NS
ESSPRI fatigue, mean ± SD

4.3 ± 3.4

(n = 65)j

3.8 ± 3.9 4.6 ± 3.0 0.314 NS
ESSPRI pain, mean ± SD

2.9 ± 3.2

(n = 65)

2.4 ± 3.4 3.2 ± 3.2 0.362 NS
Total ESSDAI score, mean ± SD

4.7 ± 5.4

(n = 62)

3.4 ± 2.8 5.5 ± 6.5 0.138 NS
Clinical ESSDAI, mean ± SD

3.2 ± 4.1

(n = 59)

2.7 ± 3.0 3.6 ± 4.8 0.443 NS
a

Values are the number of patients with positive findings/patients with available information (%), unless indicated otherwise. Regional differences refer to patients included in Brazil compared to patients included in Europe/US. SGUS = salivary gland ultrasonography; NS = nonsignificant; ESSPRI = European League Against Rheumatism (EULAR) Sjögren's Syndrome Patients Reported Index; ESSDAI = EULAR Primary Sjögren's Syndrome Disease Activity Index.

b

P values are for the comparison of SGUS+ and SGUS– patients.

c

Eight patients with systemic lupus erythematosus (SLE)/mixed connective tissue disease (MCTD), with regional differences.

d

Five patients with SLE/MCTD, with regional differences.

e

¶Five patients with SLE/MCTD.

f

Three patients with SLE/MCTD, with regional differences.

g

Eight patients with SLE/MCTD.

h

Three patients with SLE/MCTD.

i

Six patients with SLE/MCTD.

j

Regional differences.