Introduction
Materials and methods
Patients
Data collection
First symptom | Disease category | |||
---|---|---|---|---|
ACA+/SS (n = 20) [%] | SSc/(-) sicca (n = 20) [%] | SSc/(+) sicca (n = 31) [%] | ACA-/SS (n = 61) [%] | |
Raynaud phenomenon | 6 [30.00] | 18 [90.00] | 29 [93.55] | 5 [8.20] |
sicca manifestations | 15 [75.00] | - | 6 [19.35] | 52 [85.25] |
SGE | 1 [5.00] | - | 1 [3.23] | 12 [19.67] |
arthritis | 1 [5.00] | 1 [5.00] | 2 [6.45] | 4 [6.56] |
purpura, | - | - | - | 4 [6.56] |
puffy fingers/sclerodactyly | 1 [5.00] | 6 [30.00] | 8 [25.81] | 4 [6.56] |
digital ulcers | - | 2 [10.00] | 1 [3.23] | - |
dysphagia/GER | 1 [5.00] | 1 [5.00] | 1 [3.23] | - |
lung fibrosis | - | - | - | 1 [1.64] |
Statistical analysis
Results
Demographics, follow-up and disease duration of cases and controls
Disease |
Characteristics
| |||||
---|---|---|---|---|---|---|
Age at first symptom (P= 0.108)α | Age at first non-Raynaud symptom (P= 0.279)α | |||||
Mean
|
SD
|
Mean difference (P-value)
b
|
Mean
|
SD
|
Mean difference (P-value)
b
| |
ACA+/SS (n = 20)
| 52.35 | 15.0 | 53.55 | 15.2 | ||
SSc/(+) sicca (n = 31)
| 43.29 | 15.8 | 0.187 | 47.23 | 13.2 | 0.699 |
SSc/(-) sicca (n = 20)
| 42.45 | 15.5 | 0.196 | 47.3 | 15.7 | 0.954 |
ACA-/SS (n = 61)
| 46.52 | 13.31 | 0.729 | 46.6 | 13.3 | 0.341 |
Disease duration from onset of first symptom (P < 0.0001)α | Disease duration from onset of first non-Raynaud symptom (P = 0.036)α | |||||
Mean
|
SD
|
Mean difference (P-value)
b
|
Mean
|
SD
|
Mean difference (P-value)
b
| |
ACA+/SS (n = 20)
| 12.13 | 7.4 | 10.96 | 7.0 | ||
SSc/(+) sicca (n = 31)
| 17.51 | 11.1 | 0.248 | 13.48 | 6.7 | 1.000 |
SSc/(-) sicca (n = 20)
| 15.99 | 11.7 | 1.000 | 11.20 | 8.6 | 1.000 |
ACA-/SS (n = 61)
| 8.89 | 7.4 | 1.000 | 8.78 | 7.3 | 1.000 |
Duration of Follow up (P = 0.012)α | - | |||||
Mean
|
SD
|
Mean difference (P-value)
b
|
-
| |||
ACA+/SS (n = 20)
| 5.38 | 5.1 |
-
| |||
SSc/(+) sicca (n = 31)
| 7.87 | 5.2 | 0.494 |
-
| ||
SSc/(-) sicca (n = 20)
| 4.00 | 4.1 | 1.000 |
-
| ||
ACA-/SS (n = 61)
| 4.49 | 5.0 | 1.000 |
-
|
Characteristics of patients (P-value1) | ACA+/SS (n = 20) [%] | SSc/(+) sicca (n = 31) [%] | SSc/(-) sicca (n = 20) [%] | ACA-/SS (n = 61) [%] |
---|---|---|---|---|
Sex(0.558) | ||||
female
| 20 [100.0] | 28 [90.3] | 19 [95.0] | 57 [93.4] |
male
| 0 [0.0] | 3 [9.7] | 1 [5.0] | 4 [6.6] |
P-values
2
| 0.271 | 1.000 | 0.567 | |
Smoking (0.603) | 4 [20.0] | 5 [16.1] | 2 [10.0] | 14 [23.0] |
P --values
2
| 0.703 | 0.661 | 1.000 | |
Anti-Ro (<0.0001)
| 6 [30.0] | 4 [12.9] | 0 [0.0] | 43 [70.5] |
P-values
2
| 0.163 |
0.020
|
0.003
| |
Anti-La (<0.0001) | 3 [15.0] | 2 [6.5] | 0 [0.0] | 25 [41.0] |
P-values
2
| 0.369 | 0.231 |
0.056
| |
ANA (0.006)
| 20 [100.0] | 31 [100.0] | 20 [100.0] | 51 [83.6] |
P-values
2
| - |
-
| 0.060 | |
Anti-U1RNP (0.558) | 0 [0.0] | 3 [9.7] | 1 [5.0] | 4 [6.6] |
P-values
2
| 0.271 | 1.000 | 0. 567 | |
Anti-Sm (0.721) | 0 [0.0] | 1 [3.2] | 0 [0.0] | 2 [3.3] |
P-values
2
| 1.000 | - | 1.000 | |
Anti-Scl70 (0.172) | 0 [0.0] | 2 [6.5] | 1 [5.0] | 0 [0.0] |
P-values
2
| 0.514 | 1.000 | - | |
Th. Hashimoto (0.023)
| 1 [5.0] | 4 [12.9] | 0 [0.0] | 15 [24.6] |
P-values
2
| 0.636 | 1.000 | 0.102 | |
Autoim. Hepatitis (-) | 0 [0.0] | 0 [0.0] | 0 [0.0] | 0 [0.0] |
P-values
2
| - | - | - | |
Atr. gastritis (0.422) | 2 [10.0] | 2 [6.5] | 0 [0.0] | 2 [3.3] |
P-values
2
| 0.640 | 0.487 | 0.254 | |
PBC (0.209) | 3 [15.0] | 3 [9.7] | 0 [0.0] | 3 [4.9] |
P-values
2
| 0.668 | 0.231 | 0.157 | |
Celiac (0.209) | 0 [0.0] | 0 [0.0] | 0 [0.0] | 1 [1.6] |
P-values
2
| - | - | 1.000 | |
Lymphoma (0.476) | 0 [0.0] | 2 [6.5] | 0 [0.0] | 2 [3.3] |
P-values
2
| 0.514 | - | 1.000 | |
Death (0.059) | 0 [0.0] | 3 [9.7] | 1 [5.0] | 0 [0.0] |
P-values
2
| 0.271 | 1.000 | - |
Immunological profile and histological findings of cases and controls
Characteristics of patients
(P-value1) | ACA+/SS (n = 20) [%] | SSc/(+) sicca (n = 31) [%] | SSc/(-) sicca (n = 20) [%] | ACA-/SS (n = 61) [%] |
---|---|---|---|---|
Positive MSGB (0.039)
| 18 [94.7] | 12 [75.0] | - | 54 [94.7] |
P-values
2
| 0.156 | 1.000 | ||
Abnormal Rose Bengal (0.364) | 8 [72.7] | 9 [56.3] | 0 [0.0] | 22 [71.0] |
P-values
2
| 0.448 | 0.333 | 1.000 | |
Abnormal Schirmer test (0.176) | 9 [64.3] | 14 [87.5] | 0 [0.0] | 35.5 [71.4] |
P-values
2
| 0.204 | 0.400 | 0.743 | |
Abnormal BUT (0.428) | 7 [77.8] | 9 [75.0] | 0 [0.0] | 18 [69.2] |
P-values
2
| 1.000 | 0.300 | 1.000 | |
Lymphadenopathy (0,249) | 3 [15.0] | 8 [25.8] | 1 [5.0] | 9 [14.8] |
P-values
2
| 0.493 | 0.605 | 1.000 | |
Raynaud (<0.0001)
| 15 [75.0] | 31 [100.0] | 20 [100.0] | 11 [18.0] |
P-values
2
|
0.007
|
0.047
|
<0.0001
| |
SGE (0.006)
| 3 [15.0] | 4 [12.9] | 0 [0.0] | 20 [32.8] |
P-values
2
| 1.000 | 0.231 | 0.160 | |
Purpura (0,052)
| 2 [10.0] | 1 [3.2] | 0 [0.0] | 11 [18.0] |
P-values
2
| 0.553 | 0.487 | 0.502 | |
Xerostomia (<0.0001)
| 19 [95.0] | 25 [80.6] | 0 [0.0] | 53 [86.9] |
P-values
2
| 0.223 |
<0.0001
| 0.440 | |
Dry eyes (<0.0001)
| 17 [85.0] | 29 [93.5] | 0 [0.0] | 60 [98.4] |
P-values
2
| 0.369 |
<0.0001
|
0.045
| |
Telangiectasias (<0.0001)
| 2 [10.0] | 28 [90.3] | 14 [70.0] | 2 [3.3] |
P-values
2
|
<0.0001
|
<0.0001
| 0.254 | |
Puffy fingers (<0.0001)
| 7 [35.0] | 26 [83.9] | 14 [70.0] | 10 [16.4] |
P-values
2
|
0.001
|
0.056
| 0.112 | |
Sclerodactyly (<0.0001)
| 0 [0.0] | 31 [100.0] | 19 [95.0] | 0 [0.0] |
P-values
2
|
<0.0001
|
<0.0001
| - | |
Calcinosis (0. 001)
| 0 [0.0] | 6 [19.4] | 4 [20.0] | 0 [0.0] |
P-values
2
| 0.070 | 0.106 |
-
| |
Ulcers (0.249) | 2 [10.0] | 17 [54.8] | 11 [55.0] | 0 [0.0] |
P-values
2
|
0.001
|
0.006
|
0.059
| |
Dysphagia (<0.0001)
| 7 [35.0] | 18 [58.1] | 5 [25.0] | 4 [6.6] |
P-values
2
| 0.153 | 0.731 |
0.004
| |
GER (<0.0001)
| 3 [15.0] | 20 [64.5] | 10 [50.0] | 6 [9.8] |
P-values
2
|
0.001
|
0.041
| 0.682 | |
Arthritits (0.372) | 8 [35.0] | 10 [32.3] | 4 [20.0] | 14 [23.0] |
P-values
2
| 0.765 | 0.301 | 0.156 | |
Dyspnoea (<0.0001)
| 7 [40.0] | 20 [64.5] | 5 [25.0] | 9 [14.8] |
P-values
2
|
0.049
| 0.731 | 0.060 | |
PAH (0.004)
| 2 [10.0] | 8 [25.8] | 2 [10.0] | 1 [1.6] |
P-values
2
| 0.280 | 1.000 | 0.149 | |
Ground glass HRCT (0.606) | 0 [0.0] | 4 [21.1] | 1 [9.1] | 1 [12.5] |
P-values
2
| 0.544 | 1.000 | 1.000 | |
Lung fibrosis (0.001)
| 2 [10.0] | 12 [38.7] | 7 [35.0] | 5 [8.2] |
P-values
2
|
0.029
| 0.127 | 1.000 | |
Restrict. pattern PFTs (0.103) | 2 [22.2] | 8 [30.8] | 6 [42.9] | 0 [0.0] |
P-values
2
| 1.000 | 0.400 | 0.211 | |
Serositis (0.080) | 3 [15.0] | 6 [19.4] | 3 [15.0] | 2 [3.3] |
P-values
2
| 1.000 |
0.019
| 0.094 | |
Renal involvement (0.760) | 0 [0.0] | 0 [0.0] | 0 [0.0] | 1 [1.6] |
P-values
2
|
-
|
-
| 1.000 | |
Carpal tunnel (0.853) | 2 [10.0] | 3 [9.7] | 3 [15.0] | 5 [4.9] |
P-values
2
| 1.000 | 1.000 | 1.000 | |
Per. Neuropathy (0.743) | 1 [5.0] | 2 [6.5] | 0 [0.0] | 3 [86.9] |
P-values
2
| 1.000 | 1.000 | 1.000 | |
Leucopenia (0,088) | 2 [10.0] | 2 [6.5] | 2 [10.0] | 15 [24.6] |
P-values
2
| 0.640 | 1.000 | 0.216 | |
Hypergammaglobulinaemia (0.0001)
| 5 [25.0] | 6 [19.4] | 1 [5.0] | 31 [50.8] |
P-values
2
| 0.732 | 0.182 |
0.068
| |
Cryoglobulinaemia (0.609) | 0 [0.0] | 2 [6.5] | 1 [5.0] | 5 [4.9] |
P-values
2
| 0.514 | 1.000 | 0.326 | |
RF (0.015)
| 5 [25.0] | 6 [19.4] | 4 [20.0] | 29 [47.5] |
P-values
2
| 0.732 | 1.000 | 0.117 | |
Low C3,C4 (0.289) | 0 [0.0] | 5 [20.0] | 3 [23.1] | 10 [19.6] |
P-values
2
| 0.137 | 0.087 | 0.101 | |
Proteinuria (0.760) | 0 [0.0] | 0 [0.0] | 0 [0.0] | 1 [1.6] |
P-values
2
|
-
|
-
| 1.000 |