Table 4 Clinical profiles of patients with SSc in the presence or absence of gAChR Abs
Characteristics | SSc with gAChR Abs (n = 7) | SSc without gAChR Abs (n = 43) | p value |
|---|---|---|---|
Age (years) | 62.9 ± 14.0 | 62.1 ± 9.0 | 0.764 |
Age at onset (years) | 57.1 ± 12.5 | 54.8 ± 12.4 | 0.646 |
Disease duration (years) | 5.7 ± 5.6 | 7.3 ± 8.7 | 0.911 |
Sex, female (%) | 6 (85.7) | 38 (88.4) | 1.000 |
Diffuse SSc (%) | 4 (57.1) | 13 (30.2) | 0.451 |
Raynaud’s phenomenon (%) | 7 (100.0) | 43 (100.0) | 1.000 |
Digital ulcers (%) | 2 (28.6) | 7 (16.3) | 0.615 |
Renal crisis (%) | 1 (14.3) | 1 (2.3) | 0.287 |
Interstitial pneumonitis (%) | 4 (57.1) | 16 (37.2) | 0.717 |
Pulmonary hypertension (%) | 1 (14.3) | 2 (4.7) | 0.394 |
GI manifestations | |||
 GERD (%) | 3 (42.9) | 11 (25.6) | 0.677 |
 Paralytic ileus (%) | 1 (14.3) | 1 (2.3) | 0.287 |
 Constipation (%) | 2 (28.6) | 8 (18.6) | 0.637 |
 Diarrhea (%) | 1 (14.3) | 0 (0.0) | 0.157 |
Other Abs | |||
 ACA (+) (%) | 3 (42.9) | 22 (51.2) | 1.000 |
 Anti-Scl70 Abs (+) (%) | 2 (28.6) | 10 (23.3) | 1.000 |
 Other Abs (+) (%) | 1 (14.3) | 4 (9.3) | 0.559 |
 Negative (%) | 1 (14.3) | 7 (16.3) | 1.000 |
Other laboratory data | |||
 IgG (mg/dL) | 1775.0 ± 614.2 | 1548.8 ± 363.6 | 0.459 |
 KL-6 (U/mL) | 666.0 ± 678.3 | 572.6 ± 642.4 | 0.534 |
 NT-proBNP (pg/mL) | 114.1 ± 128.9 | 217.4 ± 418.6 | 0.530 |
 %FVC | 109.8 ± 25.5 | 98.8 ± 22.3 | 0.311 |
 FEV1.0% | 78.5 ± 13.6 | 79.9 ± 10.0 | 0.786 |
 %DLCO | 61.9 ± 23.8 | 71.7 ± 17.5 | 0.262 |
 DLCO/VA | 70.5 ± 15.6 | 85.7 ± 17.2 | 0.068 |
 TR-PG | 28.4 ± 17.1 | 24.0 ± 7.9 | 0.953 |
 Estimated PA systolic pressure | 26.5 ± 8.5 | 29.3 ± 8.4 | 0.528 |
 EF | 71.6 ± 3.4 | 70.8 ± 6.7 | 0.800 |
Serum biomarkers | |||
 VEGF (pg/mL) | 441.6 ± 112.1 | 480.2 ± 476.5 | 0.631 |
 PIGF (pg/mL) | 16.6 ± 6.6 | 14.8 ± 8.6 | 0.482 |
 GDF-15 (pg/mL) | 1595.9 ± 1529.0 | 1423.1 ± 1086.5 | 0.941 |
 PTX3 (ng/mL) | 2.3 ± 1.2 | 3.1 ± 1.5 | 0.336 |
 Endostatin (ng/mL) | 101.8 ± 21.4 | 83.3 ± 18.7 | 0.046* |
 TGFβ1 | 13,038.6 ± 12,382.6 | 13,334.3 ± 7692.2 | 0.506 |
Immunotherapies | |||
 Oral PSL (%) | 1 (14.3) | 6 (14.0) | 1.000 |
 Immunosuppressants (%) | 1 (14.3) | 3 (7.0) | 0.532 |
 Steroid pulse therapy (%) | 0 (0.0) | 0 (0.0) | 1.000 |
 IVIg (%) | 0 (0.0) | 0 (0.0) | 1.000 |
 Plasmapheresis (%) | 0 (0.0) | 0 (0.0) | 1.000 |