Background
Methods
Clinical, biochemical and serological assessment
Patients | All | lcSSc | dcSSc |
---|---|---|---|
Gender, males/females, n | 19/102 | 3/76 | 16/26 |
Age, mean ± SD (range) years | 57 ± 12 (22-79) | 59 ± 11 | 55 ± 12 |
Disease duration, mean ± SD (range) years | 12 ± 9 (0-53) | 12 ± 9 | 11 ± 9 |
Cutaneous Involvement | |||
ᅟModified Rodnan skin score, mean ± SD | 11 ± 9 | 7 ± 3 | 20 ± 9 |
ᅟCutaneous ulcers, all, n | 68 | 44 | 24 |
ᅟCutaneous ulcers, present, n | 23 | 10 | 13 |
ᅟCutaneous ulcers, past, n | 45 | 34 | 11 |
ᅟCalcinosis, n (N)* | 43 (107) | 30 | 13 |
Pulmonary and Cardiac involvement | |||
ᅟDLCO, mean ± SD % predicted (N)* | 64.6 ± 19.9 (118) | 64.2 ± 18.5 (77) | 66.3 ± 20.3 (41) |
ᅟPatients with DLCO < 80 (%) | 75 % | 79 % | 74 % |
ᅟFVC, mean ± SD % predicted (N)* | 95.7 ± 21.1 (118) | 99.3 ± 20.1 (77) | 89.1 ± 21.7 (41)** |
ᅟPatients with FVC < 80 (%) | 22 % | 20 % | 27 % |
ᅟRadiographically confirmed lung fibrosis, n | 21 | 14 | 7 |
ᅟElevated SPAP by echocardiography, n (N)* | 10 (115) | 6 | 4 |
Serology | |||
ᅟAnti-nuclear antibodies, n | 116 | 74 | 42 |
ᅟAnti-Scl-70 antibodies, n | 15 | 3 | 12 |
ᅟAnti-centromere antibodies, n | 49 | 41 | 8 |
ᅟPeripheral blood | |||
ᅟLeukocytes total, mean ± SD (x 109/L) | 7.2 ± 2.2 | 7.4 ± 2.5 | 7.1 ± 2.3 |
ᅟNeutrophils, mean ± SD, (x 109/L) | 4.6 ± 1.8 | 4.7 ± 2.0 | 4.4 ± 1.6 |
ᅟPlatelets, mean ± SD, (x 109/L) | 275 ± 86 | 265 ± 86 | 268 ± 72 |
Others | |||
ᅟDMARDS, n | 8 | 4 | 4 |
ᅟCurrent or previous smokers, n | 83 | 57 | 26 |
ᅟArterial hypertension, n | 30 | 18 | 12 |
Blood sampling and isolation of platelet-poor plasma
Analysis of MPs by flow cytometry
Selectins
Statistical analysis
Results
Lung function and circulating E- and P-selectins
Lung function and circulating microparticles
Lung function | ||||
---|---|---|---|---|
DLco | FVC | |||
dcSSc | lcSSc | dcSSc | lcSSc | |
(n =41) | (n = 77) | (n =41) | (n = 77) | |
r | R | r | R | |
log(AnxV− EMPs) | −0.21 | −0.32** | −0.43** | −0.15 |
log(AnxV− LMPs) | −0.22 | −0.28* | −0.42** | −0.19 |
log(AnxV− PMPs) | −0.06 | −0.08 | −0.18 | −0.03 |
Clinical Variable | AnxV− EMPs | AnxV− LMPs | AnxV− PMPs | ||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
(MP x 105) | (MP x 104) | (MP x 105) | |||||||||||||
Symptom | No symptom | P | Symptom | No symptom | p | Symptom | No symptom | p | |||||||
Mean | ± SD | Mean | ± SD | Mean | ± SD | Mean | ± SD | Mean | ± SD | Mean | ± SD | ||||
Skin
| |||||||||||||||
Cutaneous ulcer | 8.01 | ±6.83 | 11.48 | ±16.11 | 0.64 | 9.47 | ±8.80 | 5.73 | ±31.49 | 0.84 | 4.59 | ±3.92 | 5.79 | ±7.88 | 0.66 |
Calcinosis | 10.75 | ±9.71 | 9.16 | ±13.68 |
0.04
| 14.04 | ±18.78 | 14.59 | ±33.08 | 0.07 | 6.87 | ±10.39 | 4.41 | ±4.46 | 0.17 |
Heart
| |||||||||||||||
SPAP > 40 | 18.46 | ±28.31 | 10.13 | ±13.10 | 0.63 | 24.64 | ±37.96 | 14.29 | ±27.95 | 0.96 | 4.63 | ±4.84 | 5.64 | ±7.54 | 0.43 |
Lung
| |||||||||||||||
Fibrosis (among all) | 18.06 | ±22.91 | 9.30 | ±12.26 |
0.02
| 23.67 | ±35.67 | 13.35 | ±27.08 | 0.14 | 5.29 | ±4.82 | 5.62 | ±7.79 | 0.83 |
Fibrosis (among dcSSc) | 22.13 | ±28.29 | 9.43 | ±12.36 |
0.02
| 28.14 | ±39.47 | 13.46 | ±27.34 | 0.13 | 7.19 | ±6.97 | 5.49 | ±7.80 | 0.54 |
Fibrosis (among lcSSc) | 16.03 | ±20.61 | 9.43 | ±12.36 | 0.12 | 21.44 | ±34.96 | 13.46 | ±27.34 | 0.40 | 4.34 | ±3.23 | 5.49 | ±7.80 | 0.87 |