PKB

The proportion of dcSSc to lcSSc, although atypical for systemic sclerosis subsets reflects the patient population being covered by our department with a predominance of the more severe dcSSc type who typically require more aggressive treatment and are referred to our department

The proportion of dcSSc to lcSSc, although atypical for systemic sclerosis subsets reflects the patient population being covered by our department with a predominance of the more severe dcSSc type who typically require more aggressive treatment and are referred to our department. The mean age of all patients was 54.5 11.1 years. was noted. Head to head comparison between cyclophosphamide (CYC) and mycophenolate mofetil (MMF) treatment showed a reduction of CD19+ cells, but increment of plasmablasts in CYC treated patients. MannCWhitney test was used. The assessment between variables was done based on the Pearson correlation coefficient and with stepwise backward multivariable linear regression models. The CookCWeisberg test and Cameron & Trivedis decomposition test was used to test the residuals for heteroskedasticity as well as the violation of skewness and kurtosis assumptions in linear regression. Multicollinearity was evaluated by calculating the variance inflation factor (VIF), which should not exceed 5. The one way analysis of variance (ANOVA) has been used to compare the treatment groups to the control group and healthy subjects. 3. Results 3.1. Patients Characteristics A total number of 46 patients with systemic sclerosis were included into the study. The diffuse type of the disease was diagnosed in 36 (78.3%) patients, the limited form in 10 (21.7%) patients. The proportion of dcSSc to lcSSc, although atypical for systemic sclerosis subsets reflects the patient population being covered by our department with a predominance of the more severe dcSSc type who typically require more aggressive treatment and are referred to our department. The mean age of all patients was 54.5 11.1 years. The mean duration of the disease from first non-Raynauds symptom (attributable to systemic sclerosis) was 6.7 7.8 years in all patients. The concomitant diseases diagnosed in patients were interstitial lung disease, arterial hypertension, pulmonary arterial hypertension, digital ulcers, arthralgia, and esophagus dysmotility. Patients underwent immunosuppressive treatment as required on clinical status, that included cyclophosphamide, methotrexate, azathioprine, and mycophenolate mofetil. Twelve patients had no immunosuppressive therapy. The clinical and demographic characteristics of the SSc patients included are summarized in Table 1. Table 1 Demographic characteristics, clinical severity, and disease damage in patients with SSc. (%)= 46)Controls (= 20)Gender (Female/Male)31/1511/9Age (years)54.5 11.153.9 12.5Disease duration since first non-Raynauds symptom (years)6.7 7.8 Disease duration 1-Methylpyrrolidine (since formal diagnosis) (years)4.5 2.3 Age at the time of disease diagnosis (years)48.7 12.0 Disease typeDiffuse type (dcSSc), (%)36 (78.26%) Limited type (lcSSc), (%)10 (21.74%) mRSS (points)11.1 9.5 EUSTAR 2017 (points)2.1 2.1 Autoantibody presence, (%)ANA46 (100) Topoisomerase I (SCL-70)29 (63.04) CENP-B10 (21.74) SSA. SSB. Ro528 (17.39) Clinical characteristics Interstitial lung disease SSc-ILD, (%)27 (58.69) PKN1 Esophagus dysmotility, (%)24 (52.17) Arthralgia, (%)19 (41.30) Pitting scars, (%)12 (26.08) Digital ulceration, (%)9 (19.56) Arrhythmia, (%)9 (19.56) Pulmonary arterial hypertension, PAH, (%)8 (17.39) LVEF 50%4 (8.69) Digital necrosis, (%)3 (6.52) Treatment Mycophenolate mofetil (MMF), (%)19/46 (41.3) Cyclophosphamide (CYC), (%)11/46 (23.9) Azathioprine (AZA), (%)2/46 (4.3) 1-Methylpyrrolidine Metotrexate (MTX), (%)2/46 (4.3) Disease severity according to Medsger scale General healthPeripherial vascularSkinJoints/tendonsMusclesGILungsHeartKindey No changes, (%)37 (80.4)8 (17.4)5 (10.9)11 (23.9)37 (80.4)23 (50.0)15 (32.6)38 (82.6)45 (97.8) Mild, (%)6 (13.0)22 (47.8)27 (58.7)24 (52.2)7 (15.2)23 (50.0)23 (50.0)2 (4.3)0 (0.0) Moderate, (%)2 (4.3)10 (21.7)12 (26.1)9 (19.6)2 (4.3)0 (0.0)4 (8.6)1 (2.2)1 (2.2) Severe, (%)1 (2.2)4 (8.6)2 (4.3)2 (4.3)0 (0.0)0 (0.0)2 (4.3)5 (10.9)0 (0.0) End-stage, (%)0 (0.0)2 (4.3)0 (0.0)0 (0.0)0 (0.0)0 (0.0)2 (4.3)0 (0.0)0 (0.0) Open in a separate window mRSS, modified Rodnan Skin Score; ANA, antinuclear antibody; CENP-B, anti-centromere B antibody; PAH, pulmonary arterial hypertension; LVEF, left ventricle ejection fraction; SSA, SSB, Ro52, antibody anti Ro, La and Ro53 antigens respectively. 3.2. Cytometric Characterization of Patients and Controls We first performed a detailed, multiparameter immune cell profiling by flow cytometry to get a comprehensive overview about immune cell subset composition of SSc patients and compared cell profile to healthy controls. As presented in Physique 1 we identified several lines of immune cell including T, B, NK, NKT, and monocytes, which were further characterized as T helper (Th), T suppressor 1-Methylpyrrolidine (Ts), double positive T cells (Tdp CD4+CD8+), double unfavorable T cells (Tdn CD4?CD8?). To further characterize the changes in B cell subset composition, we first analyzed cell frequencies and absolute numbers of B cells, that were further characterized as B regulatory (Breg CD19+CD24++CD38++CD27?), B memory (CD19+CD24++CD38?CD27+), B mature (CD19+CD24lowCD38lowCD27+), and plasmablasts (CD19+CD24?CD38+?CD27++). NKT and.