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Background and Aims

To compare clinical parameters in systemic sclerosis(SSc) patients(pts) depending on the decrease of a-Topo-1 during rituximab(RTX) therapy.

Methods

This study included 63pts with SSc. The mean follow-up period-26,3±10,7months. The mean age-47years, female-53pts(84%), diffuse cutaneous subset of the disease had 39pts(62%). Interstitial lung disease(ILD) were observed in 60pts(95%). The mean disease duration was 6,03±4,9years. The cumulative mean dose of RTX=2,9±1,1grams. All pts received prednisone at a dose=11,1±4,4mg, immunosuppressants received 43%. All pts were positive for a-Topo-1. Pts were divided into two groups depending on the changes of a-Topo-1 on RTX therapy:group 1 (n=32)–pts with decrease in a-Topo-1 and group 2 (n=31)–pts with stable a-Topo-1. The results are presented in the form of mean values and changes in parameters(Δ).

Results

Considering the entire cohort, an improvement of almost all outcome parameters was found. In group 1 there was ΔActivity score(EScSG-AI)=1,93;ΔRodnan skin score(mRSS)=5,33;Δforced vital capacity %predicted(FVC)=11,9;Δdiffusion capacity for carbon monoxide %predicted(DLCO)=3,2. In group 2: ΔEScSG-AI=1,84;ΔmRSS=4,68;ΔFVC=5,75;ΔDLCO=2,73. When groups were analyzed separately, we observed a significantly higher increase of FVC(p=0,03) and DLCO(p=0,02) in group 1. The a-Тopo-1 level decreased from 174,2±50,1 to 148,1±66,1units/ml(p=0,0009). There was a moderate negative statistically significant correlation between the a-Topo-1 and the total dose of RTX(r=-0,298;p=0,017). A moderate negative statistically significant correlation was found between the a-Topo-1 and FVC(r=-0,322;p=0,009).

Conclusions

In our study there was a greater increase in FVC and DLCO in the group of pts with a decrease of a-Topo-1. Decrease of a-Topo-1 could be considered as a predictor of a better response to RTX therapy in pts with SSc-ILD.

Background and Aims

To compare the impact of CyP and RTM on SSc clinical manifestation,activity,and the safety of these agents in the open-label prospective non-randomized study.

Methods

107 patients with the confirmed SSc diagnosis and ILD were enrolled into the study. 36 patients(Group A) received CyP for 12±6 months at total dose 10.6±5g. 71 patients(Group B) received RTM at total dose 1.43±0.66g over the follow-up period 13.2±2 months. The time courses FVC(%), modified skin count(mRss), activity index(EScSG), left ventricle ejection fraction (LVEF,%), mean pulmonary arterial pressure, and cardiac rhythm and conductivity disorders were evaluated.

Results

In Groups A and B the therapy was associated with significant decrease in mRss (р=0.009 and 0,001) and EScSG (р=0.000165 and 0.001). Increase in LVEF was observed only in RTM-treated patients.

Evaluation of FVC time course in Groups A and B revealed significant FVC increase(р= 0.034 and 0.000045, with median increment about 5%.

In Group A 10% FVC increase was found in the third of the patients thus exceeding respective parameter in Group B(р=0.2).

During the follow-up period no change of the other studied parameters was observed.

The therapy was better tolerated in RTM-treated group.

Conclusions

Both agents effectively alleviated mRss, EScSG, and significantly improved FVC. CyP use slightly more frequently resulted in clinically significant FVC increase,probably due to low RTM cumulative dose. RTM was better tolerated. The study findings substantiate potential use of RTM both as a first-line agent for ILD treatment in SSc, and in the event of CyP inefficacy of poor tolerability, especially in the patients with cardiopathy.

Background and Aims

Pulmonary arterial hypertension in systemic sclerosis (PAH-SSc) in comparison with idiopathic PAH (IPAH) is characterized by rapid progression, higher mortality and poor response to PAH-specific therapy.

Methods

51 patients with PAH-SSc and 50 patients with IPAH. Univariate logisitic regression was used to calculate the probability (odds ratio (OR)) of SSc symptoms.

Results

We identified 29 symptoms that were associated with PAH-SSc. The most significant of them, increasing the risk of detection of SSc in patients with PAH, were age>45 years (OR 9.7, 95% CI 3.9-24.3, p<0.001), diffusion lung capacity (DLCO)<60% (OR 13.8, 95% CI 4.3-44.7, p<0.0001), ratio of forced vital capacity to DLCO<1.7 (OR 13.0, 95% CI 3.9-42.9, p <0.00001), C-reactive protein>2 mg/l (OR 12.9, 95% CI 3.96-41.8, p<0.001), uric acid level>387 μmol/L (OR 3.8, 95% CI 1.5-9.6, p=0.003), pericardial effusion (OR 2.3, 95% CI 1.0-5.1, p=0.04), mean right ventricular pressure>15.5 mmHg (OR 8.9, 95% CI 3.4-23.1, p<0.001), diastolic right ventricular pressure>4.5 mmHg (OR 4.6, 95% CI 1.9-11.2, p=0.003). Symptoms that reduce the risk of detecting SSc include hemoglobin level >146 g/l (OR 0.3, 95% CI 0.2-0.8, p=0.007), syncope (OR 0.3, 95% CI 0.1-0.9, p=0.02), mean pulmonary artery pressure>55 mmHg (OR 0.4, 95% CI 0.2-0.9, p=0.03), pulmonary vascular resistance>12 units Wood (OR 0.4, 95% CI 0.2-0.9, p=0.02).

Conclusions

The identification of specific signs of PAH will allow early diagnosis of SSc, predict the course of PAH, and more carefully approach the choice of PAH therapy. What will extend the life of patients with PAH-SSc.