The Chinese herb Tripterygium wilfordii Hook F for the treatment of systemic sclerosis-associated interstitial lung disease: data from a Chinese EUSTAR Center

AbstractObjectiveTo assess the efficacy and safety of the Chinese herbTripterygium wilfordii Hook F (TwHF) for the treatment of systemic sclerosis-associated interstitial lung disease (SSc-ILD).MethodsSSc-ILD patients who were regularly treated for more than 1  year and were currently taking a stable dose of TwHF (40–60 mg/day) or CYC (100 mg/day) were selected from the EUSTAR database of Peking Union Medical College Hospital. The efficacy of treatments was assessed by the change in pulmonary function, including the forced vital capacity (FVC) and th e percentage of predicted FVC (FVC pred%).ResultsAmong the 431 patients diagnosed with SSc-ILD, 76 fulfilled the inclusion and exclusion criteria. Twenty eight patients received TwHF monotherapy, while 48 received oral CYC monotherapy. Baseline data prior to treatment did not differ significantly between the two groups. After 1  year of treatment, significant improvements in the FVC and FVC pred% were seen in both groups (P 
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research

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This study was registered at ClinicalTrials.gov (NCT02896205).
Source: Rheumatology International - Category: Rheumatology Source Type: research
ObjectiveTo investigate the relationship between Krebs von den Lungen 6 (KL ‐6) and CCL18 levels and the severity and progression of systemic sclerosis (SSc)–related interstitial lung disease (ILD).MethodsPatients enrolled in the Scleroderma Lung Study II (cyclophosphamide [CYC] versus mycophenolate mofetil [MMF]) were included. Baseline and 12 ‐month plasma samples were analyzed by enzyme‐linked immunosorbent assay to assess CCL18 and KL‐6 levels. The forced vital capacity (FVC) and the diffusing capacity for carbon monoxide (DLco) were measured every 3 months. Joint models were created to investigate the re...
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: Original Article Source Type: research
Background: Systemic sclerosis (SSc) is a potentially serious and disabling connective tissue disease with a poor prognosis. Pulmonary interstitial lung disease associated with SSc (SSc-ILD) is among the leading causes of death. The aim of our study was to evaluate the potential utility of dosing proteins known to be useful biomarkers in ILD in the induced sputum (IS) of SSc patients.Methods: In a prospective cross-sectional analysis, we studied the IS of 28 SSc patients whom 11 with SSc-ILD compared to 25 healthy subjects (HS). We analysed sputum cell composition and several biomarkers in the supernactant assumed to be cr...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research
Objective: To assess the role of BAL fluid IL-6 and IL-7 levels in disease activity assessment in Systemic sclerosis-associated interstitial lung disease (SSc-ILD)Methods: Twenty cases of SSc-ILD were enrolled after excluding overlap syndrome, smokers, lung infection, and pulmonary hypertension. They were evaluated with six-minute walk test, spirometry, HRCT-chest with semi-quantitative scoring and bronchoalveolar lavage. Re-evaluation was done at 6 months after six cyclophosphamide pulse (CYP) therapy. BAL fluid IL-6 and IL-7 levels were estimated using ELISA based kits. Twenty patients of carcinoma esophagus undergoing p...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research
Conclusion: The primary mRSS endpoint was not met. A clinically relevant difference in FVC was seen for TCZ vs PBO, with preservation of lung function.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research
Background: Systemic sclerosis (SSc) is a rare connective tissue disease associated with potential rapid evolving interstitial lung disease (SSc-ILD), driving the mortality of these patients. Therefore, a specific biomarker associated with the evolution of that disease is highly needed to identify patients with an increased risk of death.Aim of the Study: Identify specific biomarkers of SSc-ILD to predict the evolution of the disease.Methods: In this prospective longitudinal study, we compared serum levels of biomarkers assumed to be associated with lung fibrosis (TGF-β, IGF-1, IGFBP-1, IGFBP-2, IGFBP-3, IL-8, MMP-7, ...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research
Background: Some patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) develop progressive ILD, but disease course and outcome are unknown.Objectives: To assess frequency, disease course and mortality of progressive ILD in the European Scleroderma Trials and Research group (EUSTAR) database.Methods: Adult patients fulfilling SSc classification criteria, registered since 2010, with ILD on high- resolution computed tomography, serial lung function and disease duration recordings, were eligible. Progressive ILD was defined as: significant (forced vital capacity [FVC] decline>10% or 5-10% with diff...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research
Conclusion: Compared to the conventional approaches, our new method offers more advantages including ability of multivariate interpretation and valuable information for personalized treatment plan.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research
Conclusions: Combination of HRCT and a complete PFT at baseline can help predict functional decline. HRCT may be preferred as first line screening tool whereas TLC can outperform the risk event prediction.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research
Conclusions: K-BILD scores appeared to be similar between patients with IPF and SSc-ILD after adjusting for age, gender and lung function. This new observation underscores the impact of lung function impairment on HRQL in ILD and the need for treatments that positively impact HRQL by preserving lung function.IPF (n=58)SSc-ILD (n=29)Age (years)71 (9)59 (12)Female n (%)16 (28)23 (79)FVC (% pred.)73 (18)87 (29)DLCO (% pred)45 (14)57 (21)K-BILD total score69.4 (17.9)80.5 (21.2)
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research
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