Rituximab in early systemic sclerosis.

Objectives: (1) Hypothesis testing of the potency of rituximab (RTX) in preventing fibrotic complications and (2) assessing acceptability and feasibility of RTX in early systemic sclerosis (SSc).

Methods: A small, 24-month, randomised, double-blind, placebo-controlled, single-centre trial in patients with SSc diagnosed <2 years was conducted. Patients received RTX or placebo infusions at t=0, t=15 days and t=6 months.

Predicting cardiopulmonary involvement in patients with systemic sclerosis: complementary value of nailfold videocapillaroscopy patterns and disease-specific autoantibodies.

Objective: To evaluate the prevalence of anti-extractable nuclear antigen (anti-ENA) antibodies in Dutch SSc patients and the predictive power of the combination of specific anti-ENA antibodies and nailfold videocapillaroscopy (NVC) patterns to improve identification of patients with high risk for cardiopulmonary involvement.

Methods: A total of 287 patients (79%) from the Leiden SSc-Cohort had data available on NVC-pattern (no SSc-specific, early, active, late) and anti-ENA antibodies. Associations between anti-ENA/NVC combinations with cardiopulmonary parameters were explored using logistic regression.

Therapeutic and diagnostic outcomes of a standardised, comprehensive care pathway for patients with systemic sclerosis.

Objectives: To determine the outcomes, including number of medical interventions and initiation of immunosuppressive treatment of a standardised, comprehensive, diagnostic care pathway for patients with systemic sclerosis (SSc). Patient characteristics associated with need for medical interventions and with need for immunosuppressive treatment were determined.

Methods: Data were routinely gathered in connection with a 2-day care pathway combining multidisciplinary care and complete diagnostic work-up of organ involvement in SSc.

A prediction model for progressive disease in systemic sclerosis.

Objective: To develop a model that assesses the risk for progressive disease in patients with systemic sclerosis (SSc) over the short term, in order to guide clinical management.

Methods: Baseline characteristics and 1 year follow-up results of 163 patients with SSc referred to a multidisciplinary healthcare programme were evaluated. Progressive disease was defined as: death, ≥10% decrease in forced vital capacity, ≥15% decrease in diffusing capacity for carbon monoxide, ≥10% decrease in body weight, ≥30% decrease in estimated-glomerular filtration rate, ≥30% increase in modified Rodnan Skin Score (with Δ≥5) or ≥0.

Impact of pulmonary fibrosis and elevated pulmonary pressures on right ventricular function in patients with systemic sclerosis.

Objectives: Right ventricular (RV) dysfunction is of great prognostic value in patients with SSc. The aim of the present study was to assess in these patients the relationship between pulmonary fibrosis and elevated pulmonary pressure (PHT) with RV function.

Methods: A total of 102 SSc patients who underwent thoracic CT and transthoracic echocardiography were included.

Blood flow in the hands of a predefined homogeneous systemic sclerosis population: the presence of digital ulcers and the improvement with bosentan.

Objectives: The aim of the study was to examine the effect of bosentan on blood flow in the hand in a subset of patients who had reduced blood flow relative to healthy subjects. Additionally, the relationship between blood flow in the hands of SSc patients and the presence of digital ulcers (DUs) was assessed.

Methods: SSc patients with a recent history of DUs and healthy subjects were included.

External validation of the revised Baux score for the prediction of mortality in patients with acute burn injury.

Background: Since the original Baux score was outdated and inhalation injury was recognized as an important contributor to mortality, Osler et al. developed a revised Baux score for the prediction of mortality of burn patients in an American population.The aim of this study was to validate the revised Baux score with data of patients admitted to the Rotterdam Burn Center (RBC) in the Netherlands.

Health-care utilization in Dutch systemic sclerosis patients.

This study aims to examine healthcare utilization and its determinants among patients with systemic sclerosis (SSc). A cross-sectional survey among all patients with SSc visiting an outpatient clinic of an academic hospital in the Netherlands was done. Assessments included sociodemographic characteristics and a survey on healthcare utilization including a registration of contacts with healthcare services since onset of disease, contacts, and number of visits with healthcare services over the last 12 months.