Explainable fully automated CT scoring of interstitial lung disease for patients suspected of systemic sclerosis by cascaded regression neural networks and its comparison with experts.

Visual scoring of interstitial lung disease in systemic sclerosis (SSc-ILD) from CT scans is laborious, subjective and time-consuming. This study aims to develop a deep learning framework to automate SSc-ILD scoring. The automated framework is a cascade of two neural networks.

Step forward in early recognition of systemic sclerosis: data from the Leiden CCISS cohort.

Background: Since 2009, Dutch patients with a confirmed diagnosis/suspicion of systemic sclerosis (SSc) can be referred to the Leiden Combined Care in Systemic Sclerosis (CCISS) cohort. This study evaluated whether early recognition of SSc has improved over time and whether disease characteristics and survival has changed over time.

Methods: 643 SSc patients fulfilling American College of Rheumatology/European Alliance of Associations for Rheumatology 2013 SSc criteria were included and categorised into three groups based on cohort-entry year: (1) 2010-2013 (n=229 (36%)), (2) 2014-2017 (n=207 (32%)) and (3) 2018-2021 (n=207 (32%)).

Randomized feasibility trial of the Scleroderma Patient-centered Intervention Network hand exercise program (SPIN-HAND).

Purpose: The Scleroderma Patient-centered Intervention Network (SPIN) online hand exercise program (SPIN-HAND), is an online self-help program of hand exercises designed to improve hand function for people with scleroderma. The objective of this feasibility trial was to evaluate aspects of feasibility for conducting a full-scale randomized controlled trial of the SPIN-HAND program.

Materials And Methods: The feasibility trial was embedded in the SPIN cohort and utilized the cohort multiple randomized controlled trial (cmRCT) design.

The effect of silver fibre gloves on Raynaud's phenomenon in patients with systemic sclerosis: a double-blind randomized crossover trial.

Objectives: Silver fibre gloves transport heat from the palm to the fingers, possibly reducing the burden of RP in SSc patients. We aim to evaluate the clinical efficiency of this intervention.

Methods: A multicentre, double-blind, randomized trial was performed, accounting for interindividual differences and external factors using a crossover design.

Evidence and consensus-based recommendations for non-pharmacological treatment of fatigue, hand function loss, Raynaud's phenomenon and digital ulcers in patients with systemic sclerosis.

Objective: SSc is a complex CTD affecting mental and physical health. Fatigue, hand function loss, and RP are the most prevalent disease-specific symptoms of systemic sclerosis. This study aimed to develop consensus and evidence-based recommendations for non-pharmacological treatment of these symptoms.

Evolution of Systemic Sclerosis-Associated Interstitial Lung Disease One Year After Hematopoietic Stem Cell Transplantation or Cyclophosphamide.

Objective: Hematopoietic stem cell transplantation (HSCT) and cyclophosphamide (CYC) are treatment options for progressive systemic sclerosis associated with interstitial lung disease (SSc-ILD). The aims of our retrospective observational study were to evaluate: 1) the evolution of SSc-ILD in SSc patients treated with HSCT (assessed by high-resolution computed tomography [HRCT]; a group of patients treated with CYC was included as frame of reference); 2) how results of pulmonary function tests (PFTs) are associated with HRCT findings; and 3) which factors predict ILD reduction.

Methods: We semiquantitatively scored total ILD extent, reticulations, and ground-glass opacities (GGO) scores at baseline and at the 1-year HRCTs of SSc patients treated with HSCT or CYC.

Predictive factors for treatment-related mortality and major adverse events after autologous haematopoietic stem cell transplantation for systemic sclerosis: results of a long-term follow-up multicentre study.

Background: Autologous haematopoietic stem cell transplantation (HSCT) improves survival in systemic sclerosis (SSc) with poor prognosis, but is hampered by treatment-related mortality (TRM).

Objective: To evaluate event-free survival (EFS), TRM, response to treatment, disease progression and patient characteristics associated with events.

Methods: All patients treated with HSCT for SSc in The Netherlands until 2017 (n=92) were included.

Circulating calprotectin (S100A8/A9) is higher in rheumatoid arthritis patients that relapse within 12 months of tapering anti-rheumatic drugs.

Objective: To investigate whether calprotectin (S100A8/A9 or MRP8/14), an inflammatory complex released by monocytes, could indicate residual subclinical inflammation in rheumatoid arthritis (RA) patients who are in stable remission on disease-modifying anti-rheumatic drugs (DMARDs) and serve as a marker for disease flare after DMARD tapering.

Methods: We used data from two trials. Patients from the IMPROVED study had early (< 2 years) RA, and when they achieved disease activity score remission (DAS44 < 1.

How do patients with systemic sclerosis experience currently provided healthcare and how should we measure its quality?

Objectives: To gain insight into SSc patients' perspective on quality of care and to survey their preferred quality indicators.

Methods: An online questionnaire about healthcare setting, perceived quality of care (CQ index) and quality indicators, was sent to 2093 patients from 13 Dutch hospitals.

Results: Six hundred and fifty patients (mean age 59 years, 75% women, 32% limited cutaneous SSc, 20% diffuse cutaneous SSc) completed the questionnaire.

Pulmonary Vascular Morphology Associated With Gas Exchange in Systemic Sclerosis Without Lung Fibrosis.

Purpose: Gas exchange in systemic sclerosis (SSc) is known to be affected by fibrotic changes in the pulmonary parenchyma. However, SSc patients without detectable fibrosis can still have impaired gas transfer. We aim to investigate whether pulmonary vascular changes could partly explain a reduction in gas transfer of SSc patients without fibrosis.

The association of sociodemographic and disease variables with hand function: a Scleroderma Patient-centered Intervention Network cohort study.

Objectives: Impaired hand function in systemic sclerosis (SSc) is a primary cause of disability and contributes diminished health-related quality of life. The objective of the present study was to evaluate sociodemographic, lifestyle, and disease-related factors independently associated with hand function in SSc.

Methods: Patients enrolled in the Scleroderma Patient-centered Intervention Network Cohort who completed baseline study questionnaires between March 2014 and September 2017 were included.

Randomized feasibility trial of the Scleroderma Patient-centered Intervention Network hand exercise program (SPIN-HAND): Study protocol.

Background: Significant functional impairment of the hands is nearly universal in systemic sclerosis (SSc, scleroderma). Hand exercises may improve hand function, but developing, testing and disseminating rehabilitation interventions in SSc is challenging. The Scleroderma Patient-centered Intervention Network (SPIN) was established to address this issue and has developed an online hand exercise program to improve hand function for SSc patients (SPIN-HAND).

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.

Lung Density and Pulmonary Artery Diameter are Predictors of Pulmonary Hypertension in Systemic Sclerosis.

Purpose: The aim was to evaluate computed tomography (CT)-measured pulmonary artery diameter (PAD) and lung density as predictors of pulmonary hypertension (PH) in subjects with systemic sclerosis (SSc). We compared these PAD values with normal values and between SSc subgroups with PH and/or interstitial lung disease (ILD). We investigated whether PAD predicts PH and whether lung densitometry, by using the 85th percentile density value (Perc85) as a measure for ILD, can predict PH.

Exercise habits and factors associated with exercise in systemic sclerosis: a Scleroderma Patient-centered Intervention Network (SPIN) cohort study.

Objective: Exercise is associated with improved health in many medical conditions. Little is known about the exercise habits of people with systemic sclerosis (SSc, or scleroderma). This study assessed the proportion of individuals with SSc who exercise and associations of demographic and disease variables with exercise.

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.

Using Optimal Test Assembly Methods for Shortening Patient-Reported Outcome Measures: Development and Validation of the Cochin Hand Function Scale-6: A Scleroderma Patient-Centered Intervention Network Cohort Study.

Objective: To develop and validate a short form of the Cochin Hand Function Scale (CHFS), which measures hand disability, for use in systemic sclerosis, using objective criteria and reproducible techniques.

Methods: Responses on the 18-item CHFS were obtained from English-speaking patients enrolled in the Scleroderma Patient-Centered Intervention Network Cohort. CHFS unidimensionality was verified using confirmatory factor analysis, and an item response theory model was fit to CHFS items.

Validity and responsiveness of the Michigan Hand Questionnaire in patients with systemic sclerosis.

Objective: The aim was to assess the validity and responsiveness of the Michigan Hand Questionnaire (MHQ) in patients with SSc.

Methods: Data were gathered in connection with a randomized, controlled trial comparing the effectiveness of a 12-week multidisciplinary team care programme, including a hand function treatment module, with regular care. Hand function was evaluated by the MHQ (37 items, six domains: Function, Daily activities, Pain, Work, Aesthetics and Satisfaction) and other measurements, including the HAQ, Hand Mobility in Scleroderma, Sequential Occupational Dexterity Assessment (SODA), grip strength, pinch grip and modified Rodnan Skin Score.

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.

The educational needs of people with systemic sclerosis: a cross-sectional study using the Dutch version of the Educational Needs Assessment Tool (D-ENAT).

The Dutch Educational Needs Assessment Tool (D-ENAT) systematically assesses educational needs of patients with rheumatic diseases. The present study aims to describe the educational needs of Dutch patients with systemic sclerosis (SSc). The D-ENAT was sent to 155 SSc patients registered at the outpatient clinic of a university hospital.

Effectiveness of Nonpharmacologic Interventions in Systemic Sclerosis: A Systematic Review.

Objective: To systematically and comprehensively document the effectiveness of nonpharmacologic interventions on physical functioning and psychological well-being in patients with systemic sclerosis (SSc).

Methods: Multiple electronic databases were searched for studies on the effectiveness of nonpharmacologic interventions in SSc. Randomized clinical trials (RCTs), controlled clinical trials (CCTs), and observational designs (ODs) with ≥10 participants were included.