Faecal levels of calprotectin in systemic sclerosis are stable over time and are higher compared to primary Sjögren's syndrome and rheumatoid arthritis.

Introduction: Faecal calprotectin (FC) has been proposed to be a biomarker of gastrointestinal (GI) disease in systemic sclerosis (SSc). The purpose of this study was to extend cross-sectional observations and prospectively assess the variability of FC over time in SSc patients. We also aimed to examine FC in relation to immunosuppressive therapy.

Biomarkers from bronchoalveolar lavage fluid in systemic sclerosis patients with interstitial lung disease relate to severity of lung fibrosis.

Objectives: Decision on treatment of systemic sclerosis (SSc) related interstitial lung disease (ILD) largely relies on the findings on high resolution computed tomography (HRCT) and there is a need for improvement in assessment of the fibrotic activity. The objectives of this study were to study biomarkers in bronchoalveolar lavage fluid (BALF) from SSc patients with ILD and to relate the findings to the severity and activity of lung fibrosis.

Methods: Fifteen patients with early SSc and 12 healthy controls were subjected to BAL.

Assessment of capillary density in systemic sclerosis with three different capillaroscopic methods.

Objectives: Capillary abnormalities, such as the enlargement and/or disappearance of capillary loops, occur early in the majority of patients with systemic sclerosis (SSc). The aim of this study was to compare three capillaroscopic methods of determining the capillary density in patients with SSc.

Methods: Two of the three methods involved stereo-zoom microscopy at a magnification of 20 times, used either for direct counting, or with a camera and imaging software for determination of the capillary density on coded images.

Increased serum COMP predicts mortality in SSc: results from a longitudinal study of interstitial lung disease.

Objectives: COMP is a regulator of assembly and maintenance of the fibrillar collagen I and II networks. Serum COMP reflects skin fibrosis in SSc. The purpose of this study was to examine whether serum COMP reflects fibrotic lung involvement in SSc patients and to study if serum COMP predicts mortality.

A multicentre study on the reliability of qualitative and quantitative nail-fold videocapillaroscopy assessment.

Objective: To investigate the inter- and intra-observer reliability of both qualitative and quantitative parameters used in the assessment of nail-fold capillaroscopy images.

Methods: Fifty mosaic nail-fold images of healthy controls (n = 10), patients with primary RP (n = 10) and SSc (n = 30) were assessed in random order by two blinded observers on two occasions at centres in Sweden, UK and The Netherlands. Each image was therefore scored by six observers twice.

Managing work life with systemic sclerosis.

Objective: To explore how individuals with SSc manage their work life.

Methods: We conducted four focus group interviews, which included 17 patients currently working at least 20 h per week. The interviews were recorded and transcribed verbatim.

Interleukin-15 attenuates transforming growth factor-β1-induced myofibroblast differentiation in human fetal lung fibroblasts.

Objective: Fibroproliferative diseases are common causes of morbidity and mortality. Interleukin-15 (IL-15) is a pleiotropic cytokine with multiple effects on cells of the immune system. Although IL-15 is also expressed in mesenchymal cells, its effects on the development of fibrosis are unknown.

Increased cysteinyl-leukotrienes and 8-isoprostane in exhaled breath condensate from systemic sclerosis patients.

Objectives: SSc is a systemic CTD characterized by fibrosis in skin and internal organs. Interstitial lung disease is a frequent complication with fibrosis in the lung parenchyma. The fibrotic process is believed to be influenced by leukotrienes (LTs) and also by oxidative stress.

Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.

Objectives: To determine the causes and predictors of mortality in systemic sclerosis (SSc).

Methods: Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities.

Pain, fatigue and hand function closely correlated to work ability and employment status in systemic sclerosis.

Objective: To identify factors, individual and work related, influencing work ability, and to assess the association between work ability and employment status, activities of daily life (ADLs) and quality of life in patients with SSc.

Methods: Fifty-seven consecutive patients (53 females/4 males) with SSc (47 lcSSc/10 dcSSc) were included. Median age was 58 [interquartile range (IQR) 47-62] years and disease duration 14 (9-19) years.

Presence of CD4+CD8+ double-positive T cells with very high interleukin-4 production potential in lesional skin of patients with systemic sclerosis.

Objective: Fibrotic skin changes in systemic sclerosis (SSc) are preceded by the appearance of an inflammatory infiltrate rich in T cells. Since no direct comparison with T cells in normal skin has been performed previously, this study was undertaken to functionally characterize T cells in the skin of patients with early active SSc and in normal skin.

Methods: We characterized coreceptor expression, T cell receptor (TCR) usage, cytokine production, and helper and cytolytic activity of T cell lines and clones established from skin biopsy specimens from 6 SSc patients and 4 healthy individuals.

Serum IL-15 in patients with early systemic sclerosis: a potential novel marker of lung disease.

The pathogenesis of systemic sclerosis (SSc) is characterized by autoimmunity, vasculopathy and fibrosis. IL-15 is a pleiotropic cytokine that has impact on immune, vascular and connective tissue cells. We therefore investigated IL-15 in the circulation of patients with early SSc and explored possible associations of serum IL-15 with vasculopathy and fibrosis.

Functional and phenotypical comparison of myofibroblasts derived from biopsies and bronchoalveolar lavage in mild asthma and scleroderma.

Background: Activated fibroblasts, which have previously been obtained from bronchoalveolar lavage fluid (BALF), are proposed to be important cells in the fibrotic processes of asthma and scleroderma (SSc). We have studied the motility for BALF derived fibroblasts in patients with SSc that may explain the presence of these cells in the airway lumen. Furthermore, we have compared phenotypic alterations in activated fibroblasts from BALF and bronchial biopsies from patients with mild asthma and SSc that may account for the distinct fibrotic responses.

Polarized subsets of human T-helper cells induce distinct patterns of chemokine production by normal and systemic sclerosis dermal fibroblasts.

The role of fibroblasts in inflammatory processes and their cross-talk with T cells is increasingly being recognized. Our aim was to explore the capacity of dermal fibroblasts to produce inflammatory chemokines potentially involved in fibrosis occurring in response to contact with polarized human T cells. Our findings indicate that the program of chemokine production by fibroblasts is differentially regulated depending on the T-helper (Th) cell subset used to activate them.

Small intestinal manometry in patients with systemic sclerosis.

Objectives: The study explores, by the use of manometry, the frequency and severity of small intestinal involvement in patients with systemic sclerosis, and relates the manometric findings to clinical symptoms, radiology, and some intestinal regulatory peptides.

Methods: Stationary antroduodeno-jejunal manometry was used to study small bowel involvement in 10 patients with systemic sclerosis and dysmotility of the oesophagus or signs of malabsorption. Measurements were made during fasting, after a meal, and after octreotide administration and were then compared with a sex-matched control group of healthy individuals.

Systemic sclerosis Th2 cells inhibit collagen production by dermal fibroblasts via membrane-associated tumor necrosis factor alpha.

Objective: In systemic sclerosis (SSc; scleroderma), T cells infiltrate organs undergoing fibrotic changes and may participate in dysregulated production of collagen by fibroblasts. The objective of this study was to functionally characterize T cells infiltrating skin lesions in early SSc and investigate their capacity to affect production of type I collagen and interstitial collagenase (matrix metalloproteinase 1 [MMP-1]) by dermal fibroblasts.

Methods: Four-color cytometric analysis was used to characterize subset distribution and production of interferon-gamma (IFN gamma) and interleukin-4 (IL-4) in T cell lines generated from the skin of patients with SSc.