The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index

Background Validity of European Scleroderma Study Group (EScSG) activity indexes currently used to assess disease activity in systemic sclerosis (SSc) has been criticised. Methods Three investigators assigned an activity score on a 0–10 scale for 97 clinical charts. The median score served as gold standard. Two other investigators labelled the disease as inactive/moderately active or active/very active. Univariate–multivariate linear regression analyses were used to define variables predicting the ‘gold standard’, their weight and derive an activity index. The cut-off point of the index best separating active/very active from inactive/moderately active disease was identified by a receiver-operating curve analysis. The index was validated on a second set of 60 charts assessed by three different investigators on a 0–10 scale and defined as inactive/moderately active or active/very active by other two investigators. One hundred and twenty-three were investigated for changes over time in the index and their relationships with those in the summed Medsger severity score (MSS). Results A weighted 10-point activity index was identified and validated: Δ-skin=1.5 (Δ=patient assessed worsening during the previous month), modified Rodnan skin score (mRss) \u3e18=1.5, digital ulcers=1.5, tendon friction rubs=2.25, C-reactive protein \u3e1 mg/dL=2.25 and diffusing capacity of the lung for CO (DLCO) % predicted \u3c70%=1.0. A cut-off ≥2.5 was found to identify patients with active disease. Changes in the index paralleled those of MSS (p=0.0001). Conclusions A preliminarily revised SSc activity index has been developed and validated, providing a valuable tool for clinical practice and observational studies

Low-dose pulse cyclophosphamide in interstitial lung disease associated with systemic sclerosis (SSc-ILD): efficacy of maintenance immunosuppression in responders and non-responders

To investigate the long-term disease course of patients with recently deteriorated systemic sclerosis (SSC)-interstitial lung disease (ILD) undergoing continuous immunosuppressive treatment with cyclophosphamide (CYC) as induction therapy

Lung involvement in "stable" undifferentiated connective tissue diseases: a rheumatology perspective

Previous studies of the occurrence of interstitial lung disease (ILD) in undifferentiated connective tissue diseases (UCTD) were conducted in patients admitted to Respiratory Medicine Units. The aim of the present prospective study was to investigate lung involvement in UCTD patients admitted to a Rheumatology Unit. Eighty-one consecutive UCTD patients were enrolled in the study. Each patient underwent history and physical examination, routine laboratory investigations, antinuclear antibody (ANA) profiling, B-mode echocardiography, and lung function study according to previously reported methods. Lung high resolution computed tomography (HRCT) was performed in patients who provided informed consent. Six patients (7.4%) had a history of grade II dyspnea. Three of them had a DLCO ranging from 42 to 55% of the predicted value; and a HRCT-documented ILD with a non-specific interstitial pneumonia (NSIP) pattern. Symptoms in the other three patients were due to cardiac disease. None of the 75 asymptomatic patients, had relevant findings at physical examination, 26/75 had a DLCO <80% (<70% in 10 cases). Of these, 3 of the 30 patients who underwent lung HRCT were affected by NSIP-ILD. Six of the 81 enrolled were affected by ILD, which was symptomatic in three patients. A higher percentage of patients had a reduced DLCO. The latter finding may reflect a preradiographic ILD or a preechocardiographic pulmonary vascular disease

CXCL4 in undifferentiated connective tissue disease at risk for systemic sclerosis (SSc) (previously referred to as very early SSc)

The aim of the study was to evaluate CXCL4 levels in undifferentiated connective tissue disease at risk for SSc (UCTD-SSc-risk) and confirm its increase and investigate its prognostic value. Serum CXCL4 levels were measured in 45 patients and 24 controls. CXCL4 was significantly higher in UCTD-SSc-risk patients than in controls. It resulted higher in patients with a shorter disease duration and in those lacking capillaroscopic alterations. We confirm that CXCL4 levels are increased in UCTD-risk-SSc patients. Further studies are needed to investigate the role of CXCL4 assessment in UCTD-risk-SSc

Management of giant-cell arteritis in Switzerland: an online national survey.

AIMS OF THE STUDY To assess current practices in diagnosing, treating, and following-up giant-cell arteritis by specialists in Switzerland and to identify the main barriers to using diagnostic tools. METHODS We performed a national survey of specialists potentially caring for patients with giant-cell arteritis. The survey was sent by email to all members of the Swiss Societies of Rheumatology and for Allergy and Immunology. A reminder was sent to nonresponders after 4 and 12 weeks. Its questions covered the following dimensions: respondents' main characteristics, diagnosis, treatment, and imaging's role during follow-up. The main study results were summarized using descriptive statistics. RESULTS Ninety-one specialists, primarily aged 46-65 years (n = 53/89; 59%), working in academic or nonacademic hospitals or private practice, and treating a median of 7.5 (interquartile range [IQR]: 3-12) patients with giant-cell arteritis per year participated in this survey. Ultrasound of temporal arteries/large vessels (n = 75/90; 83%) and positron-emission-tomography-computed tomography (n = 52/91; 57%) or magnetic resonance imaging (n = 46/90; 51%) of the aorta/extracranial arteries were the most common techniques used to diagnose giant-cell arteritis with cranial or large vessel involvement, respectively. Most participants reported a short time to obtain imaging tests or arterial biopsy. The glucocorticoid tapering scheme, glucocorticoid-sparing agent, and glucocorticoid-sparing treatment duration varied among the participants. Most physicians did not follow a predefined repeat imaging scheme for follow-up and mainly relied on structural changes (vascular thickening, stenosis, or dilatation) to drive treatment choice. CONCLUSIONS This survey indicates that imaging and temporal biopsy are rapidly accessible for diagnosing giant-cell arteritis in Switzerland but highlights heterogeneous practice in many disease management areas

Low-dose oral imatinib in the treatment of systemic sclerosis interstitial lung disease unresponsive to cyclophosphamide: a phase II pilot study

Introduction: Pulmonary involvement represents a major cause of death of systemic sclerosis (SSc) patients. Recent data suggest that tyrosine kinase inhibitors, such as imatinib, may be a therapeutic option for SSc patients. However, preliminary published clinical trials were inconclusive about imatinib efficacy and showed side effects. The purpose of this study was to verify efficacy and tolerability of low-dose imatinib on interstitial lung disease in a cohort of SSc patients unresponsive to cyclophosphamide therapy.Methods: Thirty consecutive SSc patients with active pulmonary involvement, unresponsive to cyclophosphamide, were treated with imatinib 200 mg/day for 6 months followed by a 6-month follow-up. A "good response" was defined as an increase of forced vital capacity (FVC) by more of 15% and/or increase of diffusing capacity of carbon monoxide (DLCO) &gt; 15% and PaO2 &gt; 90% of initial value and high-resolution computed tomography (HRCT)-scan pattern unchanged or improved.Results: Twenty-six patients completed the study. Three patients died and one patient was lost to follow-up. Four patients (15.32%) had a good response, 7 worsened and 15 had a stabilized lung disease. Overall, 19 (73.07%) patients had an improved or stabilized lung disease. After a 6-month follow-up, 12 (54.5%) of the 22 patients showed an improved or stabilized lung disease.Conclusions: Lung function was stabilized in a large proportion of patients unresponsive to cyclophosphamide therapy and a beneficial outcome emerged from the analysis of HRCT lung scans. There was no significant improvement of skin involvement, and the low dose was well tolerated. These data provide useful suggestions to design future randomized clinical trials for SSc therapeutics

Correspondence on ‘ Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension’

International audienceNo abstract availabl

Glucocorticoids prescribing practices in systemic sclerosis: an analysis of the EUSTAR database

OBJECTIVES To estimate the long-term exposure to glucocorticoids (GC), the factors associated with, and the variations in prescribing practices over time and across recruiting countries. METHODS We included patients with systemic sclerosis (SSc) having a visit in the EUSTAR database from January 2013 onward. We analyzed the prevalence and the main features of GC users, the exposure to GC over time, and the respective dosages. Multivariable linear regression analysis identified factors associated with GC intake duration. Time trends, and variations in GC utilization across recruiting countries were explored. Missing data were imputed using multiple imputation with chained equations. RESULTS The 9819 patients included were mostly females (85%), lcSSc (73%), median age 58 years. At baseline, 34% of patients (n = 2769/8109) (48% dcSSc vs. 29% lcSSc) were on GC, median dose 7.5 mg/day. GC users were more frequently males, dcSSc, anti-Scl70 positive, with a more severe disease. On average, GC users spent 25% of their follow-up time (median 33.2 months) on GC, with no significant between-subsets difference. Notably, 33% (n = 971/2959) and 22% (n = 647/2959) of patients followed-up for >1 year, had received GC for >6 and >12 months, respectively. In multivariable analysis, patient and disease's characteristics poorly explained the variability of GC exposure (adjusted-R2 = 0.06, P < 0.001). GC utilization varied within and across countries, and gradually decreased over time (36% in 2013 vs. 23% in 2018). CONCLUSIONS GC are widely and long-term prescribed in SSc, with significant between- and within-country(ies) differences. A gradual decrease in their utilization is observed

Early Systemic Sclerosis: Marker Autoantibody Positive Patients Have A Faster Pace Of The Disease.

Early Systemic Sclerosis: Marker Autoantibody Positive Patients Have A Faster Pace Of The Disease. Background/Purpose: To investigate whether patients affected with any of the 3 subsets of early systemic sclerosis (SSc) i.e. Raynaud’s Phenomenon (RP) with SSc marker autoantibody (ACA or anti-Scl70 or anti-RNA polymerase III or anti-fibrillarin or anti-Th/To) and typical capillaroscopic findings (megacapillaries and/or avascular areas) (subset I); or autoantibody positive only (subset II); or capillaroscopy positive only (subset III) (1) and unsatisfying the 2012 ACR/EULAR classification criteria for SSc (2) at admission differ in the lag time to satisfy the new SSc classification criteria. Methods: Early SSc patients consecutively admitted to a Rheumatology and an Angiology center and unsatisfying the 2012 ACR/EULAR classification criteria for SSc at admission, were subdivided into the 3 above referred subsets and followed-up for 7–101 months (median 45). S288 Sunday, October 27 They were re-evaluated six-monthly by history, clinical examination, B-mode echoDopplercardiography and Lung functional study including DLCO evaluation and yearly by lung HRCT to assess whether and when each of them satisfied new ACR/EULAR classification criteria i.e. developed a disease score 9 (2). Results: During the follow-up, 11 out of 21 subset I patients (52.3%) (baseline score 8); 10 out of 15 subset II patients (66.6%) (baseline score 6) and 0 out of 24 subset III patients (baseline score 5–7) satisfied the criteria; the difference being significant between each of the 2 autoantibody positive (subsets I and II) and the capillaroscopic positive-autoantibody negative subset (subset I versus III: X2 by log rank test17.45, p0.0001; subset II versus III: X2 11.04, p0.0009), no difference being detected between the 2 autoantibody positive subsets (X2 0.55, p0.454). The 11 subset I patients satisfied the criteria because of the development of teleangectasias in 5 cases; puffy fingers in 3 cases; lung fibrosis in 2 cases; digital ulcers in 1 case. The 10 subset II patients did it because of the development of at least 2 of the following manifestations: scleroderma capillaroscopic pattern in 5 cases, teleangectasias in 5 cases, puffy fingers in 5 cases, digital ulcers in 3 cases, pulmonary hypertension in 1 case and lung fibrosis in 1 case. Despite the unfulfillment of the criteria, among the 24 subset III patients, 21 of whom already presented puffy fingers at baseline, 2 developed telangectasias, 1 digital ulcers, 4 a DLCO 80%. Conclusion: We have recently pointed out that autoantibody positive early SSc patients differ from subset III patients in the pattern of activation markers (increased serum concentration of procollagen I carbossipropeptide versus increased serum concentration of E-selectin) and preclinical internal organ involvement (higher prevalence of decreased DLCO) (3). Here we point out that autoantibody positive patients present a faster pace of the disease. References: 1) Koenig M et al. Arthritis Rheum. 2008;58:3902–12 2) Van den Hoogen F et al. Eular Congress 2013, OP0033 3) Valentini G et al. Arthritis Res Ther. 2013; 29;15:R63 Disclos