Chemokine (C-X-C motif) ligand (CXCL)10 in autoimmune diseases.

https://www.researchgate.net/file.PostFileLoader.html?id=54195058d11b8be7498b460f&assetKey=AS%3A273605672275984%401442244083811

Complex regional pain syndrome: A comprehensive and critical review

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum.

https://www.onlinelibrary.wiley.com/doi/epdf/10.1111/jdv.14458

European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes.

Objective To assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice. Methods We performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab. Results 254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47–5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55–1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56–3.53], p Conclusion Rituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial.

https://ard.bmj.com/content/annrheumdis/early/2019/04/26/annrheumdis-2018-214816.full.pdf

Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

Prospective study to evaluate the association between systemic sclerosis and occupational exposure and review of the literature.

Systemic sclerosis at the crossroad of polyautoimmunity

Summary Background Preliminary criteria for the very early diagnosis of systemic sclerosis (VEDOSS) have been previously proposed to identify signs and symptoms in patients with Raynaud's phenomenon. Patients with all signs or symptoms of the VEDOSS criteria already fulfil the 2013 American College of Rheumatology–European League Against Rheumatism (ACR–EULAR) classification criteria for systemic sclerosis. However, prospective data for the evolution to fulfilling these criteria do not exist. We therefore aimed to determine the clinical value of the VEDOSS criteria to identify patients with Raynaud's phenomenon who progress to systemic sclerosis within 5 years. Methods The VEDOSS project was a multicentre, longitudinal registry study done in 42 European Scleroderma Trial and Research group centres located in 20 countries in Europe, North America, and South America. Patients with Raynaud's phenomenon were eligible for enrolment. Those who had fulfilled the 1980 ACR or the 2013 ACR–EULAR classification criteria for systemic sclerosis, as well as of any other ACR or EULAR classification criteria for other definite connective tissue diseases at enrolment were excluded. Data were recorded each year during follow-up visits and included the four VEDOSS criteria (ie, positivity for antinuclear antibodies [ANAs], puffy fingers, systemic sclerosis-specific autoantibodies, and abnormal nailfold capillaroscopy). The primary endpoint was the fulfilment of the 2013 ACR–EULAR classification criteria for systemic sclerosis (ie, progression from enrolment to follow-up). Proportion of progressors and VEDOSS criteria interaction were reported descriptively. Predictors of progression of the distinct VEDOSS criteria interactions were determined based on the point prevalence at 5 years. To investigate the intermediate course of progression of the distinct VEDOSS criteria and their combinations, Kaplan-Meier analysis was done. Results Between March 1, 2010, and Oct 4, 2018, we enrolled 1150 patients with Raynaud's phenomenon in the VEDOSS database. 764 (66·4%) of 1150 patients met the VEDOSS criteria for study inclusion. Of the 764 patients, 553 (72·4%) had at least one available follow-up visit and the median duration of follow-up was 3·6 years (IQR 1·7–5·8). The mean age was 45·9 years (SD 15·0), 507 (91·7%) of 553 participants were female, and the median time since the onset of Raynaud's phenomenon was 4·0 years (IQR 1·7–10·0). At baseline, 401 (73·7%) of 544 patients with Raynaud's phenomenon had detectable ANA, with 208 (39·5%) of 527 patients positive for systemic sclerosis-specific autoantibodies. Nailfold capillaroscopy abnormalities were present in 182 (36·0%) of 505 patients and puffy fingers were detected in 96 (17·8%) of 540 at baseline. 1885 follow-up visits were recorded. 254 (45·9%) of 553 patients completed the study with progression or a 5-year follow-up; of whom, 133 reached the primary endpoint, resulting in an overall progression rate of 52·4%. The absence of ANA at baseline was the factor most strongly associated with a lack of progression within 5 years, with only four (10·8%) of 37 ANA-negative patients progressing. Conversely, positivity at baseline for systemic sclerosis-specific autoantibodies and puffy fingers was the combination having the highest risk of progression (16 [94·1%] of 17). Interpretation Our results from the VEDOSS project offers a useful tool for a stratified risk approach to patients with Raynaud's phenomenon. The absence of ANA is a strong protective factor that identifies patients with very low risk of developing systemic sclerosis whereas the presence of one or two VEDOSS criteria in patients with Raynaud's phenomenon confers a progressively higher risk for systemic sclerosis over time. This stratification tool can be used both for clinical management and to inform early interventional trials. Funding European Scleroderma Trial And Research and World Scleroderma Foundation.

Progression of patients with Raynaud's phenomenon to systemic sclerosis: a five-year analysis of the European Scleroderma Trial and Research group multicentre, longitudinal registry study for Very Early Diagnosis of Systemic Sclerosis (VEDOSS)

La cirrhose biliaire primitive (CBP) est une maladie auto-immune caracterisee par une destruction chronique des canalicules biliaires, touchant principalement les femmes âgees entre 35 et 45 ans. Cette inflammation hepatique prolongee peut etre a l’origine d’une cicatrice fibreuse pouvant favoriser la survenue d’une cirrhose. Les manifestations cliniques frequentes sont le prurit, l’asthenie ou un ictere mais la majorite des patients sont asymptomatiques. Bien que la CBP puisse etre responsable d’arthralgies par elle-meme, la survenue d’une polyarthrite ou de synovites est exceptionnelle. Neanmoins, cette hepatopathie est souvent associee a d’autres maladies auto-immunes comme le syndrome de Gougerot-Sjogren pouvant etre a l’origine de manifestations articulaires. L’association entre polyarthrite rhumatoide (PR) et CBP, peu decrite dans la litterature, est estimee entre 1,8 a 5,6 % des patients atteints de CBP. Nous rapportons deux cas de patientes avec PR severe associee a une CBP et discutons leur prise en charge therapeutique specifique en prenant en compte leur atteinte hepatique et la toxicite eventuelle de certains des traitements de fond de la PR. © 2007 Elsevier Masson SAS. Tous droits reserves.

Association polyarthrite rhumatoïde et cirrhose biliaire primitive Association between rheumatoid arthritis and primary biliary cirrhosis

The present invention relates to inhibitors of pro-MMP10 expression and/or MMP10 activity for treating or preventing PAH in SSc patientsThe present invention further relates to an in vitro method for diagnosing a pulmonary arterial hypertension (PAH) in a systemic sclerosis (SSc) patient from a biological sample by measuring pro-MMP expression in said sample and comparing it to one reference value

Jérôme Avouac

Papers

Systemic sclerosis at the crossroad of polyautoimmunity

Progression of patients with Raynaud's phenomenon to systemic sclerosis: a five-year analysis of the European Scleroderma Trial and Research group multicentre, longitudinal registry study for Very Early Diagnosis of Systemic Sclerosis (VEDOSS)

Association polyarthrite rhumatoïde et cirrhose biliaire primitive Association between rheumatoid arthritis and primary biliary cirrhosis

Inhibitors for treating or preventing a pulmonary arterial hypertension in systemic sclerosis patients and method for diagnosing said disease