EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)

Kowal-Bielecka, Otylia; Landewé, Robert; Avouac, Jérôme; Chwieśko, Sylwia; Miniati, Irene; Czirják, László; Clements, Philip J.; Denton, Chris; Farge, Dominique; Fligelstone, Kim; Földvari, I; Furst, D E; Müller‐Ladner, Ulf; Seibold, James R.; Silver, Richard M.; Takehara, Kazuhiko; Toth, B Garay; Tyndall, Alan; Valentini, Gabriele; Hoogen, Frank van den; Wigley, Fred; Zulian, Francesco; Matucci‐Cerinic, Marco

doi:10.1136/ard.2008.096677

Cited by 552 publications

(366 citation statements)

References 76 publications

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“…Histological examination showed thinning of the collagen fiber bundles in the dermis. Since there are few therapeutic drugs for SSc at the present time [116], these improvements observed in two SSc patients suggest that tocilizumab appears to be a promising biologic for the treatment of SSc.…”

Section: Systemic Sclerosismentioning

confidence: 94%

Anti‐interleukin‐6 receptor antibody, tocilizumab, for the treatment of autoimmune diseases

2011

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Interleukin (IL)‐6 is a cytokine with multiple biological activities. It contributes to host defense against pathogens, whereas accelerated production of IL‐6 plays a significant pathological role in various diseases. Clinical trials have demonstrated the efficacy of tocilizumab, a humanized anti‐IL‐6 receptor antibody, for patients with rheumatoid arthritis, Castleman's disease or juvenile idiopathic arthritis, leading to approval of this innovative drug for the treatment of these diseases. Since IL‐6 has been demonstrated to play a significant role in the development of various other autoimmune and inflammatory diseases, tocilizumab can be expected to become a novel drug for such diseases as well.

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Section: Systemic Sclerosismentioning

confidence: 94%

Anti‐interleukin‐6 receptor antibody, tocilizumab, for the treatment of autoimmune diseases

2011

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“…Suda et al (2009) suggested that advanced age was a risk factor for acute exacerbation in connective tissue disease-related ILD, but the age of onset was not an associated factor in our study (Table 4). About 10%-20% of patients with SSc had overlapping features with other rheumatic diseases (Kowal-Bielecka et al 2009;Pakozdi et al 2011). Pakozdi et al (2011) reported that the most common connective tissue disease was PM/DM, followed by rheumatoid arthritis and Sjögren's syndrome.…”

Section: Discussionmentioning

confidence: 99%

High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis

Tomiyama

Watanabe

Ishii

et al. 2016

Tohoku J. Exp. Med.

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Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by extensive fibrosis and autoantibodies. Its clinical manifestations are diverse and include Raynaud's phenomenon, gastrointestinal dysmotility, interstitial lung disease (ILD), pulmonary hypertension, and renal crisis. Among these, ILD is the primary cause of SSc-related death. It has been considered that acute exacerbation of ILD (AE-ILD) is not common in patients with SSc; however, little is known about the prevalence of AE-ILD in Japanese patients with SSc. In this study, we aimed to clarify the prevalence, clinical characteristics, and prognosis of patients with SSc who developed AE-ILD and to identify predictive factors for AE-ILD in our Japanese cohorts. Clinical data of patients who visited our department from 1990 to 2014 and fulfilled the 2013 classification criteria for SSc were retrospectively reviewed. A total of 139 patients were enrolled. The mean age of onset was 49.1 years, and 113 (81.3%) patients were female; 116 (83.5%) had limited cutaneous involvement, and the overall 10-year survival rate was 92.0%. Among 66 (47.5%) patients with ILD, 13 (9.4%) developed AE-ILD. Patients with AE-ILD had a significantly higher incidence of overlap with polymyositis (PM) or dermatomyositis (DM) and lower prevalence of anticentromere antibodies with higher mortality rate compared with those without AE-ILD. Multivariate Cox regression analysis identified that an overlap with PM or DM was the most significant predictive factor for AE-ILD. Our study results suggest that Japanese patients with SSc, particularly patients overlapped with PM or DM, have a high risk of AE-ILD.

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“…Nonpharmacologic care can target a broad range of symptoms simultaneously, depending on the needs expressed by patients. To help guide medical care for patients with SSc, the European League Against Rheumatism (EULAR) and the EULAR Scleroderma Trials and Research group have developed evidence-based, consensus-derived recommendations for the pharmacologic treatment of SSc (12). The authors of these guidelines also emphasized the potential importance of psychosocial and rehabilitation interventions in disease management but could not make recommendations for or against these types of interventions.…”

Section: Introductionmentioning

confidence: 99%

Effectiveness of Nonpharmacologic Interventions in Systemic Sclerosis: A Systematic Review

Willems

Vriezekolk

Schouffoer

et al. 2015

Arthritis Care & Research

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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. Two reviewers independently assessed methodologic quality using the Downs and Black checklist. Results. Twenty-three studies (9 RCTs, 4 CCTs, and 10 ODs) were included. Studies assessing comparable interventions were grouped, resulting in data for 16 different interventions. The total number of patients included per study ranged from 10 to 53. Seventeen different outcome domains were assessed, with hand function, limitations in activities, and quality of life being assessed most frequently. Three studies, all RCTs, were rated as high quality. These RCTs reported that 1) a multifaceted oral health intervention improves mouth hygiene, and additional orofacial exercises did not improve mouth opening, 2) a multidisciplinary team-care program improves limitations in activities, mouth opening, and hand grip strength, and 3) manual lymph drainage improves hand function, limitations in activities, and quality of life. Conclusion. The body of knowledge regarding nonpharmacologic care in SSc is very limited due to the wide variety in studied interventions and outcomes in the relatively uncommon but highly disabling disease. To structure and focus future research, an international consensus should be established to prioritize primary targets for nonpharmacologic treatment and the content of interventions and to agree on a core set of outcome measures.

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EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)

Cited by 552 publications

References 76 publications

Anti‐interleukin‐6 receptor antibody, tocilizumab, for the treatment of autoimmune diseases

Anti‐interleukin‐6 receptor antibody, tocilizumab, for the treatment of autoimmune diseases

High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis

Effectiveness of Nonpharmacologic Interventions in Systemic Sclerosis: A Systematic Review

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