Subsets in systemic sclerosis: one size does not fit all

Leclair, Marc‐David; Hudson, Marie; Proudman, S.; Stevens, W.; Mj, Fritzler; Wang, M; Pope, Janet; Baron, Murray; Markland, Janet; Robinson, David; Khalidi, Nader; Masetto, A.; Sutton, Evelyn; Thorne, Carter; Gyger, Geneviève; Smith, D. P.; Fortin, Paul R.; Larché, Maggie; Rodriguez-Reyna, TS; Nikpour, Mandana; Sahhar, J.; Zochling, Jane; Roddy, Janet; Walker, Jenny; Youssef, Peter

doi:10.5301/jsrd.5000212

Cited by 11 publications

(12 citation statements)

References 16 publications

(24 reference statements)

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“…Cluster analysis has been applied to various conditions, such as gout , chronic heart failure , asthma , mixed connective tissue diseases , and antineutrophil cytoplasmic antibody–associated vasculitis . Cluster analyses have also been carried out in 2 SSc studies, to our knowledge . One of them included patients from the EULAR European Scleroderma Trials and Research (EUSTAR) cohort but was centered on capillaroscopy patterns .…”

Section: Introductionmentioning

confidence: 99%

“…One of them included patients from the EULAR European Scleroderma Trials and Research (EUSTAR) cohort but was centered on capillaroscopy patterns . Another recent study took into account a limited number of cluster variables and a limited number of patients . The aim of this study was to distinguish and characterize homogeneous groups of SSc patients using cluster analysis within the large EUSTAR cohort, and analyze survival between the clusters obtained.…”

Section: Introductionmentioning

confidence: 99%

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Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis

Sobanski

Giovannelli

Allanore

et al. 2019

Arthritis & Rheumatology

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Objective Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. Methods A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty‐four clinical and serologic variables were used for clustering. Results Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. Conclusion Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis

Sobanski

Giovannelli

Allanore

et al. 2019

Arthritis & Rheumatology

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“…4 For this reason, LeRoy et al 5 proposed new criteria, including antibodies (Abs), nailfold videocapillaroscopy (NVC) and clinical features, also recognizing two cutaneous subsets of the disease: limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc), based on skin involvement and clinical features. Although, the sub-classification of SSc in different subsets or clusters remains a challenge for rheumatologists, 6 in the recent years, more attention was posed on SSc-specific Abs and on NVC in order to try to identify patients in an ‘early phase’ of SSc. 7 In 2013, the American College of Rheumatology (ACR) and the European League against Rheumatism (EULAR) developed the new classification criteria for SSc, including clinical, laboratory (the three specific Abs, anticentromere -ACA-, anti-topoisomerase -TopoI- and anti-RNA polymerase III -anti-RNAP-) and instrumental features.…”

Section: Introductionmentioning

confidence: 99%

Systemic sclerosis and primary biliary cholangitis: An overlapping entity?

Lepri

Randone

Cerinic

et al. 2018

Journal of Scleroderma and Related Disorders

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Systemic sclerosis (SSc) is a complex autoimmune disease that may lead to skin and internal organ fibrosis. Based on skin involvement, two subsets of the disease are recognized (limited cutaneous SSc and diffuse cutaneous SSc). The new 2013 American College of Rheumatology/European League against Rheumatism classification criteria allow to identify SSc patients at the early stage of the disease that allows new research avenues. The aetiology of the disease is still unknown, but it has an important autoimmune basis and its association with other autoimmune diseases has been reproducibly reported. Among them, primary biliary cholangitis is considered the most common liver disease in SSc. The aim of this review is to provide an overview on recent findings about SSc associated to primary biliary cholangitis. Although the aetiology of the two diseases is still unknown, data suggest that these two disorders share the expression of fibrogenic cytokines, involved both in generation and function of T lymphocytes subpopulation (Th17 cells) and regulatory T lymphocytes. In addition, the relationships between SSc and primary biliary cholangitis may be closer as suggested by the presence of primary biliary cholangitis-specific antibodies in SSc patients and vice versa. Recent findings confirm a prevalence of overt primary biliary cholangitis in about 2% of SSc population, in particular in patients with limited cutaneous SSc and positive anticentromere antibodies. The prevalence increases if also patients with only primary biliary cholangitis-specific antibodies are considered. Data regarding SSc prevalence in primary biliary cholangitis patients have also been recently clarified. Altogether, stimulating results are moving the field forward regarding the relationships of these two autoimmune and fibrotic disorders that may belong to an overlapping entity.

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“…Skin fibrosis is a well-known distinctive trait of the disease and the severity of skin involvement has been reported to be predictive of disease mortality [6]. Usually, the disease is classified according to skin involvement extension in a limited and a diffuse subset, but today it is clearly evident that this subsetting does not express the practical reality of the disease [7,8]. In fact, the disease is characterized by a wide variability in both the clinical phenotype and in progression.…”

mentioning

confidence: 99%