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)

Bellando-Randone, Silvia; Galdo, Francesco Del; Lepri, Gemma; Minier, Tünde; Huscher, Dörte; Fürst, Daniel E.; Allanore, Yannick; Distler, Oliver; Czirják, László; Bruni, Cosimo; Guiducci, Serena; Avouac, Jérôme; Cutolo, Maurizio; Smith, Vanessa; Matucci‐Cerinic, Marco

doi:10.1016/s2665-9913(21)00244-7

Cited by 71 publications

(53 citation statements)

References 25 publications

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“…Moreover, in patients with Raynaud’s phenomenon, detection of autoantibodies predicts microvascular damage in nailfold capillary microscopy and subsequent diagnosis of SSc [ 12 ]. This aspect is taken into account in the criteria for “Very Early Diagnosis Of SSc” (VEDOSS), which include the presence of autoantibodies [ 13 ].…”

Section: Which Mechanisms Are Involved In Maturation Of Autoreactive ...mentioning

confidence: 99%

Autoantibodies as Biomarker and Therapeutic Target in Systemic Sclerosis

et al. 2022

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Systemic sclerosis (SSc) is a rare connective tissue disorder characterized by immune dysregulation evoking the pathophysiological triad of inflammation, fibrosis and vasculopathy. In SSc, several alterations in the B-cell compartment have been described, leading to polyclonal B-cell hyperreactivity, hypergammaglobulinemia and autoantibody production. Autoreactive B cells and autoantibodies promote and maintain pathologic mechanisms. In addition, autoantibodies in SSc are important biomarkers for predicting clinical phenotype and disease progression. Autoreactive B cells and autoantibodies represent potentially promising targets for therapeutic approaches including B-cell-targeting therapies, as well as strategies for unselective and selective removal of autoantibodies. In this review, we present mechanisms of the innate immune system leading to the generation of autoantibodies, alterations of the B-cell compartment in SSc, autoantibodies as biomarkers and autoantibody-mediated pathologies in SSc as well as potential therapeutic approaches to target these.

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Section: Which Mechanisms Are Involved In Maturation Of Autoreactive ...mentioning

confidence: 99%

Autoantibodies as Biomarker and Therapeutic Target in Systemic Sclerosis

et al. 2022

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“…This study followed 1150 subjects with RP, of whom 553 met VEDOSS criteria and had available data at follow-up. 11 Disease progression was defined as the fulfillment of ACR/EULAR classification criteria for SSc. Of the 553 patients, 254 experienced progression or reached 5-year follow-up.…”

Section: Very Early Sscmentioning

confidence: 99%

Predicting the Progression of Very Early Systemic Sclerosis: Current Insights

Bellocchi¹,

Chung²,

Volkmann³

2022

OARRR

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Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease with distinct pathological hallmarks (ie, inflammation, vasculopathy, fibrosis) that may predominate at different stages in the disease course with varying severity. Initial efforts to classify patients with SSc identified a subset of patients with very early SSc. These patients possessed signs of SSc (eg, Raynaud phenomenon, SSc specific autoantibodies and/or nailfold capillary abnormalities) without fulfilling complete SSc classification criteria. Recognizing the inherent value in early diagnosis and intervention in SSc, researchers have endeavored to identify risk factors for progression from very early SSc to definite SSc. The present review summarizes the clinical phenotype of patients with very early and early SSc. Through a scoping review of recent literature, this review also describes risk factors for progression to definite SSc with a focus on the specific clinical features that arise early in the SSc disease course (eg, diffuse cutaneous sclerosis, interstitial lung disease, esophageal dysfunction, renal crisis, cardiac involvement). In addition to clinical risk factors, this review provides evidence for how biological data (ie, serological, genomic, proteomic profiles, skin bioengineering methods) can be integrated into risk assessment models in the future. Furthering our understanding of biological features of very early SSc will undoubtedly provide novel insights into SSc pathogenesis and may illuminate new therapeutic targets to prevent progression of SSc.

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“…Key investigations are nailfold capillaroscopy and detection of autoantibodies as they are both strongly predictive of the development of SSc or other autoimmune diseases [12,13 ▪▪ ]. Nailfold capillaroscopy can be performed using low- (e.g., handheld dermatoscope or USB-microscope) and high-magnification (e.g., videocapillaroscopy) systems.…”

Section: Diagnosismentioning

confidence: 99%

Practical management of Raynaud's phenomenon – a primer for practicing physicians

Ramahi

Hughes

Khanna

2022

Current Opinion in Rheumatology

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Purpose of reviewRaynaud's phenomenon (RP) is a common vasospastic condition that results in digital hypoperfusion in response to cold and/or emotional stress and is associated with significant pain and disability. The aim of our review is to provide a practical approach for clinicians to inform assessment and management of patients with RP.Recent findingsAutoantibodies and nailfold capillaroscopy are key investigations to stratify the risk of progression to systemic sclerosis (SSc) in patients RP, which was recently confirmed in the multicenter, very early diagnosis of systemic sclerosis (VEDOSS) project. Research has explored the complex lived-patient experience of RP including digital vasculopathy in SSc and has highlighted the need for outcome measure development to facilitate research in the field. Pharmacological treatment strategies vary significantly internationally and there is continued interest in developing surgical approaches.SummaryWe provide a practical and up-to-date approach to inform the assessment and management of patients with RP including guidance on drug initiation and escalation. Calcium channel blockers are first-line treatment and can be initiated by primary care physicians. We also highlight second-line drug therapies used for refractory RP and the potential role for surgical intervention.

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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)

Cited by 71 publications

References 25 publications

Autoantibodies as Biomarker and Therapeutic Target in Systemic Sclerosis

Autoantibodies as Biomarker and Therapeutic Target in Systemic Sclerosis

Predicting the Progression of Very Early Systemic Sclerosis: Current Insights

Practical management of Raynaud's phenomenon – a primer for practicing physicians

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