Systemic sclerosis without antinuclear antibodies or Raynaud's phenomenon: a multicentre study in the prospective EULAR Scleroderma Trials and Research (EUSTAR) database

Schneeberger, Daniel; Tyndall, Alan; Kay, Jonathan; Søndergaard, Klaus; Carreira, Patrícia; Morgiel, Ewa; Deuschle, Katrin; Derk, Chris T.; Widuchowska, Małgorzata; Walker, Ulrich A.

doi:10.1093/rheumatology/kes315

Cited by 26 publications

(20 citation statements)

References 23 publications

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“…Patients within this group generally do not have organ involvement, Raynaud's phenomenon and also have negative antibodies; these data may suggest that even though they are classified as SSc probably they do not have an overt disease, and that non-specific capillary abnormalities may precede a full-blown scleroderma pattern. This subgroup has been recently studied in depth by other EUSTAR members (Schneeberger et al, 2013).…”

Section: Discussionmentioning

confidence: 99%

Nailfold capillaroscopy in systemic sclerosis: Data from the EULAR scleroderma trials and research (EUSTAR) database

Ingegnoli

Ardoino

Boracchi

et al. 2013

Microvascular Research

103

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Section: Discussionmentioning

confidence: 99%

Nailfold capillaroscopy in systemic sclerosis: Data from the EULAR scleroderma trials and research (EUSTAR) database

Ingegnoli

Ardoino

Boracchi

et al. 2013

Microvascular Research

103

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“…In this Chinese cohort of SSc subjects, 4.2% of subjects were negative for ANA, which is approximately in agreement with published ndings. EUSTAR has also identi ed their subjects without ANA or Raynaud's phenomenon as a very small subgroup of SSc [8]. Hamaguchi and colleagues [9] reported that the absence of ANA was found in 5% SSc subjects in the Japanese population.…”

Section: Discussionmentioning

confidence: 99%

Systemic Sclerosis Patients With Negative Antinuclear Antibodies Have Distinctive Clinical Manifestations: a Multicenter CRDC Cohort in China

Hui

Zhou

Zhang

et al. 2021

Preprint

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Objective: The presence of circulating antinuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). A variety of ANAs are associated with unique sets of disease manifestations and are widely used in clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvement and prognosis in SSc. This study aimed to investigate the clinical features of SSc patients negative for ANAs in a Chinese Rheumatism Data Center (CRDC) multicenter cohort in China.Methods: Based on the CRDC database, patients were prospectively recruited between April 2008 and June 2019 from 154 clinical centers nationwide, and all cases fulfilled the 2013 ACR/EULAR classification criteria for systemic sclerosis. Results for antinuclear antibodies were intensively collected. Demographic, clinical, and laboratory data were compared between ANA-positive SSc patients and those negative for ANAs.Results: Antinuclear antibodies were detected in 2129 of 2809 patients enrolled in the study; 4.2% patients were negative. There was a significant difference between patients negative and positive for ANAs based on sex (29/60 vs 294/1746, p＜0.001). The presence of Raynaud’s phenomenon was less common (71.8% vs 91.8%, p＜0.001) in the ANA-negative patients. In addition, the incidence of certain critical organ involvement, including gastroesophageal reflux (5.6% vs 18.5%, p=0.002), interstitial lung disease (65.2% vs 77.9%, p=0.015) and pulmonary arterial hypertension (11.5% vs 29.0%, p=0.006), was significantly lower in ANA-negative patients than in ANA-positive patients. The proportion of abnormal ESR (32.4% vs 47.6%, p=0.013) and IgG elevation (14.3% vs 37.0%, p=0.003), an indicator of disease activity, was significantly lower in ANA-negative patients than in ANA-positive patients.Conclusion: Antinuclear antibodies are strongly associated with the clinical manifestations of systemic sclerosis, with ANA-negative SSc patients tending to exhibit relatively milder disease.

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“…Fourth, although—or because of—independence from industry, the database facilitated novel treatment approaches, which assist to alter the course of the disease significantly 15 16. This will apply also to the rare juvenile32 the late-onset33 and the ANA/Raynauds’ negative34 forms. Fifth, perhaps the most important and durable in the outcome of the EUSTAR initiative, is the launching of an immense scientific interest in the disease, which resulted in an increasing number of spinoff cooperative international projects,35 for example, together with the US-based scleroderma trials consortium (SCTC) that far exceeds the initial idea of a ‘database’.…”

Section: How Has the Eustar Database Contributed To New Understandingmentioning

confidence: 99%

Ten years EULAR Scleroderma Research and Trials (EUSTAR): what has been achieved?

et al. 2013

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Ten years ago a group of researchers interested in and dedicated to the field of systemic sclerosis started a unique experiment called European League Against Rheumatism (EULAR) Scleroderma Trials And Research (EUSTAR) that was designed to establish a large-scale database in a rare disease to facilitate the exact characterisation of this debilitating disease, spread the knowledge even beyond the borders of Europe and stimulate innovative research targeting the major problems of the affected patients. This EUSTAR experiment, with all its facets, including the creation of a large-scale database, the initiation of more than 40 investigator-driven clinical and basic science projects, the teaching of more than 400 young clinicians in the field of systemic sclerosis and the realisation of multicenter EU grants, which were all facilitated by an initial research grant from EULAR, is outlined and commented upon by the members of the steering committee in this viewpoint article on behalf of the now more than 150 contributing centres and the international systemic sclerosis patients' association, the Federation of European Scleroderma Associations.

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Systemic sclerosis without antinuclear antibodies or Raynaud's phenomenon: a multicentre study in the prospective EULAR Scleroderma Trials and Research (EUSTAR) database

Abstract: We have identified a very small subgroup of SSc patients who lack both circulating ANA and RP, none of whom fulfils the diagnostic criteria for any known SSc mimic. Prospective studies are needed to elucidate the clinical presentation, evolution and outcome of such patients.

Cited by 26 publications

References 23 publications

Nailfold capillaroscopy in systemic sclerosis: Data from the EULAR scleroderma trials and research (EUSTAR) database

Nailfold capillaroscopy in systemic sclerosis: Data from the EULAR scleroderma trials and research (EUSTAR) database

Systemic Sclerosis Patients With Negative Antinuclear Antibodies Have Distinctive Clinical Manifestations: a Multicenter CRDC Cohort in China

Ten years EULAR Scleroderma Research and Trials (EUSTAR): what has been achieved?

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Systemic sclerosis without antinuclear antibodies or Raynaud's phenomenon: a multicentre study in the prospective EULAR Scleroderma Trials and Research (EUSTAR) database

Abstract: We have identified a very small subgroup of SSc patients who lack both circulating ANA and RP, none of whom fulfils the diagnostic criteria for any known SSc mimic. Prospective studies are needed to elucidate the clinical presentation, evolution and outcome of such patients.

Cited by 26 publications

References 23 publications

Nailfold capillaroscopy in systemic sclerosis: Data from the EULAR scleroderma trials and research (EUSTAR) database

Nailfold capillaroscopy in systemic sclerosis: Data from the EULAR scleroderma trials and research (EUSTAR) database

Systemic Sclerosis Patients With Negative Antinuclear Antibodies Have Distinctive Clinical Manifestations: a Multicenter CRDC&nbsp;Cohort in China

Ten years EULAR Scleroderma Research and Trials (EUSTAR): what has been achieved?

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Systemic Sclerosis Patients With Negative Antinuclear Antibodies Have Distinctive Clinical Manifestations: a Multicenter CRDC Cohort in China