Ethnic influence on the phenotype of French patients with systemic sclerosis

Steelandt, Alexia; Benmostefa, Nouria; Avouac, Jérôme; Mouthon, Luc; Allanore, Yannick

doi:10.1016/j.jbspin.2020.09.013

Cited by 15 publications

(5 citation statements)

References 21 publications

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“…Nonetheless, recent studies suggest that antibody subtypes could be surrogate markers of genetic differences in patients with SSc [32]. Within the same geographical region, race may also impact the phenotype and antibody subtypes of patients with SSc [33]. Black patients more frequently have SSc-ILD and a lower prevalence of anti-centromere antibodies as compared with white patients [9,33,34].…”

Section: Discussionmentioning

confidence: 99%

“…Within the same geographical region, race may also impact the phenotype and antibody subtypes of patients with SSc [33]. Black patients more frequently have SSc-ILD and a lower prevalence of anti-centromere antibodies as compared with white patients [9,33,34]. We did not include ethnicity in our study as this question was recently explored in EUSTAR, with a study showing that black patients had more severe disease when considering the prevalence of dcSSc compared with white patients and that the mortality rate was higher in Asian patients than in white patients [9].…”

Section: Discussionmentioning

confidence: 99%

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Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region

et al. 2022

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Objectives The prevalence and characteristics of systemic sclerosis-associated interstitial lung disease (SSc-ILD) vary between geographical regions worldwide. The objectives of this study were to explore the differences in terms of prevalence, phenotype, treatment, and prognosis in patients with SSc-ILD from predetermined geographical regions in the EUSTAR database. Methods Patients were clustered into seven geographical regions. Clinical characteristics and survival of patients with SSc-ILD were compared among these pre-determined regions. Results For baseline analyses, 9260 SSc patients were included, with 6732 for survival analyses. The prevalence of SSc-ILD in the overall population was 50.2%, ranging from 44.0% in “Western Europe & Nordic countries” to 67.5% in “Eastern European, Russia & Baltic countries”. In all regions, anti-topoisomerase antibodies were associated with SSc-ILD. Management also significantly differed; mycophenolate mofetil was prescribed at baseline in 31.6% of patients with SSc-ILD in “America (North & South)” and 31.7% in “Middle East” but only 4.3% in “Asia & Oceania” (P < 0.0001). Patients from “America (North & South)” and “Middle East” had the highest survival rate at the end of follow-up (85.8% and 85.2%, respectively). Conclusion Our study highlights key differences among regions in terms of clinical presentation and prognosis of SSc-ILD. This work also demonstrates that the management of SSc-ILD is highly variable among the different regions considered, suggesting that efforts are still needed for the standardisation of medical practice in the treatment of this disease.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region

et al. 2022

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“…Digestive symptoms were overall more frequently found in dcSSc, with dysphagia also being significantly more prevalent in this subgroup. Previously published research describing the prevalence and severity of gastrointestinal involvement according to disease phenotype has reported discrepant results, with some studies identifying more frequent or more severe digestive symptoms in lcSSc [59,60].…”

Section: Discussionmentioning

confidence: 92%

Diet in Scleroderma: Is There a Need for Intervention?

et al. 2021

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Systemic sclerosis (SSc) patients exhibit a plethora of risk factors for nutritional decline, including the presence of chronic inflammation and the progressive nature of disease-related multisystem involvement. The prevalence and consequences of nutritional decline in scleroderma are frequently underestimated, its management currently remaining a subject of debate. The main objective of the present study was to perform a detailed assessment of scleroderma patients’ diet as well as their eating habits and to describe the relationships with weight loss and malnutrition risk in the absence of professional nutritional counseling. Methods: We used a translated and validated version of the EPIC-Norfolk FFQ (European Prospective Investigation into Cancer and Nutrition Norfolk Food Frequency Questionnaire) to evaluate the patients’ diet and MUST (Malnutrition Universal Screening Tool) to investigate the risk of malnutrition. Disease activity was estimated using the EUSTAR-AI (European Scleroderma Trials and Research group Activity Index). Results: We included 69 patients with SSc, of which 42 underwent a detailed dietary assessment. Dietary factors were connected to body composition and digestive symptoms. We found high sodium intake and frequent suboptimal energy consumption in our study group, including patients with cardiopulmonary involvement. Liver transaminases were inversely correlated with the consumption of nuts and seeds. Malnutrition and weight loss were significantly associated with pulmonary hypertension, heart failure, albumin levels, and the extent of skin fibrosis, but not advanced age. Although the patients with EUSTAR-AI ≥ 2.5 were more frequently included in the moderate and high malnutrition risk categories, these results did not reach statistical significance. Conclusions: Currently, there is an unmet need for longitudinal and interventional research focusing on the long-term significance, ramifications, and management of nutritional impairment in SSc patients with various clinical manifestations. Our results indicate that scleroderma patients could benefit from personalized nutritional counseling in an interdisciplinary setting.

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“…Among African American and Hispanic patients with similar sociodemographic parameters in previous studies, Hispanic patients had higher DLCO, less fibrosis and lower frequency of PAH. 12 Black/African American patients have been shown to have higher levels of both anti-ribonucleoprotein (U1-RNP) and antifibrillin antibodies (U3-RNP) compared to non-Black patients with SSc, [45][46][47] a higher proportion of Asian patients compared to White patients have anti-U1-RNP positivity, 10 and more severe fibrosis is associated with anti-U1 RNP-positivity. 9 Among African American patients with scleroderma in the Genome Research in African American Scleroderma Patients (GRASP) cohort, 14-18% were positive for anti-RNPs and 20% had a concomitant overlapping autoimmune disease.…”

Section: Discussionmentioning

confidence: 99%

Racial and ethnic associations with interstitial lung disease and healthcare utilization in patients with systemic sclerosis

Tukpah,

Rose,

Seger

et al. 2024

Preprint

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Rationale: Racial and ethnic differences in presentation and outcomes have been reported in systemic sclerosis (SSc) and SSc-interstitial lung disease (ILD). However, diverse cohorts and additional modeling can improve understanding of risk features and outcomes, which is important for reducing associated disparities. Objective(s): To determine if there are racial/ethnic differences associated with SSc-ILD risk and age; time intervals between SSc and ILD, and with emergency department (ED) visit or hospitalization rates. Methods: A retrospective cohort study using electronic health record data from an integrated health system, over a 5.5 year period was conducted using clinical and sociodemographic variables, models were generated with sequential adjustments for these variables. Logistic regression models were used to examine the association of covariates with ILD and age at SSc-ILD. Healthcare outcomes were analyzed with complementary log-log regression models. Results: The cohort included 756 adults (83.6% female, 80.3% non-Hispanic White) with SSc with a mean age of 59 years. Overall, 33.7% of patients in the cohort had an ILD code, with increased odds for Asian (odds ratio [OR], 2.59; 95% confidence interval [CI], 1.29, 5.18; P=.007) compared to White patients. The age in years of patients with SSc-ILD was younger for Hispanic (mean difference, -6.5; 95% CI, -13 , -0.21; P = 0.04) and Black/African American patients (-10; 95% CI -16 , -4.9; P <0.001) compared to White patients. Black/African American patients were more likely to have an ILD code before an SSc code (59% compared to 20.6% of White patients), and had the shortest interval from SSc to ILD (3 months). Black/African American (HR, 2.59; 95% CI 1.47, 4.49; P=0.001) and Hispanic patients (HR 2.29; 95% CI 1.37, 3.82; P=0.002) had higher rates of an ED visit. Conclusion: In this study, SSc-ILD presentation and outcomes differed by racial/ethnic group (increased odds of SSc-ILD, younger age at SSc-ILD, and preceding diagnosis with respect to SSc, rates of ED visit), some of which was attenuated with adjustment for clinical and sociodemographic characteristics. Differing presentation may be driven by social drivers of health (SDOH), autoantibody profiles, or other key unmeasured factors contributing to susceptibility and severity.

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Ethnic influence on the phenotype of French patients with systemic sclerosis

Cited by 15 publications

References 21 publications

Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region

Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region

Diet in Scleroderma: Is There a Need for Intervention?

Racial and ethnic associations with interstitial lung disease and healthcare utilization in patients with systemic sclerosis

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