Systemic sclerosis: one year in review 2022

Lepri, Gemma; Orlandi, Martina; Battista, Marco Di; Mattia, Gianmarco De; Rio, Mattia Da; Codullo, Veronica; Guiducci, Serena; Rossa, Alessandra Della

doi:10.55563/clinexprheumatol/3401fl

Cited by 5 publications

(5 citation statements)

References 44 publications

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“…This figure is very similar to the frequency of anti-Th/To among SSc patients reported by a recent review of the literature who collected data from 25 articles (15); notably, these antibodies seemed to be less frequent in Europe (1.5%) than in other areas of the world (15). We confirmed the main known clinical associations of anti-Th/To antibodies: all our patients were affected by limited cutaneous involvement, accordingly with previous reports (median percentage: 89%) ( with lower survival in SSc patients, including male gender, non-Caucasian ethnicity, longer disease duration, and smoking habit (17)(18)(19)(20)(21)(22), were reported to be more prevalent among anti-Th/ To+ patients followed in Pittsburgh (7,9). The higher frequency of smokers among anti-Th/To+ patients might be particularly interesting, considering the potential link with the high frequency of ILD in this SSc subset, and was also reported by others (11) and confirmed in our study.…”

Section: Discussionsupporting

confidence: 89%

Anti-Th/To antibodies in systemic sclerosis: analysis of long-term follow-up of pulmonary involvement, organ damage accrual and mortality in an Italian cohort with a case-control study

Moschetti

Lazzaroni

Cavazzana

et al. 2022

Clinical and Experimental Rheumatology

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ObjectivesIn systemic sclerosis (SSc) American patients, anti-Th/To antibodies were reported to be associated with interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). Few data in European patients are available, so we aimed at describing the clinical associations of anti-Th/To antibodies, focusing on ILD outcome, organ damage and mortality in an Italian single-centre cohort. Methods Case-control study: anti-Th/To+ SSc patients vs. anti-topoisomerase (anti-topo)1+, anticentromere (ACA)+ and quadruple-negative (anti-topo 1-, ACA-, anti-RNAP3-, anti-Th/To-) SSc patients (1:3; matched for sex and age at SSc onset).Organ damage was assessed with the SCTC-Damage Index. ResultsThirteen anti-Th/To+ patients were evaluated: 100% had limited cutaneous involvement; 46% digital ulcers; none had PAH, synovitis, joint contractures. As compared to anti-topo 1+ and quadruple-negative patients, anti-Th/To+ patients developed less frequently ILD (40% vs. 85% and 84%), that required less immunosuppression (8% vs. 41% and 44%), and rarely had functional worsening (15.4% at 5 years), without development of long-term complications (no need for O 2 , pulmonary hypertension, death). In anti-Th/To+ patients, the Damage Index was lower than in anti-topo 1+ and quadruple-negative patients at various timepoints, and remained low during the long-term follow-up (median: 16 years). The 5-and 10-year survival of anti-Th/To+ patients was 92% and 72%, respectively, and did not differ from those of the SSc matched patients; none of the anti-Th/To+ patients died due to SSc, while mortality was mainly related to cancer. ConclusionsIn this study, anti-Th/To+ patients showed a mild SSc phenotype, characterised by low organ damage, favourable ILD outcome and good survival.

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Section: Discussionsupporting

confidence: 89%

Anti-Th/To antibodies in systemic sclerosis: analysis of long-term follow-up of pulmonary involvement, organ damage accrual and mortality in an Italian cohort with a case-control study

Moschetti

Lazzaroni

Cavazzana

et al. 2022

Clinical and Experimental Rheumatology

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“…4B). Beta-hexosaminidase A enzyme deficiency is associated with Tay-Sachs disease (33) (38). Anti-DNA topoisomerase IIα autoantibody levels were increased in Japanese SSc patients compared to HC, and interstitial lung disease was more frequently observed in the SSc patients who had anti-DNA topoisomerase IIα antibody compared to SSc patients without the antibody (39).…”

Section: Discussionmentioning

confidence: 99%

Gut microbiome profiling in systemic sclerosis: a metagenomic approach

Tan

Chandrasekaran

Leung

et al. 2022

Clinical and Experimental Rheumatology

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ObjectivesThe early gastrointestinal (GI) manifestation of systemic sclerosis (SSc) suggests a possible GI microbiota engagement in the pathophysiology and/or progression of SSc. Previous studies have revealed dysbiosis among Caucasian SSc patients.This study extends these findings in Asian SSc patients.Methods Adult SSc patients, stratified according to 1) on immunosuppressive (On-IS) drugs or 2) no immunosuppressive drugs (No-IS), and age-and-sex-matched healthy controls (HC) were recruited. Metagenomic sequencing of stool DNA was compared between SSc patients and HC, and between SSc (On-IS) and (No-IS) patients. Alpha and beta-diversity, taxonomic and functional profiling were evaluated. Results Twenty-three female SSc patients (12 On-IS; 11 No-IS; 5 diffuse and 18 limited SSc subtype) and 19 female HC, withmedian age of 54 years and 56 years, respectively, were recruited. Median SSc disease duration was 3.3 years. Alpha diversity was significantly higher in SSc versus HC (p=0.014) and in SSc (No-IS) versus HC (p=0.006). There was no significant difference in beta diversity between SSc and HC (p=0.307). At the phyla level, there were significantly increased abundance of Firmicutes and Actinobacteria in SSc versus HC, and reduced abundance of Bacteroidetes (all p<0.001). At the species level, there were significantly increased abundance of several Lactobacillus, Bifidobacterium, and Coprococcus species in SSc, and increased abundance of Odoribacter, Bacteroides and Prevotella species in HC. KEGG pathway analysis demonstrated distinct differences between SSc vs HC, and between SSc (No-IS) and SSc (On-IS). Conclusion Using metagenomic sequencing, our study further underlines distinct alterations in microbiota profiling among Asian SScpatients.

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“…O metotrexato também apresenta benefício, principalmente sobre as formas cutâneas. Corticoides não são úteis para o tratamento da ES, devendo ser utilizado apenas nos casos com apresentações clínicas inflamatórias (serosite, artrite, fase edematosa cutânea) e sempre dando preferência a doses mais baixas (prednisona de 5-20mg/dia) 24,25 .…”

Section: O Colégio Americano Deunclassified

“…O objetivo é impedir a progressão da doença para os demais órgãos e sistemas, bem como individualizar o tratamento conforme as necessidades de cada paciente 4,24,25,29,30 .…”

Section: Um Estudo Transversalunclassified

Esclerose Sistêmica: Relato De Caso De Um Diagnóstico Precoce Na Ausência De Fenômeno De Raynaud E Revisão De Literatura

Debona,

Ferreira,

Murta Júnior

et al. 2024

Rev. Contemp.

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A Esclerose Sistêmica (ES) é uma doença autoimune crônica, multissistêmica, progressiva e de apresentação heterogênea envolvendo a pele e vários sistemas orgânicos. É considerada uma doença rara, com incidência anual estimada em 14 casos por milhão de habitantes e prevalência de 35 casos por milhão de habitantes. Tem apresentação clínica variável e pode ser classificada com base na extensão do acometimento cutâneo, associada ao padrão de envolvimento de órgãos e sistemas (gastrointestinal, pulmonar, renal, cardiovascular e musculoesquelético). Os sinais clínicos são diversos, sendo os mais comuns o espessamento e endurecimento da pele, por vezes associado a edema e fenômeno de Raynaud (FRy). Diagnosticar esta condição é um desafio para os médicos devido à sua apresentação diversificada. Para auxiliar no diagnóstico, os critérios de classificação do Colégio Americano de Reumatologia e da Liga Europeia Contra o Reumatismo (ACR/EULAR) de 2013 são usados para orientar o raciocínio diagnóstico. Não existe tratamento curativo para esta condição; o objetivo é prevenir a progressão para estágios avançados de fibrose. Este artigo tem como objetivo revisar o assunto e relatar o diagnóstico precoce de um paciente com ES Cutânea Difusa que apresentou sintomas de edema de dedos, esclerodermia de dedos e membros superiores durante 5 meses na ausência de FRy e que obteve de forma precoce o diagnóstico apesar do padrão incomum de acometimento.

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Systemic sclerosis: one year in review 2022

Cited by 5 publications

References 44 publications

Anti-Th/To antibodies in systemic sclerosis: analysis of long-term follow-up of pulmonary involvement, organ damage accrual and mortality in an Italian cohort with a case-control study

Anti-Th/To antibodies in systemic sclerosis: analysis of long-term follow-up of pulmonary involvement, organ damage accrual and mortality in an Italian cohort with a case-control study

Gut microbiome profiling in systemic sclerosis: a metagenomic approach

Esclerose Sistêmica: Relato De Caso De Um Diagnóstico Precoce Na Ausência De Fenômeno De Raynaud E Revisão De Literatura

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