Raynaud's phenomenon is associated with an increased risk of cardiovascular disease and venous thromboembolism.

Objectives: Raynaud's phenomenon (RP) is common affecting 3-5 % of the population; however, there are little data concerning vascular outcomes. We aimed to estimate risk of cardiovascular disease (CVD) and venous thromboembolism (VTE) in individuals with RP without underlying relevant systemic autoimmune rheumatic diseases (SARDs).

Methods: A cohort study using data from North American electronic healthcare organization records.

Esophageal dysmotility patterns are associated with distinct clinical phenotypes and prognosis in patients with systemic sclerosis.

Objectives: Esophageal dysmotility is a common manifestation of SSc, contributing to substantial morbidity. We sought to determine whether esophageal dysmotility patterns, by high-resolution esophageal manometry were associated with distinct SSc clinical phenotypes and different outcomes.

Methods: We analyzed a cohort of SSc patients with detailed clinical and immunological data.

The role of surgery in esophageal involvement in systemic sclerosis: A systematic literature review.

Background: Gastroesophageal reflux disease (GERD) is one of the earliest clinical manifestations of systemic sclerosis (SSc). Proton pump inhibitors are ineffective in controlling symptoms in up to 40 % of cases, and their chronic use (often) at high-doses and long-term safety are a concern. For this reason, surgery has been proposed as an alternative management strategy for refractory GERD in SSc patients.

Gastrointestinal manifestations of systemic sclerosis: current approaches and emerging therapies.

Purpose Of Review: This review highlights recent advances in the understanding and management of gastrointestinal manifestations in systemic sclerosis (SSc). It is intended for clinicians and researchers aiming to improve diagnostic accuracy and therapeutic strategies in managing SSc-related gastrointestinal disease.

Recent Findings: Gastrointestinal involvement in SSc is highly variable in terms of clinical presentation, symptom severity, progression, timing of onset, and response to treatment.

Antimitochondrial antibodies in systemic sclerosis target enteric neurons and are associated with GI dysmotility.

Objectives: Most patients with systemic sclerosis (SSc) experience gastrointestinal (GI) dysmotility. The enteric nervous system (ENS) regulates GI motility, and its dysfunction causes dysmotility. A subset of SSc patients harbour antimitochondrial M2 autoantibodies (AMA).

Hand Swelling and Other Non-Raynaud Phenomenon Symptoms as the Initial Presentation of Systemic Sclerosis: Prevalence and Clinical Associations in Two US Cohorts.

Objective: Raynaud phenomenon (RP) is often the initial clinical manifestation of systemic sclerosis (SSc), but some patients develop other manifestations first. To help elucidate the diversity of SSc presentation in its early stages, we describe the initial clinical manifestations and antinuclear antibody (ANA) profiles of patients in two early SSc cohorts.

Methods: All patient data in the Genetics vs Environment in Scleroderma Outcomes Study (GENISOS) and Collaborative National Quality and Efficacy Registry (CONQUER) cohorts were reviewed.

Avoidant or Restrictive Food Intake Disorder Symptoms in Adults With Systemic Sclerosis: A Nationwide Study in Spain.

Objective: Patients with systemic sclerosis (SSc) may restrict food intake to manage their symptoms (particularly gastrointestinal [GI]). Whether some patients may develop nutritional and/or quality-of-life impairments indicative of an eating disorder, avoidant or restrictive food intake disorder (ARFID), is unknown. We aimed to (1) identify the prevalence and characteristics of ARFID symptoms in patients with SSc and (2) explore the relationship among ARFID symptoms, GI symptom burden, and health-related quality of life.

Clinicians' perspectives concerning treatment initiation and escalation strategies for digital ulcers in patients with systemic sclerosis.

Objectives: Digital ulcers (DUs) are a major cause of pain and disability in systemic sclerosis (SSc) patients and remain a major treatment challenge. Our aim was to explore clinicians' perspectives towards treatment initiation and escalation, akin to a 'Treat to Target' (T2T) strategy.

Methods: SSc clinicians were invited to participate in an online survey.

Anti-muscarinic 3 antibodies associate with a severe clinical phenotype in patients with systemic sclerosis.

Objectives: Functional antibodies play a role in SSc gastrointestinal (GI) disease, but their clinical relevance is unclear. We examined GI and extraintestinal features associated with anti-M3R antibodies in SSc patients.

Methods: In a cohort enriched for GI symptoms, SSc patients were tested for anti-M3R antibodies using an enzyme-linked immunosorbent assay.

Systemic pharmacotherapy approaches for the treatment of systemic sclerosis.

Introduction: Systemic sclerosis (SSc) represents a complex, multisystem rheumatologic disorder characterized by immune dysregulation, vascular dysfunction, and multi-organ fibrosis. This review discusses the efficacy of the available therapeutic options and the significance of developing effective strategies against its varied manifestations, pivotal to improving patient outcomes.

Areas Covered: The review elaborates on the pharmacological treatments available for managing key manifestations of SSc, including skin and lung involvement, and vascular complications, as well as the most recent findings in the field.

An international perspective on the future of systemic sclerosis research.

Systemic sclerosis (SSc) remains a challenging and enigmatic systemic autoimmune disease, owing to its complex pathogenesis, clinical and molecular heterogeneity, and the lack of effective disease-modifying treatments. Despite a century of research in SSc, the interconnections among microvascular dysfunction, autoimmune phenomena and tissue fibrosis in SSc remain unclear. The absence of validated biomarkers and reliable animal models complicates diagnosis and treatment, contributing to high morbidity and mortality.

The role of prokinetics in managing gastrointestinal involvement in SSc: a systematic literature review.

Objectives: Gastrointestinal involvement (GI) in SSc is frequent and heterogeneous, manifesting with different degrees of dysmotility. This systematic literature review aimed to summarize evidence on prokinetics for treating SSc-related GI dysmotility.

Methods: Studies investigating the effects of prokinetic agents on GI function and/or GI symptoms in patients with SSc were systematically identified on PubMed and Embase.

Abnormal Esophageal Scintigraphy Associates With a Distinct Clinical Phenotype in Patients With Systemic Sclerosis.

Objective: In systemic sclerosis (SSc), absent contractility (AC) rather than ineffective esophageal motility on manometry is associated with a severe esophageal and extraintestinal phenotype. We sought to determine whether slow esophageal transit on scintigraphy associates with a comparable clinical phenotype to that of AC on manometry, as scintigraphy may serve as a noninvasive approach to risk-stratify patients with SSc.

Methods: Clinical, demographic, and serologic features were compared between patients with and without delayed esophageal transit on scintigraphy.

Evidence for targeting autonomic dysfunction in systemic sclerosis: A scoping review.

Autonomic dysfunction is a common and early complication among patients with systemic sclerosis, suggesting that it may play a role in the pathogenesis of the disease and be a potential target for therapeutic interventions. Although the true prevalence of autonomic dysfunction among patients with systemic sclerosis is still unclear, it is estimated that as many as 80% of patients may be affected. Autonomic dysfunction may lead to widespread multi-organ dysfunction through its effects on the cardiovascular system, gastrointestinal tract, urinary tract, sweat and salivary glands, and pupils.

Systemic sclerosis-related fecal incontinence: a scoping review focusing on a neglected manifestation.

Objectives: This scoping review sought to summarize the current knowledge on the epidemiology, pathogenesis and clinical presentation of, and the investigations that may help characterize faecal incontinence (FI) in patients with SSc.

Methods: The planned scoping review was based on the methodological framework proposed by Arksey and O'Malley. Two databases were screened: PubMed (Medline), (Web of Science), and data extraction was performed using a predefined template.

Anti-mitochondrial antibodies in systemic sclerosis target enteric neurons and are associated with GI dysmotility.

Background: Most patients with systemic sclerosis (SSc) experience gastrointestinal (GI) dysmotility. The enteric nervous system (ENS) regulates GI motility, and its dysfunction causes dysmotility. A subset of SSc patients harbor autoantibodies against the M2 mitochondrial antigen (AMA).

Colour changes in the feet: a sign of autonomic symptoms in systemic sclerosis.

Objectives: Patients with autonomic dysfunction, or dysautonomia, often report discolouration of their dependent extremities, which is thought to be from venous pooling or acrocyanosis. A subset of patients with SSc are affected by dysautonomia but may be challenging to identify. We sought to determine whether patients with SSc who report discolouration in their feet have a higher burden of autonomic symptoms, including orthostatic, gastrointestinal (GI), urinary, secretomotor and pupillomotor.

Understanding the gastrointestinal microbiome in systemic sclerosis: methodological advancements and emerging research.

Purpose Of Review: This review highlights the role of the gastrointestinal (GI) microbiome in systemic sclerosis (SSc). We describe techniques for evaluating the GI microbiome in humans, and emerging research linking GI microbiome alterations (i.e.

Evaluating the Associations Among Dysautonomia, Gastrointestinal Transit, and Clinical Phenotype in Patients With Systemic Sclerosis.

Objective: Our objective was to identify patients with systemic sclerosis (SSc) with a high burden of autonomic symptoms and to determine whether they have a distinct clinical phenotype, gastrointestinal (GI) transit, or extraintestinal features.

Methods: In a prospective cohort of patients with SSc with GI disease, clinical data were systematically obtained at routine visits. Dysautonomia was identified by the Composite Autonomic Symptom Score (COMPASS)-31questionnaire.

2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases.

Objective: We provide evidence-based recommendations regarding the treatment of interstitial lung disease (ILD) in adults with systemic autoimmune rheumatic diseases (SARDs).

Methods: We developed clinically relevant population, intervention, comparator, and outcomes questions. A systematic literature review was then performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology.