Licia Vultaggio

The clinical profile of systemic sclerosis without a ‘scleroderma pattern’ at nailfold capillaroscopy: results from the multicenter SPRING registry of the Italian Society of Rheumatology

Antonio Tonutti, Maria De Santis, Francesca Motta, Gianluigi Bajocchi et autres

Background: Microvascular alterations can be detected with nailfold videocapillaroscopy, useful for systemic sclerosis (SSc) diagnosis upon identification of the "scleroderma pattern" (NVC-SP). However, this pattern is missing in a subset of SSc patients. Methods: This retrospective analysis on the multicenter Italian SPRING …

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Clinical and serological features of triple autoantibody-negative patients with systemic sclerosis: insights from the multicentric SPRING registry of the Italian Society for Rheumatology

Veronica Batani, Ilaria Cavazzana, Martina Orlandi, Rossella De Angelis et autres

BACKGROUND: Antitopoisomerase I (ATA), anticentromere (ACA) and anti-RNA polymerase III (RNAP3) antibodies are included in the 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for systemic sclerosis (SSc). A subset of patients with SSc satisfy criteria but may …

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Travel distance to tertiary healthcare reflects disease severity and medication access in Italian Systemic Sclerosis patients: a geographical study on adult-onset rare diseases

Enrico Lorenzis, Gerlando Natalello, Rossella De Angelis, Lucrezia Verardi et autres

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Age and onset timing of Raynaud’s phenomenon and first non-Raynaud symptom as prognostic factors in systemic sclerosis: a retrospective analysis from the Italian national multicenter Systemic Sclerosis Progression INvestiGation registry of the Italian Society for Rheumatology (SPRING-SIR)

Silvia Peretti, Cosimo Bruni, Francesco Bonomi, Rossella De Angelis et autres

Background: The sequence and temporal relationship between Raynaud's phenomenon (RP) and the first non-Raynaud's sign/symptom (NRP) in systemic sclerosis (SSc) have been partially investigated. Objectives: To evaluate whether the mode and ages of clinical onset are associated with disease endotype and survival …

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Mycophenolate Mofetil Treatment Reduces the Risk of Treatment Escalation Due to Vascular Complications in Limited Cutaneous Systemic Sclerosis: Emulation of a Target Trial From the Italian Rheumatology Society SPRING Registry

Enrico De Lorenzis, Gerlando Natalello, Rossella De Angelis, L. Verardi et autres

OBJECTIVE: Mycophenolate mofetil (MMF) use in limited cutaneous systemic sclerosis (lcSSc) is relatively uncommon because of the lower fibrotic burden and the predominance of vascular complications. In vitro observations and clinical data from transplanted patients suggest a protective effect of MMF on …

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Prevalence, distribution and associations of the scleroderma capillaroscopic patterns: new insights from the Italian SPRING-SIR registry

Rossella De Angelis, Clodoveo Ferri, Edoardo Cipolletta, Valeria Riccieri et autres

OBJECTIVES: To assess the relationship between disease duration and the prevalence/distribution of nailfold videocapillaroscopy (NVC) patterns, named according to the current classification as 'early', 'active' and 'late', in a large cohort of systemic sclerosis (SSc) patients. METHODS: A cross-sectional analysis was conducted …

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Elderly-onset systemic sclerosis defines a distinct clinical subset: analysis from the SPRING registry of the Italian Society for Rheumatology

Antonio Tonutti, Francesca Motta, Gianluigi Bajocchi, Silvia Bellando-Randone et autres

OBJECTIVE: Elderly-onset systemic sclerosis (SSc) is relatively uncommon, and its clinical phenotype and prognostic implications remain poorly characterized, with conflicting evidence regarding disease course and outcomes. METHODS: Within the Italian SPRING (Systemic Sclerosis PRogression INvestiGation) registry, we compared demographic and clinical characteristics …

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Gastrointestinal involvement in very early and established systemic sclerosis: insights from the SPRING-SIR national Italian registry

Francesco Bonomi, Cosimo Bruni, Silvia Peretti, Rossella De Angelis et autres

OBJECTIVES: To describe the prevalence of gastrointestinal (GI) symptoms in SSc and Very Early Diagnosis of SSc (VEDOSS), identify clinical and serological features associated with GI involvement and explore a cranio-caudal pattern of symptom distribution, using data from the Italian SPRING-SIR registry. …

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First‐line diagnostic tests to intercept primary heart involvement in systemic sclerosis: Clinical associations from the SPRING‐SIR registry

Antonio Tonutti, Francesca Motta, Rossella De Angelis, Edoardo Cipolletta et autres

INTRODUCTION: Primary heart involvement (pHI) is an overlooked and poorly characterised complication of systemic sclerosis (SSc), associated with the risk of heart failure, arrhythmia and death. Despite consensus definition by the World Scleroderma Foundation/Heart Failure Association (WSF/HFA), diagnostic criteria and risk factors …

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Menopause in systemic sclerosis: the impact on clinical presentation in a multicenter cross-sectional analysis from the National Registry of the Italian Society for Rheumatology (SPRING-SIR)

Martina Orlandi, Dilia Giuggioli, Clodoveo Ferri, Rossella De Angelis et autres

Background: Hormonal changes in menopause might interact with the presentation of underlying autoimmune diseases, such as systemic sclerosis (SSc). Objectives: Our study aimed to evaluate the association of (1) current menopausal status, (2) early menopause, and (3) disease onset during fertile or …

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Prevalence and clinical relevance of digital ulcers in systemic sclerosis patients from the real-life: the experience of the SPRING Registry of the Italian Society for Rheumatology

Martina Orlandi, Giacomo De Luca, Clodoveo Ferri, Amelia Spinella et autres

INTRODUCTION: Digital ulcers (DU) are one of the most frequent manifestations in systemic sclerosis (SSc). The presence of DU seems to be a sentinel sign of internal organ involvement and is related to a poor prognosis of the disease. The aim of …

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Pulmonary Arterial Hypertension Incidence in Patients With Systemic Sclerosis Treated With Bosentan for Digital Ulcers: Evidence From the SPRING-SIR Registry

Fabio Cacciapaglia, Rossella De Angelis, Clodoveo Ferri, Gianluigi Bajocchi et autres

OBJECTIVE: Bosentan (BOS) is approved for treating pulmonary arterial hypertension (PAH) and preventing digital ulcers (DU) in systemic sclerosis (SSc). Our study aimed to evaluate whether BOS prescribed for DU could reduce the incidence of PAH in a large SSc cohort from …

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