Digital ulcers are one of the earliest and most disabling manifestations of systemic sclerosis (SSc). We report the clinical case of a female patient with SSc and severe digital ulcers, recurrent and refractory to the classic treatments to whom it was prescribed off-label macitentan with complete resolution of the condition.
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http://dx.doi.org/10.1136/bcr-2018-228295 | DOI Listing |
Clin Rheumatol
January 2025
Rheumatology Unit, Scleroderma Unit, University Hospital of Modena, Via del Pozzo, 71-41125, Modena, Italy.
The aims of this study were to investigate the prevalence of cryofibrinogenemia in a cohort of patients with systemic sclerosis (SSc) regardless of clinical manifestations, who were admitted to our hospital and determine the associations among CF positivity, disease features and ongoing therapies. This was a monocentric and retrospective study. The inclusion criteria were a diagnosis of SSc (according to the ACR/EULAR 2013 classification criteria), regular administration of i.
View Article and Find Full Text PDFJ Rheumatol
January 2025
Florenzo Iannone, Rheumatology Unit - Department of Precision and Regenerative Medicine of Jonian Area University of Bari, Bari, Italy.
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 the SPRING registry.
Methods: Patients with SSc from the SPRING registry, meeting ACR/EULAR 2013 classification criteria with data on PAH onset, DU status, BOS exposure, and at least a one-year follow-up between 2015 and 2020, and no known PAH at baseline were included.
Clin Rheumatol
January 2025
Department of Rheumatology, Huashan Hospital, Fudan University, No.12 Wulumuqi Zhong Road, Shanghai, 200040, China.
To evaluate the association of anti-IFI16 antibodies with peripheral vasculopathy and the predictive value of anti-IFI16 antibodies for the development or persistence of digital ulcers (DPDU) in SSc. A total of 42 SSc patients and 42 age- and sex-matched healthy controls were enrolled. Anti-IFI16 antibodies were examined by ELISA.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Biomedical Sciences, University of Sassari, Sassari, Italy.
Introduction: The critical role played by vascular dysfunction and ineffective angiogenesis in the pathophysiology of systemic sclerosis (SSc) suggests that circulating biomarkers reflecting these alterations may be useful in the clinical evaluation of this patient group. We sought to address this issue by conducting a systematic review and meta-analysis of studies investigating a such candidate biomarker, endostatin, an endogenous glycoprotein exerting anti-angiogenic effects, in SSc patients and healthy controls.
Methods: A literature search was conducted in the electronic databases Web of Science, PubMed, and Scopus from inception to 27 May 2024.
Cureus
December 2024
Department of Rheumatology, Mayo Clinic, Jacksonville, USA.
The term Raynaud's phenomenon (RP) is used to describe complex symptoms related to vascular compromise, which are typically exacerbated by cold-induced vasoconstriction, emotional stress, or other sympathomimetic factors. In almost all patients with limited cutaneous systemic sclerosis (SSc), the first symptom is RP, often two to five years before any other symptom of scleroderma. The clinical course and severity of this disease are variable and highly fatal in some individuals, which has led to the development of strategies for timely diagnosis; hence, criteria for the very early diagnosis of systemic sclerosis have been established.
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