Evaluation of non-invasive low-risk criteria in patients with connective tissue disease-associated pulmonary arterial hypertension: A 12-months analysis from the INSPECTIO study.

Background: Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) is a progressive, high-risk subtype of PAH characterized by immune-mediated vascular remodeling, poor treatment response, and reduced survival. Real-world data on therapeutic response and risk evolution in this population remain limited.

Methods: This post hoc analysis of the multicenter, prospective INSPECTIO study evaluated the CTD-PAH subpopulation treated with macitentan and/or selexipag.

Sex differences in pulmonary arterial hypertension: Insights from the FOCUS-PAH registry.

Background: Despite the higher incidence of pulmonary arterial hypertension (PAH) in women, sex differences in terms of clinical presentation and prognosis are still uncertain. We assess the impact of sex in patients with PAH.

Methods: Sex differences were evaluated in the multicenter FOCUS-PAH registry, that included patients diagnosed with PAH at 7 tertiary care centers between 2001 and 2022.

The Italian Society for Rheumatology guidelines on reproductive health in patients with rheumatic diseases.

Objective: To date, there is no shared national guideline in Italy for the management of reproductive health in rheumatic diseases (RHRD). The Italian Society for Rheumatology (SIR) has committed to developing clinical practice recommendations to provide guidance on both management and treatment regarding RHRD in Italy.

Methods: Using the GRADE-ADOLOPMENT methodology, a systematic literature review was conducted to update the scientific evidence that emerged after the publication of the reference recommendations from the American College of Rheumatology.

Impact of an expert-derived, quick hands-on tool on classifying pulmonary hypertension in chest computed tomography: a study on inexperienced readers using RAPID-CT-PH.

Purpose: To test the inter-reader agreement in classifying pulmonary hypertension (PH) on chest contrast-enhanced computed tomography (CECT) between a consensus of two cardio-pulmonary-devoted radiologists (CRc) and inexperienced readers (radiology residents, RRs) when using a CECT-based quick hands-on tool built upon PH imaging literature, i.e., the "Rapid Access and Practical Information Digest on Computed Tomography for PH-RAPID-CT-PH".

Phenotype and outcomes according to loop diuretic use in pulmonary arterial hypertension.

Aims: The use of loop diuretics in pulmonary arterial hypertension (PAH) is less frequent compared with heart failure. The clinical and prognostic characteristics of PAH patients according to loop diuretic use remain unexplored. In this study, we retrospectively analysed the characteristics and survival of PAH patients requiring different doses of loop diuretics.

Bosentan Does Not Affect Renal Resistive Index in Scleroderma/Systemic Sclerosis Patients.

Introduction: If properly evaluated, chronic kidney disease can be found in up to 50% of patients with systemic sclerosis (SSc). The renal resistive index (RRI) is a marker of intrarenal vascular resistance and can predict SSc-associated vasculopathy. This study aimed to determine the impact of bosentan, a nonselective endothelin-1 receptor antagonist, on RRI and kidney function in SSc patients with recurrent digital ulcers.

Performance of risk stratification scores and role of comorbidities in older vs younger patients with pulmonary arterial hypertension.

Background: Risk scores are important tools for the prognostic stratification of pulmonary arterial hypertension (PAH). Their performance and the additional impact of comorbidities across age groups is unknown.

Methods: Patients with PAH enrolled from 2001 to 2021 were divided in ≥65 years old vs <65 years old patients.

Structured report improves radiology residents' performance in reporting chest high-resolution computed tomography: a study in patients with connective tissue disease.

PURPOSE To evaluate the performance of radiology residents (RRs) when using a dedicated structured report (SR) template for chest HRCT in patients with suspected connective tissue disease-interstitial lung disease (CTD-ILD), compared to the traditional narrative report (NR). METHODS We retrospectively evaluated 50 HRCT exams in patients with suspected CTD-ILD. A chest-devoted radiologist reported all the HRCT exams as the reference standard, pointing out pulmonary fibrosis findings (i.

Leflunomide-induced pulmonary arterial hypertension: Case report and review of literature.

Unlabelled: Leflunomide, an isoxazole derivative, is a disease-modifying antirheumatic drug, that has successfully been used for the treatment of rheumatoid arthritis and psoriatic arthritis as a feasible alternative to methotrexate. Among side effects, pulmonary arterial hypertension (PAH) has been described in a few case reports.We present a 55-year-old woman treated with leflunomide for psoriatic spondyloarthritis who consulted our hospital because of progressive exertional dyspnea.

Chest high-resolution computed tomography in patients with connective tissue disease: pulmonary conditions beyond "the usual suspects".

The term "connective tissue diseases" (CTDs) refers to a heterogeneous group of autoimmune disorders, including systemic sclerosis, rheumatoid arthritis, Sjögren's syndrome, systemic lupus erythematosus, polymyositis, dermatomyositis, antisynthetase syndrome, and mixed connective tissue disease. Chest high-resolution computed tomography (HRCT) is the imaging method of choice for evaluating patients with known or suspected CTD-related interstitial lung disease (CTD-ILD), a complication accounting for substantial morbidity and mortality. While specific HRCT patterns and signs of CTD-ILD have been extensively described (hence the designation "the usual suspects"), the knowledge of various, less frequent conditions involving the lungs in patients with CTD would help the radiologist produce a clinically valuable report, thus potentially influencing patient management.

First Report of the Italian Registry on Immune-Mediated Congenital Heart Block (Lu.Ne Registry).

Neonatal Lupus (NL) is a rare syndrome caused by placental transfer of maternal anti-SSA/Ro and anti-La/SSB autoantibodies to the fetus. The rarity of this condition requires the establishment of multidisciplinary registries in order to improve our knowledge. Inclusion criteria in this retrospective study were the maternal confirmed positivity for anti-SSA/Ro and/or anti-SSB/La antibodies, and the presence of II or III degree congenital heart block (CHB) or neonatal period (up to 27 days after birth).

Pregnancy and foetal outcomes following anti-tumor necrosis factor alpha therapy: A prospective multicentre study.

Objective: As many inflammatory rheumatic diseases affect patients in childbearing age, some concern has been expressed about the safety of biologic drugs during pregnancy. This study evaluated the effects of anti-tumor necrosis factor alpha (TNFα) agents on pregnancy/foetal outcomes.

Methods: Thirty-eight pregnancies were followed prospectively from November 2008 to February 2015.

Treatment strategies and pregnancy outcomes in antiphospholipid syndrome patients with thrombosis and triple antiphospholipid positivity. A European multicentre retrospective study.

Previous thrombosis, diagnosis of systemic lupus erythematosus (SLE) and triple antiphospholipid (aPL) antibody positivity have recently been found to be independent factors associated to pregnancy failure during conventional therapy in women with antiphospholipid syndrome (APS). This study aimed to assess the effect of various treatment strategies on pregnancy outcomes in women with APS and the risk factors for pregnancy failure. One hundred ninety-six pregnancies of 156 patients diagnosed with APS were analysed: 118 (60.

Brief report: successful pregnancies but a higher risk of preterm births in patients with systemic sclerosis: an Italian multicenter study.

Objective: To assess fetal and maternal outcomes in women with systemic sclerosis (SSc).

Methods: Prospectively collected data on 99 women with SSc from 25 Italian centers were analyzed retrospectively. Women with SSc were observed during 109 pregnancies (from 2000 to 2011), and outcomes were compared to those in the general obstetric population (total of 3,939 deliveries).

Mononuclear cell response to lipopolysaccharide in patients with rheumatoid arthritis: relationship with tristetraprolin expression.

Objective: To analyze tumor necrosis factor-alpha (TNF-alpha) synthesis by mononuclear cells stimulated with lipopolysaccharide (LPS) in patients with rheumatoid arthritis (RA).

Methods: TNF-alpha molecular expression and extracellular release were assessed in the peripheral blood mononuclear cells (PBMC) of 27 RA patients and 16 healthy blood donor controls during 8 hours of LPS stimulation. We also analyzed the mRNA expression of tristetraprolin (TTP), the major TNF-alpha mRNA destabilizing factor.

Tumor necrosis factor-alpha receptor II polymorphism in patients from southern Europe with mild-moderate and severe rheumatoid arthritis.

Objective: To define the frequency of the exon 6 tumor necrosis factor-alpha (TNF-alpha) receptor II (TNFRII) gene polymorphism in severe and mild-moderate rheumatoid arthritis (RA) and its possible influence on anti-TNF-alpha treatment responsiveness.

Methods: Two cohorts of patients with RA, the first (n = 97) defined as methotrexate responders (MTX-R) with mild-moderate synovitis, and the second (n = 78) defined as nonresponders to combination therapy and receiving anti-TNF-alpha treatment because of their severe and aggressive disease (TNF-T), were studied retrospectively and compared to age, sex, and ethnically matched controls (n = 84). In the prospective study, 66 patients with severe RA were followed over the first 6 months of anti-TNF-alpha therapy and their response was examined according to genotype.

Tumor necrosis factor-alpha gene polymorphism in severe and mild-moderate rheumatoid arthritis.

Objective: To examine whether severe rheumatoid arthritis (RA) carries a -238 or +489 tumor necrosis factor-alpha (TNF-alpha) genotype different from mild-moderate RA.

Methods: We investigated 163 patients (66 with severe disease) and 67 healthy blood donor controls. Genotyping was performed by polymerase chain reaction-restriction fragment length polymorphism.