Validation of the Italian version of the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) questionnaire.

Objectives: The primary objective of this study was the translation and validation of the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) questionnaire into Italian, denoted as AAV-PRO_ita. The secondary objective was to evaluate the impact of ANCA-associated vasculitis (AAV) on quality of life (QoL) and work impairment in a large cohort of Italian patients.

Methods: The study design took a prospective cohort study approach.

Clinical and histological features of patients with primary Sjögren's syndrome and autoimmune thyroiditis: a national multicentre cross-sectional study.

Objectives: Primary Sjögren's syndrome (pSS) is frequently associated with autoimmune thyroiditis (AT). The aim of this study was to evaluate the prevalence of AT in a national cohort of pSS and to describe the clinical and histological phenotype of patients with pSS and associated AT.

Methods: In this multicentre cross-sectional study, data from 2546 pSS were collected and the presence of AT was reported.

Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study.

Background: What baseline predictors would be involved in mortality in people with primary Sjögren syndrome (SjS) remains uncertain. This study aimed to investigate the baseline characteristics collected at the time of diagnosis of SjS associated with mortality and to identify mortality risk factors for all-cause death and deaths related to systemic SjS activity measured by the ESSDAI score.

Methods: In this international, real-world, retrospective, cohort study, we retrospectively collected data from 27 countries on mortality and causes of death from the Big Data Sjögren Registry.

Multicentre case-control study evaluating the safety of anti-SARS-CoV-2 vaccines in a cohort of patients with systemic vasculitis.

Objectives: Data on the safety of anti-SARS-CoV-2 vaccines in patients with rare rheumatic diseases, such as systemic vasculitis (SV), are limited. The aim of this study was to evaluate the occurrence of a disease flare and the appearance of adverse events (AEs) following administration of anti-SARS-CoV-2 vaccine in a multicentre cohort of patients with SV.

Methods: Patients with SV and healthy controls (HC) from two different Italian rheumatology centres were asked to complete a questionnaire assessing disease flares occurrence, defined as new onset of clinical manifestations related to vasculitis needing an implementation of therapy, and local/systemic AEs appearance following anti SARS-CoV-2 vaccination.

Application of Machine Learning Models in Systemic Lupus Erythematosus.

Systemic Lupus Erythematosus (SLE) is a systemic autoimmune disease and is extremely heterogeneous in terms of immunological features and clinical manifestations. This complexity could result in a delay in the diagnosis and treatment introduction, with impacts on long-term outcomes. In this view, the application of innovative tools, such as machine learning models (MLMs), could be useful.

Resilience in women with primary Sjögren's syndrome.

To assess the relationship between resilience and several diseases and individual features in primary Sjӧgren's Syndrome (SS) patients. Resilience was assessed using the Resilience Scale (RS-14). Disease activity, damage, and reported symptoms were assessed by means of ESSDAI (EULAR Sjögren's syndrome disease activity index), SSDDI (Sjӧgren's Syndrome Disease Damage Index) and ESSPRI (EULAR Sjӧgren's Syndrome Patient Reported Index).

Peripheral Nervous System Involvement in Sjögren's Syndrome: Analysis of a Cohort From the Italian Research Group on Sjögren's Syndrome.

Purpose: The prevalence of peripheral nervous system (PNS) involvement in primary Sjögren's syndrome (pSS) has been reported to range from 2% to over 50%. Bias in study designs, including low number of patients and unclearly defined rheumatological and neurological diagnosis could explain such variability. Consequently, the exact depiction of PNS involvement in pSS is still lacking.

Childhood-onset of primary Sjögren's syndrome: phenotypic characterization at diagnosis of 158 children.

Objectives: To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS.

Methods: The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria.

Salivary Gland Ultrasonography in Sjögren's Syndrome: A European Multicenter Reliability Exercise for the HarmonicSS Project.

Salivary gland ultrasonography (SGUS) is increasingly applied for the management of primary Sjögren's syndrome (pSS). This study aims to: (i) compare the reliability between two SGUS scores; (ii) test the reliability among sonographers with different levels of experience. In the reliability exercise, two four-grade semi-quantitative SGUS scoring systems, namely De Vita et al.

Multicolor flow cytometric analysis of TLR2 and TLR9 expression and function in NK cells from patients with ANCA-associated vasculitis.

Background: The primary objective of this study was to provide an assessment of NK cells in patients with ANCA-associated vasculitis (AAV).

Methods: Patients were classified based on the presence or absence of ANCAs and compared with healthy controls (HCs). By multiparameter flow cytometry, we evaluated the number and proportion of NK cells (CD3-CD56+) and the CD56 , CD56 , CD56 CD57 subsets; TLR2 and TLR9 expression; intracellular IFN-γ production upon stimulation with TLR2 and TLR9 ligands; degranulation activity; serum cytokines; immunohistochemical staining of available biopsies.

Myositis in primary Sjögren's syndrome: data from a multicentre cohort.

Objectives: In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequent while myositis has been reported in 3% of patients. The aim of this study was to describe the prevalence of myositis in a multicentre Italian pSS cohort and to address the clinical manifestations, histological findings and therapeutic strategies.

Methods: Clinical, serological and therapeutic data from a pSS cohort of patients were retrospectively collected.

The use of musculoskeletal ultrasound in a rheumatology outpatient clinic.

Introduction: Musculoskeletal ultrasound (US) represents a valid, reliable and sensitive-to-change tool for the evaluation of patients suffering from rheumatic conditions. This method demonstrates a wide applicability and availability, finding place in the clinical practice in rheumatology outpatient clinic.

Aim: To perform an epidemiological evaluation related to the use of US in a university rheumatology outpatient clinic.

Primary Sjogren's syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients.

Objective: The aims of this study were to describe the clinical presentation of primary SS (pSS) in a large cohort of patients by assessing the prevalence of the patient subgroups at high risk for severe extraglandular manifestations and to explore the influence of the patients' serological profile on disease severity and on immunosuppressive drug utilization.

Methods: Cumulative demographic, clinical, serological, histological and therapeutic data of 1115 pSS patients were retrospectively evaluated. Independent serological markers for glandular and extraglandular disease manifestations were identified by logistic regression.

The Italian MSUS Study Group recommendations for the format and content of the report and documentation in musculoskeletal ultrasonography in rheumatology.

Objective: The objective of this study was to draw up a set of recommendations for the format and content of the musculoskeletal ultrasonography (MSUS) report in rheumatology.

Methods: A panel of rheumatologists, members of the MSUS Study Group of the Italian Society of Rheumatology, met in order to identify the main discrepancies in the MSUS report. A set of 15 recommendations was then defined, aimed at resolving the main discrepancies.

Ultrasound in rheumatoid arthritis.

Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by synovial inflammation that can lead to structural damage of cartilage, bone and tendons. Assessing the inflammatory activity and the severity is essential in RA to help rheumatologists in adopting proper therapeutic strategies and in evaluating disease outcome and response to treatment. In the last years musculoskeletal (MS) ultrasonography (US) underwent tremendous technological development of equipment with increased sensitivity in detecting a wide set of joint and soft tissues abnormalities.

Outcome of pregnancy in Italian patients with primary Sjögren syndrome.

Objective: To investigate pregnancy and fetal outcomes in patients with primary Sjögren syndrome (pSS).

Methods: An obstetric history of 36 women with established diagnosis of pSS at pregnancy was obtained from a multicenter cohort of 1075 patients. In a subgroup case-control analysis, 12 deliveries in patients with pSS were compared with 96 control deliveries.

Myopathy complicating lupus pregnancy.

Systemic lupus erythematosus was diagnosed in a 34-year-old pregnant woman because of leukopenia, typical skin rash, clinical and biochemical signs of muscle involvement, and positive serology (antinuclear antibodies and anti-double-stranded DNA). Corticosteroids and hydroxychloroquine (HCQ) were started at 18 weeks of gestation with overall benefit except for muscle involvement, which proved resistant even to higher dose of corticosteroids and high-dose intravenous immunoglobulin. Muscle biopsy showed signs of HCQ toxicity, and the drug was stopped.

Osteoarthritis of the foot: a review of the current state of knowledge.

Foot osteoarthritis(OA)is very common disease that mainly affects older people deeply influencing their quality of life.The join most frequently involved in the articular degenerative process is the first metatarsophalangeal joint. Its severe impairment may lead to a specific clinical pattern known as hallux rigidus that often requires surgical treatment.

Fatigue and widespread pain in systemic lupus erythematosus and Sjögren's syndrome: symptoms of the inflammatory disease or associated fibromyalgia?

Fatigue and generalised pain are debilitating symptoms that negatively impact the quality of life in patients with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS). Chronic widespread musculoskeletal pain and fatigue are the clinical hallmarks of fibromyalgia (FM), a clinical entity which can be associated to connective tissue disease. The aim of the present study was to assess the prevalence of FM syndrome, fatigue and widespread pain in SLE and pSS patients and to evaluate the contribution of inflammatory disease and FM on those constitutional symptoms.

Unilateral painful, swollen and erythematosus knee. Case report.

Musculoskeletal ultrasound (US) is gaining an increasing role in the assessment of a variegate set of joint and periarticular soft tissues abnormalities in rheumatology. In addition, US represents a useful technique in guiding local procedures such as aspiration of fluid collections and injections within the joints and periarticular musculoskeletal structures. We recently performed US in a patient with unilateral painful, swollen and erythematosus knee.