Efficacy and safety of rituximab and low dosage of glucocorticoids for ANCA associated vasculitis interstitial lung disease: a proof-of-concept study.

Introduction: The prevalence of interstitial lung disease (ILD) in ANCA associated vasculitis (AAV) ranges from 13 % to 45 %, and both its definition and management represent a quandary. The occurrence of lung fibrosis and ANCA positivity, particularly in subjects without any other evidence of vasculitis, is far from being clearly interpreted, as well as its proper therapeutic management. In this regard, aim of this study is to assess whether Rituximab (RTX) in association with a low dosage of glucocorticoids (GCs), could be effective also in the treatment of AAV-ILD.

Predictors of uveitic macular edema and functional prognostic outcomes: real-life data from the international AIDA Network uveitis registry.

Objectives: To detect factors capable of predicting the development of macular edema (ME) throughout the disease course in patients affected by non-infectious uveitis (NIU).

Methods: Predictive factors leading to the development of ME were analyzed through regression analysis. The functional impact of ME on best corrected visual acuity (BCVA) was also examined.

Type II diabetes in systemic sclerosis patients: insights from an observational, multicenter study of GIRRCS (Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale).

Objective: To assess the contribution of Systemic sclerosis (SSc)-specific features on type II diabetes mellitus (T2D) in a large cohort of Italian SSc patients.

Methods: A total of 613 SSc patients from 11 tertiary Rheumatology Units across Italy were included. All patients underwent full history taking, clinical examination, and relevant laboratory and radiological evaluations.

Managing the clinical heterogeneity of patients with Still's disease, from early diagnosis to timely treatment.

Still's disease is an inflammatory syndrome affecting patients across all ages, previously known as systemic juvenile idiopathic arthritis (sJIA) in children and adult-onset Still's disease (AOSD) in adults. Multiple lines of evidence reported overlapping clinical features between sJIA and AOSD, commonly manifesting with daily fever, arthritis, evanescent salmon-coloured skin rash. The concomitant various degree of multiorgan involvement may increase the heterogeneity of the patient clinical picture.

Serum ferritin as a specific biomarker of anti-MDA5-interstitial lung disease: a multicenter, case-control study in observational and validation datasets.

Background: Serum ferritin has been proposed as biomarker of interstitial lung disease (ILD) in anti-MDA5 dermatomyositis (DM). Nevertheless, no data exist for serum ferritin in other idiopathic inflammatory myopathies (IIM)-ILD nor in IIM without ILD. Aim of this case-control study was to assess whether serum ferritin may be considered a specific and sensitive biomarker for IIM-ILD, as well as to assess whether it correlates with clinical and immunological findings.

Tocilizumab effectiveness in paediatric non-infectious uveitis: data from the International AIDA Network Registries on ocular inflammatory disorders.

To describe tocilizumab (TCZ) effectiveness in 15 children with refractory non-infectious uveitis. Reported outcomes are the number of relapses before and after treatment, steroid-sparing effect and drug retention rate. Macular oedema, fluorangiographic findings and ocular complications are also reported.

Long-term efficacy and safety of colchicine and anti-IL-1 blockers in FMF: results from the Eurofever multicenter observational study.

Introduction: The majority of currently available data on familial Mediterranean fever (FMF) come from retrospective national or international studies.

Methods: An observational study collected data on the Eurofever international FMF cohort. Patients fulfilling genetic and clinical Eurofever criteria were considered as FMF+.

Adalimumab Monotherapy or Combination Therapy With Methotrexate in Paediatric Uveitis: Data From the AIDA Network Uveitis Registry.

Background: The study objective was to compare the effectiveness of adalimumab (ADA) in monotherapy and in combination with methotrexate (MTX) for paediatric noninfectious uveitis (NIU).

Methods: Registry-based observational study. Children receiving ADA for active uveitis were divided into the ADA monotherapy group (group 1) and the ADA plus MTX combination group (group 2).

Predictors of proteinuria, amyloidosis and kidney failure in familial Mediterranean fever: data from the International AIDA Network Registry.

Objectives: Proteinuria, amyloidosis and kidney failure are the main long-term renal complications of FMF. This study assesses their risk factors, independent of ethnicity or residence.

Methods: Patients' data were drawn from the International AIDA Network registry for monogenic autoinflammatory diseases.

Efficacy and safety of conventional disease-modifying antirheumatic drugs in VEXAS syndrome: real-world data from the international AIDA network.

Background: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset autoinflammatory condition resulting in severe, often treatment-refractory inflammation. Currently, there are no established treatment guidelines for VEXAS syndrome.

Objectives: To assess the efficacy and safety of conventional disease-modifying antirheumatic drugs (cDMARDs) in a cohort of VEXAS patients.

Efficacy and safety profile of biotechnological agents and Janus kinase inhibitors in VEXAS syndrome: data from the international AIDA Network VEXAS registry.

Background: VEXAS syndrome, a recently identified systemic autoinflammatory disorder, poses new diagnostic and management challenges. Based on experience with other autoinflammatory diseases, anti-interleukin (IL)-1, anti-IL-6, anti-tumor necrosis factor (TNF) biotechnological agents, and Janus kinase inhibitors (JAKis) have been widely employed in VEXAS patients. The aim of this study is to evaluate the global effectiveness and safety of biotechnological agents and JAKis using data from the real-world context.

Development and implementation of the international AIDA network spondylarthritis registry.

During the last decade, spondyloarthritis (SpA) has increasingly been considered a disease at the crossroads between autoimmunity and autoinflammation. Some patients may even present with autoinflammatory-related manifestations, including fever, hidradenitis suppurativa, other neutrophilic dermatoses, and an unusually high increase in inflammatory markers. Therefore, a subgroup of SpA patients may be identified, and specific details about this cluster need to be investigated.

The clinical assessment of lung involvement in patients with Still's disease, results from the multicentre international AIDA Network Still's Disease Registry.

Objectives: To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.

Methods: A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry.

Small molecules in idiopathic inflammatory myopathies: a systematic review and a multicenter case series about Janus kinase inhibitors and apremilast.

Objective: Idiopathic inflammatory myopathies (IIM) are rare autoimmune diseases that primarily affect striated muscles; skin, joints, and lungs may be involved with different degrees of severity. Traditional treatment relies on high-dose glucocorticoids and conventional synthetic disease-modifying antirheumatic drugs.

Methods: A growing amount of evidence is demonstrating the potential role of novel treatments in the management of IIM.

Evaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry.

Objective: We aimed to (1) evaluate the cardiac involvement, with a focus on myocarditis, in patients with Still disease included in the multicenter Autoinflammatory Disease Alliance (AIDA) Network Still disease registry; and (2) assess the predictive factors for myocarditis by deriving a clinical risk patient profile for this severe manifestation.

Methods: A multicenter observational study was established, in which consecutive patients with Still disease in the AIDA Network Still disease registry were characterized by cardiac involvement. Cardiac involvement was defined according to the presence of pericarditis, tamponade, myocarditis, and/or aseptic endocarditis.

Methodology and clinical utility of longitudinal UBA1 tracking in VEXAS syndrome.

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) is a haemato-inflammatory syndrome genetically defined by somatic mutations in the X-linked UBA1 gene, typically Val/Thr/Leu substitutions at the Met41 hotspot. Clinical manifestations are heterogeneous and refractory to most haemato-rheumatological treatments. To date, no guidelines exist for the management of VEXAS, and scarce is the evidence on methodology and clinical significance of longitudinal UBA1 clonal burden evaluation upon therapy.

Efficacy and safety of infliximab or adalimumab in severe mucocutaneous Behçet's syndrome refractory to traditional immunosuppressants: a 6-month, multicentre, randomised controlled, prospective, parallel group, single-blind trial.

Introduction: Evidence from randomised controlled trials on anti-tumour necrosis factor (TNF) agents in patients with Behçet's syndrome (BS) is low.

Method: We conducted a phase 3, multicentre, prospective, randomised, active-controlled, parallel-group study to evaluate the efficacy and safety of either infliximab (IFX) or adalimumab (ADA) in patients with BS. Adults patients with BS presenting with active mucocutaneous manifestations, occurring while on therapy with either azathioprine or cyclosporine for at least 3 months prior to study entry, were eligible.