Effectiveness and safety of rituximab across the four phenotypes of IgG4-related disease: a European multi-center cohort study of 115 patients.

Objective: Assess relative effectiveness and safety of rituximab (RTX) across the four phenotypes of IgG4-related disease (IgG4-RD).

Methods: We included phenotypically defined adult IgG4-RD patients treated with RTX +/- glucocorticoids (GC) who had evaluable data at 6 months on the composite primary outcome, which included: (i) treatment response (>= 2-point decline in responder index (RI)); (ii) absence of flare; and (iii) GC dose <= 7.5 mg prednisolone.

Association of IgG4-related disease with human inborn errors of immunity.

Objectives: IgG4-related disease (IgG4-RD) is considered a complex multifactorial condition because immunological, environmental, and genetic factors contribute to its pathogenesis and protean clinical manifestations. While immunological and environmental factors have been extensively investigated, the contribution of genetic factors remains poorly understood. We sought to investigate a predisposing genetic background associated with IgG4-RD.

The complex diagnosis of primary heart involvement in systemic sclerosis: red flags to drive the choice of cardiac magnetic resonance.

Objectives: To analyse clinical features and biomarkers associated with primary heart involvement (pHI) confirmed at cardiac magnetic resonance (CMR) in a monocentric cohort of SSc patients.

Methods: A retrospective study. SSc patients who underwent CMR for clinically suspected pHI were identified.

Step-down treatment with mepolizumab for eosinophilic granulomatosis with polyangiitis: a real-life single-centre study.

Objectives: To evaluate the efficacy and safety of a step-down treatment approach using mepolizumab for eosinophilic granulomatosis with polyangiitis (EGPA) in a real-life single-centre cohort. The study aimed to assess outcomes following a transition from high-dose (300 mg/4 weeks) to low-dose (100 mg/4 weeks) mepolizumab after achieving remission.

Methods: This retrospective study included EGPA patients treated with mepolizumab between April 2014 and December 2024.

Immunosuppressive therapy to treat newly diagnosed primary heart involvement in patients with systemic sclerosis: An Italian cardiac magnetic resonance based study.

Background: Primary heart involvement (pHI) is frequent in systemic sclerosis (SSc), and is associated with a poor prognosis. Therapeutic strategies to treat SSc-pHI are not yet defined.

Objectives: To evaluate the efficacy of immunosuppressive therapy on cardiac magnetic resonance (CMR) features in patients with CMR-proven SSc-pHI.

Long-term retention rate, adverse event temporal patterns and rescue treatment strategies of mycophenolate mofetil in systemic sclerosis: insights from real-life.

Objectives: MMF is a mainstay for the treatment of SSc. The occurrence and implications of MMF-related adverse events (AEs) on drug retention rates in real life remain poorly defined. We aimed to determine the MMF retention rate and to investigate the causes and patterns of discontinuation, AEs and treatment options used after discontinuation.

Therapeutic strategies for primary heart involvement in systemic sclerosis.

Primary heart involvement (pHI) is frequent in systemic sclerosis (SSc), even though often underdiagnosed. SSc-pHI has been recently defined as cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications. SSc-pHI represents a major determinant of mortality in SSc, accounting alone for about 12% of disease-related deaths; its early recognition and promptly therapeutic interventions are therefore crucial.

Rituximab retention rate in systemic sclerosis: a long term real-life multicentre study.

Objectives: To report real-life data on rituximab retention rate as an indicator of safety and efficacy in a multicentric national cohort of systemic sclerosis patients.

Methods: SSc patients treated with rituximab and followed for at least 36 months were included, clinically characterized and longitudinally monitored. A competing risk analysis with sub-hazard ratio (sHR) definition was performed to explore the clinical variables linked to specific cause of rituximab discontinuation.

Effectiveness and safety of mycophenolate mofetil and rituximab combination therapy for immune idiopathic myopathies.

Introduction: Idiopathic inflammatory myopathies (IIM) represent a rare and heterogenous group diseases, and their treatment is not fully defined yet. According to previous small case series, the combination of mycophenolate mofetil (MMF) and rituximab (RTX) may be effective in controlling difficult-to-treat patients. Our aim was to further explore the efficacy and safety of this combined approach in patients with IIM.

Association of IgG4-related disease and systemic rheumatic disorders.

Purpose: Autoimmune disorders can occur together especially in genetically predisposed individuals. We here aimed to assess the occurrence of IgG4-related disease (IgG4-RD) in association with other systemic immune-mediated conditions.

Methods: We retrospectively analyzed the clinical records of patients with IgG4-RD followed at the IgG4-RD Clinic of San Raffaele Hospital (Milan, Italy) for pre-existing or concomitant immune-mediated disorders.

Is mild asthma truly mild? The patients' real-life setting.

Objectives: Asthma exacerbations and, more rarely, fatal asthma attacks have been reported in mild asthma patients, suggesting poor disease control and awareness of its potential burden. Our study aimed to explore outside the hospital/specialist setting the perspective and disease treatment behavior of patients self-reporting a mild asthma diagnosis.

Methods: Computer-Assisted Personal Interviewing (CAPI) technique was used to investigate the identified study population.

Cardiac Safety of mRNA-Based Vaccines in Patients with Systemic Lupus Erythematosus and Lupus-like Disorders with a History of Myocarditis.

Anti-severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines may trigger immune-mediated adverse events, including myocarditis. Evidence of vaccine safety in patients with rheumatic disorders and underlying autoimmune myocarditis is scarce. To address this issue, we studied 13 patients with systemic lupus erythematosus (SLE) and allied conditions with a history of myocarditis and receiving mRNA-based vaccines.

Clinical response to biologicals for severe asthma: any relevance for sex in different age ranges?

Background: Whether sex can influence the clinical response to biological treatment in patients with severe asthma has not been fully addressed.

Aims And Methods: The aim of this study was to investigate in patients with severe asthma undergoing biological treatment the individual evolution of lung function measurements and patient-reported asthma control scores over a 12-month follow-up period, in relation to patients' sex, in different age ranges. Second, the change in the administered dose of oral corticosteroids (OCS) before and after 12 months of treatment was investigated.

Relationship between hair shedding and systemic inflammation in COVID-19 pneumonia.

A higher risk for COVID-19 infection and severity for men compared to women has been described since the beginning of the pandemic. The role of androgens has been recently highlighted as they control two key steps of coronavirus infection mediated through the transmembrane protease serin 2 (TMPRRS2) and the angiotensin-converting enzyme 2 (ACE2) receptor in the lung tissue. Furthermore, a high incidence of androgenic alopecia among males with COVID-19 disease have been reported.

COVID-19 Vaccination in Patients with Severe Asthma on Biologic Treatment: Safety, Tolerability, and Impact on Disease Control.

Background: COVID-19 vaccination has been recommended for severe asthmatics. We aimed to evaluate the safety, tolerability, and impact on disease control and patient's quality of life of the mRNA SARS-CoV-2/COVID-19 vaccine in severe asthma patients regarding biologic treatment.

Methods: Severe asthmatic patients regularly managed by two big allergy and respiratory referral centers were offered to undergo Pfizer COVID 19 vaccination at the hospital site.

Oral health in asthmatic patients: a review : Asthma and its therapy may impact on oral health.

Different drugs used to treat asthma, such as beta 2 agonists and inhaled steroids, may promote a higher risk of caries, dental erosion, periodontal disease and oral candidiasis. This article reviews the evidences of mechanisms involved in oral diseases in patients affected by asthma. The main mechanism involved is the reduction of salivary flow.

Baricitinib restrains the immune dysregulation in patients with severe COVID-19.

BACKGROUNDPatients with coronavirus disease 2019 (COVID-19) develop pneumonia generally associated with lymphopenia and a severe inflammatory response due to uncontrolled cytokine release. These mediators are transcriptionally regulated by the JAK/STAT signaling pathways, which can be disabled by small molecules.METHODSWe treated a group of patients (n = 20) with baricitinib according to an off-label use of the drug.

Factors related to alexithymia in patients with systemic sclerosis: a tight relationship with facial image dissatisfaction.

To assess clinical and psychosocial factors related to alexithymia in systemic sclerosis (SSc). We enrolled 40 consecutive SSc patients in a cross-sectional study evaluating alexithymia with Toronto Alexithymia scale (TAS-20). We measured Beck Depression inventory (BDI), Hamilton Anxiety rating scale (HAM-H), 36-Items Short-Form Healthy Survey (SF-36), Functional Assessment of Chronic Illness Therapy (FACIT)-Fatigue, Visual Analog Scale (VAS) pain, Pittsburgh Sleep Quality Index (PSQI), Satisfaction with Appearance Scale (SWAP), and Mouth Handicap in Systemic Sclerosis (MHISS).

Belimumab and low-doses of mycophenolate mofetil as induction therapy of class IV lupus nephritis: case series and literature review.

Background: The treatment of Lupus Nephritis (LN) is an unmet need in the management of patients with Systemic Lupus Erythematosus (SLE). CASE PRESENTATION : We report two cases of women affected by Lupus Nephritis (LN) ISN/RNP Class IV with serological active disease, high disease activity and marked fatigue. In both cases, Mycophenolate mofetil (MMF), as induction therapy, was poorly tolerated because of gastrointestinal toxicity.

Physical activity and sedentary behavior in patients with Systemic Lupus Erythematosus.

Introduction: The aim of this study was to evaluate the proportion of patients with Systemic Lupus Erythematosus (SLE) who did not met the WHO recommendations for physical activity and to evaluate the amount of time spent in sedentary behavior.

Methods: SLE patients were consecutively enrolled in a cross sectional study. The type and the time spent in physical activity and sedentary behavior were evaluated using the IPAQ short form questionnaire.

The relation between, metabolic syndrome and quality of life in patients with Systemic Lupus Erythematosus.

Introduction: Systemic Lupus Erythematosus (SLE) is associated to an increased prevalence of Metabolic Syndrome (MeS) and to a reduction of Quality of Life (QoL). The aim of this study is to evaluate the association between MeS and QoL in SLE.

Methods: SLE patients were consecutively enrolled in a cross sectional study.