Systemic lupus erythematosus (SLE) is a complex autoimmune disease influenced by genetic, environmental, and immunological factors. Variations in cytokine genes, including tumor necrosis factor α (TNF-α) and interleukin-10 (IL-10), have been implicated in SLE pathogenesis, but their associations remain uncertain owing to conflicting study results.

Methotrexate (MTX) is a chemotherapy agent and immune system suppressant that can cause liver fibrosis in long-term usage. This study aimed to investigate the relationship between the dose of MTX and the incidence of liver fibrosis in patients with rheumatoid arthritis (RA).

Childhood-onset rheumatic diseases, such as juvenile idiopathic arthritis, juvenile-onset lupus and juvenile dermatomyositis, appear to be associated with an increased risk of comorbidities in adulthood compared to the general population. For the first stage of a research project evaluating this topic, we wanted to capture views from young people with juvenile-onset rheumatic disease to ensure that further work was relevant to their lived experience and priorities. This study aimed to determine (i) which comorbidities young people identify as important, (ii) how they access information about their disease, including comorbidity risk, whether (iii) they would like to hear about the risk of comorbidities whilst they are under paediatric care, and (iv) would be motivated to make lifestyle choices to decrease the risk of potential comorbidities.

Antiphospholipid syndrome (APS) is an acquired autoimmune prothrombotic condition. Vascular liver disorders (VLD), such as portal vein thrombosis (PVT), Budd-Chiari syndrome (BCS) and porto-sinusoidal vascular disorder (PSVD), are rare and related to an underlying hypercoagulable state in most cases. We aimed to describe the clinical and immunological features of APS patients with VLD.

Intravenous immunoglobulin (IVIG) plus corticosteroids is recommended as the initial intensified therapy for high-risk IVIG-resistant Kawasaki disease (kDa) patients. However, some patients still require additional rescue therapy despite this treatment. This study aimed to identify risk factors associated with non-response to initial IVIG plus methylprednisolone (mPSL) therapy in high-risk refractory kDa patients.

Regular screening for pulmonary hypertension (PH) is recommended in patients with systemic sclerosis (SSc) for the early detection and treatment of pulmonary arterial hypertension (PAH). Whether Doppler echocardiography may predict subsequent development of PH is still unknown. In this context, there is growing awareness of the potential importance of right atrial (RA) function in reflecting an initial overload of the right heart due to the hypertensive state in the pulmonary circulation is a matter of considerable interest.

Lupus nephritis (LN) is associated with significant renal morbidity, although some patients may experience a responsive and monophasic course. We aimed to assess the proportion of LN patients with such a course and identify its associated baseline characteristics.

This study evaluates the long-term effectiveness and safety of methotrexate-tacrolimus combination therapy compared to methotrexate monotherapy in maintaining successful tumor necrosis factor (TNF) inhibitor discontinuation in rheumatoid arthritis (RA) patients.

IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder usually characterized by multi-organ involvement. Its pathogenesis is complex and involves genetic and environmental factors, while immune responses usually mediate organ damage and promote fibrosis, which is a key feature of the disease. IgG4 responses, however, are not exclusive to IgG4-RD and can be encountered in other diseases with phenotypes that partially overlap that of IgG4-RD. Although IgG4-RD has clinical and histological hallmarks, the lack of validated diagnostic criteria often makes the diagnosis challenging, requiring a multi-dimensional approach that integrates clinical, radiological and serological data. The present Review covers recent advances in the understanding of disease drivers and its clinical phenotypes, mainly focusing on the differential diagnosis with potential IgG4-RD mimickers, namely histiocytoses, lymphoproliferative disorders, systemic vasculitides and other immune-mediated conditions. The Review also provides a schematic approach to IgG4-RD treatment, including a brief overview of glucocorticoid-sparing agents and emerging therapies, from B cell-depleting monoclonal antibodies to cytokine-targeting drugs, the majority of which are currently under investigation in randomized clinical trials.

Type I interferon (IFN) pathway activation has been associated with severe systemic sclerosis. We aimed to examine the association of serum IFN scores with disease activity and outcomes in two cohorts of patients with diffuse cutaneous systemic sclerosis.

This study aimed to predict the risk of requiring a primary hip or knee replacement 1 and 5-years in advance, using Clinical Codes.

The clinicopathological features of immune-mediated necrotizing myopathy (IMNM) sometimes mimic muscular dystrophy, complicating accurate diagnosis. The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria of idiopathic inflammatory myopathies (IIMs) are superior in terms of sensitivity and specificity; however, the sensitivity is reported to be relatively low in IMNM. We examined the clinicopathological characteristics and the prognoses of anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody-positive and anti-signal recognition particle (SRP) antibody-positive cases that do not satisfy the EULAR/ACR classification criteria.