Objectives: To assess the characteristics and risk of lymphoma in a large cohort of patients with SLE. Methods: A case–cohort analysis was performed within a dynamic cohort of SLE patients from the Spanish Society of Rheumatology Lupus Registry (RELESSER). Clinical and analytical features were compared between the lymphoma SLE group and the control SLE group using an independent-sample Student’s t-test or Mann–Whitney test for continuous variables and the χ2 test for categorical variables with Fisher’s exact test if necessary. The multivariate analysis was based on a generalized linear model. Results: Twenty-one patients with SLE and lymphoma and 3965 non-lymphoma controls with SLE were studied. Most lymphomas were of B cell origin (n = 15/21), with diffuse large B cell lymphoma being the most frequent histological type (8/21, 38.1%). As in the general population, the risk of lymphoma in SLE was higher in male than in female patients and increased with age. In the lymphoma SLE group, bivariate analysis showed a significantly higher percentage of pericarditis, organic brain syndrome, seizures, vasculitis, haemolytic anaemia, splenomegaly, venous thrombosis and mean modified (excluding lymphoma) SLICC/ACR damage index. In contrast, renal involvement, positive anti-dsDNA, and antimalarials ever were less frequent. Conclusions: In this large multicentre Spanish cohort, we identified characteristics of SLE that are associated with a higher risk of lymphoma. Antimalarials were significantly negatively associated with risk of lymphoma in SLE patients. Nevertheless, further prospective studies are needed to clarify these findings.

Objective: The objective of this study was to assess the effectiveness and safety of baricitinib (BARI) in interstitial lung disease associated with RA (RA-ILD) in clinical practice. Methods: : This was a national multicentre retrospective study of 72 RA-ILD patients treated with BARI. We analysed the following outcomes at baseline and at 3, 6, 12, 18 and 24 months, and at last follow-up: (i) dyspnea (modified Medical Research Council scale), (ii) forced vital capacity (FVC), (iii) diffusing capacity of the lungs for carbon monoxide (DLCO), (iv) chest high-resolution CT (HRCT), (v) arthritis activity (DAS28-ESR), and (vi) CS-sparing effect. Additionally, we analysed the safety data and performed a literature review up to December 2023. Results: We included 72 patients (52 women; mean (s.d.) age 68 (10) years). All patients had received DMARDs. The median [interquartile range (IQR)] ILD duration up to BARI initiation was 25 [13–63] months. The most frequent ILD patterns were usual interstitial pneumonia (n = 33; 49%) and non-specific interstitial pneumonia (n = 22; 32%). BARI was used in monotherapy in 43 (60%) patients and combined with conventional synthetic DMARDs in 29 (40%). Mean (s.d.) baseline values of FVC and DLCO (% predicted) were 86 (28) and 69 (20), respectively. After a median [IQR] follow-up of 32 [13–65] months, dyspnea, FVC, DLCO and HRCT improved or stabilized in 90%, 88%, 65% and 72%, respectively. The mean DAS28-ESR improved from 4.29 to 2.99, and the median prednisone dose was reduced from 5 to 2.5 mg/day. Relevant adverse events were uncommon. Conclusion: BARI may be a useful and safe alternative in both pulmonary and joint disease in RA-ILD patients, even in refractory cases.

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune dysregulation and progressive fibrosis, typically affecting the skin, with variable internal organ involvement. Interstitial lung disease (ILD), with a prevalence between 35 and 75%, is the leading cause of death in patients with SSc, indicating that all newly diagnosed patients should be screened for this complication. Some patients with SSc-ILD experience a progressive phenotype, which is characterized by worsening fibrosis on high-resolution computed tomography (HRCT), a decline in lung function, and premature mortality. To assess progression and guide therapeutic decisions, regular monitoring is essential and should include pulmonary function testing (PFT), symptom assessment, and repeat HRCT imaging when indicated. Multidisciplinary discussion allows a comprehensive evaluation of the available information and its consequences for management. There has been a shift in the approach to managing SSc-ILD, which includes the addition of targeted biologic and antifibrotic therapies to standard immunosuppressive therapy (particularly mycophenolate mofetil or cyclophosphamide), with autologous hematopoietic stem-cell transplantation and lung transplantation reserved for refractory cases.

To describe the characteristics of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD), compare their presentation and evolution, and analyse possible complication predictors. Multicenter study. Data were retrieved from a hospital-based study of patients with a diagnosis or suspected diagnosis of sJIA or AOSD according to the responsible physician and followed-up for at least one year. Descriptive variables (classification criteria, clinical manifestations, complications, family, and personal history) were collected at disease onset and during follow-up. We present the clinical characteristics of 326 patients, 67% of whom had a diagnosis of sJIA and 33% of AOSD. Clinical manifestation frequencies were similar between the two groups, except for odynophagia, which was significantly more frequent in AOSD than in sJIA (78.4% vs. 25.5%; p < 0.0001). Among the complications, macrophage activation syndrome (MAS) was significantly more common in sJIA than in AOSD (24.4% vs. 9.5%; p = 0.002), to the extent that an sJIA diagnosis significantly increased the risk of MAS, together with serositis presence, and the need for biological therapy. Patients with sJIA and AOSD showed similar characteristics, supporting the idea that they are both part of Still’s disease, but are expressed at different ages. Differences in manifestations and complications might be due to different management between diseases and immune response maturity.