Safety of mycophenolate mofetil in systemic lupus erythematosus maintenance therapy: insights from the LUNA registry in a nationwide prospective cohort study.

Objective: We aimed to compare the incidence of severe infections between mycophenolate mofetil (MMF) and other immunosuppressants in patients with systemic lupus erythematosus (SLE) on maintenance therapy using data from the Lupus Registry of Nationwide Institutions in Japan.

Methods: This study employed a prospective cohort design, including patients with SLE undergoing maintenance therapy. Exposure was defined as MMF treatment, and the control group included individuals who received other immunosuppressants (non-MMF) treatments.

Anti-LGI1 antibody-positive autoimmune encephalitis concurrent with neuropsychiatric systemic lupus erythematosus.

Anti-leucine-rich glioma-inactivated protein 1 (LGI1) antibody-positive limbic encephalitis is a form of autoimmune encephalitis (AE). The importance of anti-LGI1 antibody in neuropsychiatric systemic lupus erythematosus (NPSLE) remains unclear. We present a 65-year-old woman with amnesia and disorientation, positive anti-LGI1 and anti-Smith antibodies, hyponatremia, leukopenia, and hypocomplementemia.

Impact of glucocorticoid tapering speed on renal outcomes in proliferative lupus nephritis: a multicentre retrospective study.

Objectives: Recent guidelines and recommendations for LN suggest rapid glucocorticoid (GC) reduction; however, robust supporting evidence remains limited. This study aimed to evaluate the impact of rapid GC reduction on renal outcomes in patients with proliferative LN.

Methods: We conducted a multicentre retrospective chart review of patients with GC-naïve, biopsy-proven proliferative LN with available urinary protein-to-creatinine ratio (UPCR) data before and 52 weeks after GC treatment.

Implications of serum calcitonin gene-related peptide and type I interferon in systemic lupus erythematosus-associated headaches.

ObjectiveHeadache is a common neurological symptom in patients with systemic lupus erythematosus (SLE). Calcitonin gene-related peptide (CGRP) is a potential chemical mediator of headaches, while type I interferon (IFN) plays a pivotal role in the immune system of SLE. This study investigated the implications of CGRP and type I IFNs as headache biomarkers in patients with SLE.

Risk factors for cytomegalovirus reactivation during the treatment of ANCA-associated vasculitis: A retrospective cohort study of the J-CANVAS study.

Objectives: Cytomegalovirus (CMV) reactivation during immunosuppressive therapy poses a risk of severe infections. This study aimed to investigate the risk factors of CMV reactivation in patients with microscopic polyangiitis and granulomatosis with polyangiitis using a nationwide cohort in Japan.

Methods: This retrospective cohort study used data from the Japan Collaborative Registry of antineutrophil cytoplasmic antibody-associated vasculitis.

Effectiveness for remission maintenance rate and safety of different rituximab regimens for treating anti-neutrophil cytoplasmic antibody-associated vasculitis in Japan: a J-CANVAS study.

Rituximab (RTX) has been reported to effectively maintain remission in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). In this multicenter study involving 57 patients who achieved remission after 24 weeks, we evaluated the effectiveness of RTX in maintaining remission in patients with AAV. Patients were divided into three groups based on RTX administration: continuous, induction phase-only, and maintenance phase-only groups.

Differences in phenotypes, treatments, and outcomes of ANCA-associated vasculitis across Europe, Japan and the USA in 2020.

Objectives: To clarify the differences in clinical phenotypes, therapeutic patterns, and outcomes of patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) across geographic regions using a multinational cohort.

Methods: Data were collected from patients with newly diagnosed or relapsing GPA or MPA in Europe, Japan and the USA from January to July 2020. The composite outcome of kidney failure and/or death within 52 weeks after treatment was evaluated, and the hazard ratios across the regions were estimated using the Cox proportional hazard model.

Clinical practice pattern of Pneumocystis pneumonia prophylaxis in systemic lupus erythematosus: a cross-sectional study from lupus registry of nationwide institutions (LUNA).

Background: Pneumocystis jirovecii pneumonia (PCP) is an opportunistic infection in patients undergoing immunosuppressive therapy, such as glucocorticoid (GC) medication, for systemic autoimmune diseases like systemic lupus erythematosus (SLE). Despite the confirmed effectiveness of PCP prophylaxis, its clinical administration, especially in conjunction with GC dosage, remains unclear. We aimed to describe the clinical practice of PCP prophylaxis in association with SLE in Japan, evaluate the relationship between GC dosage and PCP prophylaxis, and explore the practice patterns associated with PCP prophylaxis.

Effectiveness and safety of rituximab in severely relapsed antineutrophil cytoplasmic antibody-associated vasculitis: a retrospective analysis of a Japanese multicentre cohort from the J-CANVAS.

We aimed to clarify the long-term safety and efficacy of rituximab (RTX) as a remission induction therapy following severe relapse in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We retrospectively collected the data of patients with severely relapsed AAV from a Japanese multicentre cohort. The primary exposure was RTX use; the primary outcome was complete remission (CR) proportions at week 24.

Headache in systemic lupus erythematosus: The LUNA registry cross-sectional study.

Objectives: This study investigated the clinically relevant factors for headaches in patients with systemic lupus erythematosus (SLE) using a registry from a Japanese multicenter cohort.

Methods: This cross-sectional study analysed the clinical information of patients with SLE who experienced headache episodes using the Migraine Disability Assessment (MIDAS) questionnaire. Significant findings in the comparisons between patients with headache (HA patients) and those without headache (non-HA patients) and in the comparisons depending on the grades of headache-induced disability in daily life based on the MIDAS scores were evaluated.

Effectiveness of intravenous methylprednisolone pulse in patients with severe microscopic polyangiitis and granulomatosis with polyangiitis.

Objectives: To evaluate the effectiveness and safety of two different intravenous methylprednisolone (IVMP) pulse doses in patients with severe microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA).

Methods: We emulated a target trial using observational data from the nationwide registry in Japan. Patients with severe glomerulonephritis or diffuse alveolar haemorrhage were selected and pseudo-randomized into three groups using propensity score-based overlap weighting as follows: non-IVMP, IVMP 0.

Improvement of active salivary gland ultrasonography findings in Sjögren's syndrome in response to short-term glucocorticoid treatment: A case report and review of the literature.

Salivary gland ultrasonography is a non-invasive imaging technique that helps in the diagnosis and assessment of disease activity in Sjögren's syndrome. However, it remains unclear whether the salivary gland ultrasonography findings are reversible in response to treatment. We present a case of a woman in her 20s who presented with parotid swelling and pain lasting for 3 months.

Optimal dose of intravenous cyclophosphamide during remission induction therapy in ANCA-associated vasculitis: A retrospective cohort study of J-CANVAS.

Objectives: To identify the optimal dose of intravenous cyclophosphamide (IVCY) for induction therapy for anti-neutrophil cytoplasmic antibody-associated vasculitis.

Methods: We retrospectively assessed patients with antibody-associated vasculitis who received IVCY every 2-3 weeks during the remission induction phase. The associations of the IVCY dose with infection-free survival and relapse-free survival were analysed using a Cox regression model.

Association between hypogammaglobulinaemia and severe infections during induction therapy in ANCA-associated vasculitis: from J-CANVAS study.

Objectives: To investigate the association between decreased serum IgG levels caused by remission-induction immunosuppressive therapy of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and the development of severe infections.

Methods: We conducted a retrospective cohort study of patients with new-onset or severe relapsing AAV enrolled in the J-CANVAS registry, which was established at 24 referral sites in Japan. The minimum serum IgG levels up to 24 weeks and the incidence of severe infection up to 48 weeks after treatment initiation were evaluated.

Number of Attending Physicians and Accumulated Organ Damage in Patients with Systemic Lupus Erythematosus: LUNA Registry Cross-Sectional Study.

Introduction: Patients with systemic lupus erythematosus (SLE) frequently change attending physicians. The number of changes in attending physicians is related to the accumulated organ damage in patients with diabetes mellitus and inflammatory bowel disease, although similar results are not known for patients with SLE. This study investigated whether the number of attending physicians after the onset of SLE is associated with organ damage.

Latent trajectory modelling of pulmonary artery pressure in systemic sclerosis: a retrospective cohort study.

Objectives: To visualise the trajectories of pulmonary arterial pressure (PAP) in systemic sclerosis (SSc) and identify the clinical phenotypes for each trajectory, by applying latent trajectory modelling for PAP repeatedly estimated by echocardiography.

Methods: This was a multicentre, retrospective cohort study conducted at four referral hospitals in Kyoto, Japan. Patients with SSc who were treated at study sites between 2008 and 2021 and who had at least three echocardiographic measurements of systolic PAP (sPAP) were included.

Late-onset rheumatoid arthritis registry study, LORIS study: study protocol and design.

Background: Although drug treatment strategies for rheumatoid arthritis (RA) are relatively well established, there is a paucity of evidence on the treatment in older patients. The purpose of this study is to build a registry for late-onset RA (LORA), which is expected to increase rapidly worldwide. In addition, we aim to propose optimal treatment strategies according to the patient background including frailty, thereby contributing to improving the quality of treatment and daily living in patients with RA.