Real-world effectiveness and safety of tocilizumab in the management of polymyalgia rheumatica: a retrospective study from the KEIO-PMR cohort.

Objective: This study aimed to evaluate the effectiveness and safety of tocilizumab in polymyalgia rheumatica (PMR) in the real world.

Methods: All consecutive patients diagnosed with PMR who visited Keio University Hospital between May 2012 and October 2022 were retrospectively reviewed. Patients were divided into three groups: the tocilizumab group, the immunosuppressant group without biological agents, and the glucocorticoid monotherapy group.

Isolated IgG4-related prostatitis masquerading as prostate cancer: a diagnostic pitfall in patients with elevated prostate-specific antigen and prostatic mass.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated condition that can affect multiple organs. Prostatic involvement, although uncommon, may closely mimic prostate cancer due to the presence of a prostatic mass and elevated prostate-specific antigen (PSA) levels. In rare cases, IgG4-related prostatitis occurs in isolation, without evidence of other organ involvement.

Successful tocilizumab monotherapy for multicentric Castleman disease complicated by rheumatoid arthritis and Sjögren's disease: a case-based literature review.

Multicentric Castleman disease (MCD) is a rare and complex lymphoproliferative disorder marked by multiple lymph node enlargement, characteristic histopathological features, and systemic inflammation driven by excessive interleukin-6 (IL-6) production. MCD can occasionally coexist with other autoimmune diseases, but the therapeutic approach in such cases remains poorly defined. Here, we present the first documented case of MCD complicated by both rheumatoid arthritis (RA) and Sjögren's disease (SjD), where all conditions were successfully treated with tocilizumab (TCZ) monotherapy, an IL-6 receptor antagonist.

Risk factors of cytomegalovirus reactivation in patients with rheumatic musculoskeletal diseases undergoing remission induction therapy with high-dose glucocorticoids; a prospective short-term cohort study.

Objective: To elucidate the risk factors of cytomegalovirus (CMV) infection and the involvement of immunity status in CMV infection in patients with rheumatic musculoskeletal disease during remission induction therapy.

Methods: Patients with rheumatic musculoskeletal disease who underwent induction therapy with high-dose glucocorticoids were consecutively enrolled. All patients were screened for CMV-IgG at baseline and monitored weekly for CMV pp65 antigen in polymorphonuclear leukocytes from peripheral blood until discharge.

Tubulointerstitial nephritis with predominant monotypic plasma cell infiltration in three cases of Sjögren's syndrome: case reports and literature review.

Tubulointerstitial nephritis (TIN) is a common kidney manifestation of Sjögren's syndrome, typically characterized by interstitial infiltration of T and B cells, with plasma cells being more prominent than in other etiologies. While most plasma cell infiltration in TIN is polytypic and the importance of the immunoglobulin isotypes expressed by the infiltrating plasma cells in the pathogenesis is unknown, recent reports have identified rare cases with monotypic IgA-positive plasma cell infiltration accompanied by monoclonal gammopathy of undetermined significance (MGUS). Here, we present three cases of Sjögren syndrome-associated TIN characterized by monotypic plasma cell infiltration.

Interleukin-32-expressing CD4 T cells are a potentially pathogenic subset in systemic sclerosis with interstitial lung disease.

Background: Systemic sclerosis (SSc) is an autoimmune disease characterized by vasculopathy, fibrosis, and inflammation. CD4 T cells produce cytokines that are crucial in the pathogenesis of SSc. However, the role of CD4 T cells in SSc-associated interstitial lung disease (SSc-ILD) remain unclear.

An IL-6 inhibitor subsides iatrogenic aortitis induced by granulocyte-colony stimulating factor without interruption of chemotherapy.

Recombinant human granulocyte-colony stimulating factor (G-CSF) is widely used for primary or secondary leukopenia induced by chemotherapy with strong anticancer drugs. Recently, there have been rare but accumulating cases of aortitis in patients receiving G-CSF agents, which are usually treated with glucocorticoids. Here, we report a case of G-CSF-induced aortitis complicated with intensive chemotherapy for Ewing's sarcoma, which was successfully treated with one bolus of intravenous tocilizumab, an anti-interleukin-6 inhibitor, resulting in early suppression of aortic inflammation and prompt resumption of chemotherapy.

Clinical features, pathogenesis, and treatment of inflammatory bowel disease-associated spondyloarthritis.

Inflammatory bowel disease-associated spondyloarthritis (IBD-SpA) is a unique subtype of SpA that affects approximately 10-20 % of patients with IBD. It encompasses both peripheral arthritis and axial involvement, with enthesitis being increasingly recognized. Despite its clinical significance, there are currently no established screening tools, classification criteria, or standardized treatment guidelines specific to IBD-SpA.

Serum cytokine levels towards precision medicine in Still's disease: a subanalysis of a randomized controlled trial of tocilizumab.

Objectives: To predict the efficacy of IL-6 inhibition in patients with Still's disease by analysing inflammatory cytokine profiles before and during an IL-6 receptor inhibitor therapy.

Methods: This is a subanalysis of the 52-week, randomized, double-blind, placebo-controlled trial of tocilizumab, an IL-6 receptor inhibitor, in patients with Still's disease. Multiple serum cytokine levels were measured regularly, and their pattern and profiles were analysed based on the response to tocilizumab.

Recommendations for the treatment of juvenile idiopathic arthritis with oligoarthritis or polyarthritis from the 2024 update of the Japan College of Rheumatology Clinical Practice Guidelines for the management of rheumatoid arthritis including juvenile idiopathic arthritis with oligoarthritis or polyarthritis - secondary publication.

Objectives: To conduct systematic reviews (SRs) and develop clinical practice guidelines (CPGs) for managing juvenile idiopathic arthritis (JIA) with oligoarthritis or polyarthritis.

Methods: The Grading of Recommendations, Assessment, Development, and Evaluation methodology was employed to carry out SRs and formulate the CPGs. An expert panel, including patients, paediatric and nonpaediatric rheumatologists, guideline specialists, and patient representatives, used the Delphi method to discuss and agree on the recommendations.

Polyangiitis overlap syndrome is a high-risk clinical phenotype for relapse: Case series from the KEIO-vasculitis cohort.

Background: Polyangiitis overlap syndrome, characterized by the coexistence of giant cell arteritis (GCA) and anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), is a rare and poorly understood condition. Clinical features, treatment responses, and long-term prognosis of this entity remain unclear. This study aimed to elucidate clinical characteristics, relapse patterns, and potential therapeutic strategies for polyangiitis overlap syndrome.

Protocol: Filgotinib in addition to methotrexate versus filgotinib monotherapy in patients with rheumatoid arthritis with an inadequate response to methotrexate: A multicenter, prospective, open-label, randomized controlled trial (FAITHFUL Study).

Background: Filgotinib (FIL), a Janus kinase-1 preferential inhibitor, has been studied for its efficacy and safety in rheumatoid arthritis. The FINCH3 trial compared FIL monotherapy, FIL plus methotrexate (MTX) combination therapy, and MTX monotherapy in MTX-naïve patients. However, comparisons in patients with an inadequate response to MTX remain unclear.

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.

Association between Rheumatoid Arthritis and the Incidence of IgA Nephropathy.

Introduction: IgA nephropathy and rheumatoid arthritis are both common autoimmune diseases, but epidemiological data are limited on the relationship between these two diseases. We aimed to assess the association between rheumatoid arthritis and the risk of developing IgA nephropathy.

Methods: In this study, we analyzed 4,311,393 adults using a nationwide epidemiological database in Japan.

Pearls and pitfalls in imaging of axial spondyloarthritis for rheumatologists.

This review provides key insights into sacroiliitis imaging, covering anatomy, imaging techniques, and interpretation. It focuses on normal physiological changes in young to middle-aged women, children, and the elderly that can resemble sacroiliitis. We emphasize the importance of distinguishing between active and structural lesions, as highlighted in the recent ASAS-SPARTAN standardized magnetic resonance imaging protocol.

The patient journey and burden of disease in progressive pulmonary fibrosis in Japan: a cross-sectional survey.

Background: For patients with interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) with a progressive pulmonary fibrosis (PPF) phenotype, current knowledge of patient experience and symptom burden is limited. This study aimed to describe the patient journey for patients with PPF and IPF in a real-world setting in Japan.

Methods: Data were analyzed from the Adelphi Real World PPF-ILD Disease Specific Programme™, a cross-sectional survey with elements of retrospective data collection of pulmonologists and rheumatologists in Japan from April to October 2022.

Efficacy of tocilizumab monotherapy for autoimmune hemolytic anemia associated with idiopathic multicentric Castleman disease: a case-based review.

Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder characterized by generalized lymphadenopathy with distinctive histopathological features and systemic inflammation driven by excessive interleukin-6 (IL-6) production. Anemia due to chronic inflammation is a common manifestation of iMCD; however, this disease can also be complicated by autoimmune hemolytic anemia (AIHA). While tocilizumab (TCZ), an IL-6 receptor blockade, has demonstrated efficacy in treating iMCD, its therapeutic effect on AIHA secondary to iMCD remains unclear.

Identification of BHLHE40-Expressing CD4 T Cells Producing GM-CSF in Rheumatoid Arthritis.

Background: Granulocyte-macrophage colony-stimulating factor (GM-CSF: CSF2) plays a crucial role in the pathogenesis of autoimmune diseases. The basic helix-loop-helix family member e40 (BHLHE40) gene is important for GM-CSF production in CD4 T cells. However, the relationship between the expression of these genes and rheumatoid arthritis (RA) remains unclear, particularly in interleukin-1 (IL-1)-enriched inflammatory sites.

Radiological extent predicts prognosis and relapse after acute exacerbation of interstitial lung disease in patients with rheumatoid arthritis: KEIO-RA-cohort.

To clarify how the extent of radiological interstitial lung abnormalities impacts the prognosis and risk of relapse in patients with rheumatoid arthritis (RA) following acute exacerbation of interstitial lung disease (AE-ILD). We conducted a retrospective review of all consecutive RA patients from the KEIO-RA cohort who experienced AE-ILD during 2012 to 2024. We investigated the association between the extent of abnormalities on high-resolution computed tomography (HRCT) images, as assessed by Goh's criteria, and outcomes in patients who survived AE-ILD versus those who did not, as well as between patients who survived AE-ILD without relapse and those who experienced relapse.

Discontinuation vs. continuation of concomitant methotrexate in patients with rheumatoid arthritis on certolizumab pegol: results from a randomised, controlled trial.

Objective: The present non-inferiority study was designed to compare the effect of discontinuing versus continuing methotrexate (MTX) alongside certolizumab pegol (CZP) on maintaining low disease activity (LDA) in rheumatoid arthritis (RA) patients already stable on combination therapy.

Methods: This multicentre, open-label, randomised, controlled trial included RA patients with sustained LDA (Clinical Disease Activity Index [CDAI] ≤ 10) for ≥ 12 weeks with CZP + MTX. Patients were randomised 1:1 by computer to either continue MTX (CZP + MTX group) or discontinue MTX after a 12-week reduction period (CZP group) using a dynamic allocation strategy with the minimisation method.

Achievement and Usefulness of Intermediate Treatment Targets for Still Disease Proposed by the European Alliance of Associations for Rheumatology and the Paediatric Rheumatology European Society.

Objective: Intermediate treatment targets in Still disease have been proposed by the European Alliance of Associations for Rheumatology (EULAR) and the Paediatric Rheumatology European Society (PReS) in 2024. This study aimed to evaluate the utility of the targets in clinical practice.

Methods: Consecutive patients with adult-onset Still disease (AOSD) based on the Yamaguchi criteria who visited Keio University Hospital from April 2012 until May 2024 were retrospectively reviewed.

Trans-omics landscape of systemic vasculitis identified matrix metalloproteinase 12 as a novel signature molecule.

Objectives: To identify key molecules involved in the disease pathophysiology of systemic vasculitis through trans-omics analysis, with a specific focus on demonstrating matrix metalloproteinase (MMP) 12 as a potential biomarker in rheumatic entities.

Methods: Patients with newly diagnosed or relapsed rheumatic and musculoskeletal diseases from June 2013 until September 2022 were enrolled. We screened vasculitis-specific molecules by combining findings from serum proteome analysis and whole-blood RNA sequencing.