Publications by authors named "Ran Nakashima"

Article Synopsis
  • *Methods*: Researchers analyzed mesenteric lymph nodes in lupus model mice to identify specific bacteria and their connection to autoantibody production using various laboratory techniques.
  • *Results*: The study found that the bacterium Lactobacillus murinus was linked to heightened anti-dsDNA IgG levels in lupus mice, and its ABC transporter was identified as a cross-reactive antigen that could trigger antibody production in SLE.
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  • - Idiopathic inflammatory myopathies (IIM) are autoimmune diseases primarily affecting muscles and can lead to serious complications like interstitial lung disease (ILD), which significantly impacts survival and quality of life.
  • - Myositis-specific antibody (MSA) profiles help determine different clinical responses, prognoses, and racial differences in IIM-ILD; for instance, the anti-melanoma differentiation-associated gene 5 antibody is linked to rapidly progressive ILD (RP-ILD), especially in Asian populations.
  • - Early aggressive treatment with corticosteroids and immunosuppressants can improve outcomes for IIM-ILD, but patients with certain antibodies, like anti-aminoacyl-tRNA synthetase (ARS), may have a
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  • - This study compared juvenile idiopathic inflammatory myopathy (IIM) and adult-IIM by analyzing autoantibodies, clinical characteristics, and drug-free conditions in 320 patients over 20 years.
  • - Juvenile-IIM showed a higher rate of achieving drug-free conditions (34%) compared to adult-IIM (18%), with specific autoantibodies like anti-TIF1-γ linked to fewer muscle symptoms and lower malignancy rates in juveniles.
  • - Both groups had overlapping features with certain autoantibodies (like anti-MDA-5 and anti-ARS) associated with severe symptoms and a low likelihood of drug-free conditions, while treatment patterns differed notably between the two age groups.
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Vesiculobullous dermatomyositis (VD) is a rare manifestation of dermatomyositis (DM) and has been suggested to be associated with malignancy. Although the myositis-specific autoantibodies are associated with distinct clinical presentations of DM, those associated with VD remain unclear. Here, we present the case of a 54-year-old man with VD who tested positive for anti-nuclear matrix protein 2 (NXP-2) antibody, one of the DM-specific autoantibodies.

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Anti-aminoacyl tRNA synthetase (ARS) antibodies are the most frequent in idiopathic inflammatory myopathy, notably associated with anti-synthetase syndrome (ASyS), which is characterized by six clinical features: arthritis, myositis, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanical hands. Although patients with ASyS often respond well to initial glucocorticoid (GC) therapy, they tend to have a chronic, recurrent disease course. In anti-ARS-positive patients, the treatment goal involves suppressing disease recurrence and progression while achieving a minimal GC dose.

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Objective: Infections are a critical concern for patients with microscopic polyangiitis (MPA). This study aimed to identify the risk factors associated with serious infections (SIs) and infection-related mortality in patients with MPA, as well as the effect of glucocorticoid (GC) dose tapering on these outcomes.

Methods: This multicentre, retrospective, and observational study utilised data from a cohort of patients with MPA in Japan [Registry of Vasculitis Patients to Establish REAL World Evidence (REVEAL) cohort].

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Article Synopsis
  • Avacopan is an oral medication that acts as a C5a receptor antagonist and is effective in treating microscopic polyangiitis by inhibiting the activation of neutrophils.
  • A patient case showed that despite high-dose steroids and azathioprine not reducing myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA), adding avacopan lowered these antibody levels.
  • The findings suggest avacopan might help in decreasing MPO-ANCA and improving ANCA-associated vasculitis, but more research with larger studies is needed to confirm these effects.
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  • The CHANGE survey aims to assess gender equity in rheumatology and gather physician insights on bullying, harassment, and equitable opportunities.
  • Launched in January 2023, the survey is a cross-sectional questionnaire available in six languages, targeting rheumatologists and healthcare professionals, with responses analyzed for gender-based discrimination.
  • This global initiative seeks to identify gender-related disparities in rheumatology to inform strategies for promoting inclusivity and equitable access to opportunities for all professionals in the field.
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Dermatomyositis (DM) is an autoimmune disease that causes proximal muscle weakness in the extremities leading to severe immobility and dysphagia. Approximately 20% of patients with DM are positive for anti-TIF-1γ antibody and frequently accompanied by malignant tumors. Although DM remission after tumor resection has been reported, the indications for surgery in patients with severe DM are unknown.

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Anti-aminoacyl-tRNA synthetase (ARS) antibodies are myositis-specific antibodies associated with anti-synthetase syndrome (ASSD). Some patients are positive for anti-ARS antibodies on enzyme-linked immunosorbent assay (ELISA) but negative on RNA-immunoprecipitation (RNA-IP) (the gold standard method). Whether these patients should be considered truly positive for anti-ARS antibodies remains unclear.

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TAFRO syndrome is an acute systemic inflammatory disease characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal dysfunction, and organomegaly. There have been increasing reports that TAFRO is a disease distinct from idiopathic multicentric Castleman disease and that TAFRO patients may be positive for anti-SSA antibodies. To assess anti-SSA antibody positivity and the clinical characteristics of the two diseases, we retrospectively compared 7 TAFRO and 10 iMCD patients in our hospital.

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  • IgG4-related disease (Ig4-RD) is a condition that can impact various organs, such as the salivary and lacrimal glands, pancreas, and liver.
  • A 72-year-old woman exhibited multiple symptoms including high eosinophil counts, elevated IgG4 levels, and dysfunction in liver and nerves, which led to a diagnosis of Ig4-RD confirmed through biopsies.
  • The patient showed improvement with glucocorticoid treatment, emphasizing the importance of biopsies to correctly differentiate Ig4-RD from similar disorders.
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Objective: Antimelanoma differentiation-associated gene 5 (anti-MDA5)-positive dermatomyositis with interstitial lung disease (DM-ILD) progresses rapidly and has a poor prognosis. Previously, we reported the efficacy of a combination therapy comprising high-dose glucocorticoids (GCs), calcineurin inhibitors (CNIs), and intravenous cyclophosphamide (IV CYC) in a multicenter clinical trial (UMIN000014344). In the present study, we evaluated the long-term outcomes and effects of induction therapy on the maintenance of remission.

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Advanced systemic sclerosis-associated interstitial lung disease (SSc-ILD) can be treated with lung transplantation. There is limited data on lung transplantation outcomes in patients with SSc-ILD, in non-Western populations.We assessed survival data of patients with SSc-ILD, on the lung transplant (LT) waiting list, and evaluated post-transplant outcomes in patients from an Asian LT center.

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Objectives: Decreased sialylation of IgG-Fc glycans has been reported in autoimmune diseases, but its role in systemic lupus erythematosus (SLE) is not fully understood. In this study, we examined the pathogenicity of IgG desialylation and its association with Th17 in SLE using an animal model.

Methods: B6SKG mice, which develop lupus-like systemic autoimmunity due to the ZAP70 mutation, were used to investigate the pathogenicity of IgG desialylation.

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Background: Systemic lupus erythematosus (SLE) is an intractable disease characterized by autoantibody production and autoreactive B and T cell proliferation. Although several studies have revealed multiple genetic and environmental associations, the underlying mechanisms remain unknown.

Methods: We performed proteomics and transcriptomics using liquid chromatography-mass spectrometry and DNA microarray, using peripheral blood B cells from patients with SLE, and healthy controls (HC).

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Objectives: Although the SLE Disease Activity Score (SLE-DAS) and its definitions to classify disease activity have been recently developed to overcome the drawbacks of the SLE Disease Activity Index 2000 (SLEDAI-2K), the performance of the SLE-DAS for patient-reported outcomes (PROs) has not been fully examined. We aimed to compare SLE-DAS with SLEDAI-2K and validate the classifications of disease activity based on SLE-DAS in terms of PROs.

Methods: We assessed generic quality of life (QoL) using the Medical Outcome Survey 36-Item Short-Form Health Survey (SF-36), disease-specific QoL using the lupus patient-reported outcome tool (LupusPRO), burden of symptoms using the SLE Symptom Checklist (SSC), patient global assessment (PtGA) and physician global assessment (PhGA).

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Article Synopsis
  • * Tests confirmed EBV reactivation contributed to the lymphoma, but after stopping certain medications, he was treated with chemotherapy, achieving complete remission of both the lymphoma and MDA5-DM.
  • * A review of 19 similar cases revealed mixed outcomes; while some improved after stopping immunosuppressants, others required chemotherapy, with a few fatalities, emphasizing the risks of aggressive treatment for related conditions.
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Immunoglobulin (Ig) G4 is an IgG subclass that can exhibit inhibitory functions under certain conditions because of its capacity to carry out Fab-arm exchange, inability to form immune complexes, and lack of antibody-dependent and complement-dependent cytotoxicity. Although several diseases have been associated with IgG4, its role in the disease pathogeneses remains unclear. Since mice do not express an IgG subclass that is identical to the human IgG4 (hIgG4), we generated hIGHG4 knock-in (KI) mice and analyzed their phenotypes.

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Objectives: Osteoporosis and compression fractures of the lumbar spine are some of the major adverse effects of glucocorticoid therapy in patients with systemic lupus erythematosus (SLE). This study examined the association between bone mineral density, bone turnover markers, presence of vertebral fractures, and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index in SLE patients.

Methods: This was a cross-sectional study of 246 outpatients with SLE at the Kyoto University Hospital.

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  • The study explores how rheumatoid factor (RF) recognizes the complex of IgG heavy chain (IgGH) and a specific HLA class II molecule linked to rheumatoid arthritis (RA).
  • It involved synthesizing various IgGH segments and testing their expression and recognition by RF using a human kidney cell line.
  • Results showed that the CH1 domain of IgGH binds to the HLA-DR4 molecule and that RF specifically targets the CH3 domain of the IgGH/HLA-DR4 complex.
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Autoimmune diseases are often accompanied by acute exacerbation. However, the mechanism underlying systemic lupus erythematosus (SLE) flares remains unclear. We investigated whether short-term enteric Toll-like receptor 7 (TLR7) stimulation can exacerbate SLE using B6SKG mice, which spontaneously develop SLE due to a mutation in the zeta‒chain‒associated protein kinase 70 (Zap70) gene.

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