Japanese Spotted Fever with Severe Myositis: A Diagnostic Challenge.

A woman in her late 70s presented with a fever, rashes, and marked proximal muscle weakness. Noninfectious conditions, including myositis and vasculitis, were initially considered. Treatment with cephalosporins was ineffective, but the symptoms improved with minocycline, indicating possible Japanese spotted fever (JSF) despite no apparent history of tick exposure.

Influence of doctor-patient relationships and health-related factors on the medical satisfaction of patients with Sjögren's disease.

Objectives: Patients with chronic, incurable conditions rely on their providers to help relieve their symptoms. Dissatisfaction with their care can erode the doctor-patient relationship and reduce the effectiveness of treatment. We investigated the relationships between satisfaction and symptoms, the doctor-patient relationship, and health-related factors in patients with Sjögren's disease (SjD) in Japan.

The long-term safety and tolerability of anifrolumab for patients with systemic lupus erythematosus in Japan: TULIP-LTE subgroup analysis.

Objectives: Evaluate the long-term safety and tolerability of anifrolumab 300 mg, alongside standard therapy, in patients from Japan with systemic lupus erythematosus (SLE) in the TULIP-LTE trial (NCT02794285).

Methods: TULIP-LTE was a 3-year, randomized, double-blind, placebo-controlled long-term extension (LTE) of the TULIP trials. The primary safety outcome included serious adverse events (SAEs) and AEs of special interest (AESIs) during the LTE period.

Questionnaire survey on the prevention and development of cervical cancer in patients with systemic lupus erythematosus in Japan.

Objectives: The aim is to evaluate the prevention and development of cervical cancer in systemic lupus erythematosus (SLE) patients in Japan and its background based on a questionnaire survey.

Methods: The questionnaire was handed to 460 adult female SLE patients at 12 medical institutions. The participants were grouped by age, and data related to their human papillomavirus vaccination status, age at first coitus, cervical cancer screening, and diagnosis of cervical cancer were analysed.

Social independence evaluation index for Japanese patients with childhood-onset chronic diseases.

Introduction: This study established an independent evaluation index for patients with childhood-onset chronic diseases in Japan.

Methods: From November to December 2020, three Delphi rounds were conducted. Thirty-nine participants completed at least one survey.

Clinical variables, including novel joint index, associated with future patient-physician discordance in global assessment of rheumatoid arthritis (RA) disease activity based on a large RA database in Japan.

Background: Discordance between patient global assessment (PGA) and physician global assessment (PhGA) of rheumatoid arthritis (RA) disease activity is mainly determined by pain and functional disabilities. This study aimed to investigate the shift in PGA-PhGA discordance and the variables associated with future positive discordance (PGA > PhGA) based on the NinJa database in Japan.

Methods: We examined 7557 adults with RA registered in both NinJa 2014 and 2018, with a discordance cutoff of 3 on a 10-cm scale.

Distal Interphalangeal Joint Involvement May Be Associated with Disease Activity and Affected Joint Distribution in Rheumatoid Arthritis.

We investigated the relationship between distal interphalangeal (DIP) joint involvement and disease activity in 10,038 patients with adult-onset rheumatoid arthritis (RA). The affected joint distribution was investigated using the joint indices (JI) , , and , corresponding to the upper and lower joints, and the predominance of large-joint involvement, respectively. DIP joint involvement (defined by the presence of tenderness and/or swelling in DIP joints) was present in 206 (2.

Abatacept ameliorates both glandular and extraglandular involvements in patients with Sjögren's syndrome associated with rheumatoid arthritis: Findings from an open-label, multicentre, 1-year, prospective study: The ROSE (Rheumatoid Arthritis with Orencia Trial Toward Sjögren's Syndrome Endocrinopathy) and ROSE II trials.

Objective: To clarify the efficacy and safety of intravenous abatacept for glandular and extraglandular involvements in Sjögren's syndrome (SS) associated with rheumatoid arthritis (RA).

Materials And Methods: We performed an open-label, prospective, 1-year, observational multicenter study (ROSE and ROSE II trials). The primary endpoint was the remission rate as measured by SDAI at 52 weeks.

Transitioning from paediatric to adult rheumatological healthcare: English summary of the Japanese Transition Support Guide.

Issues related to transitioning from paediatric to adult healthcare are currently receiving international attention. In Japan, 1000 patients with childhood-onset chronic rheumatological diseases reach adulthood every year and require transition from care by paediatric to care by adult rheumatologists. Here, we propose a guide for the latter, wherein the adult caregiver poses the clinical questions about transitional support that they need to have answered, and the paediatric caregiver mainly compiles the plans for the transition.

Factors associated with delayed diagnosis of Sjögren's syndrome among members of the Japanese Sjögren's Association for Patients.

Objectives: The heterogeneous nature of the signs and symptoms of Sjögren's syndrome (SS) often causes delays in diagnosis. The reasons for these delays have not been investigated in Japan and need to be determined.

Methods: We conducted a questionnaire survey of members of the Japanese Sjögren's Association for Patients (JSAP).

The impact of shrunken pore syndrome in patient with rheumatic diseases on bone mineral metabolism.

The study aimed to investigate the influence of shrunken pore syndrome (SPS), defined as a cystatin C (CysC)-based estimated glomerular filtration rate (eGFR) <60% of the creatinine (Cr)-based eGFR (eGFR), on bone mineral density (BMD) in patients with rheumatic diseases. A total of 831 patients with rheumatic diseases were enrolled in the study. Patients were classified into the SPS group (G-SPS) and non-SPS group (G-nSPS).

Influence of seasonal changes on disease activity and distribution of affected joints in rheumatoid arthritis.

Background: Previous studies suggest that RA activity is sensitive to seasonal changes. This study explored the influence of season on RA activity, particularly the distribution of affected joints, using a nationwide database in Japan.

Methods: We investigated 12,839 patients whose RA activity was recorded in spring (n = 3250), summer (n = 916), fall (n = 1021), and winter (n = 7652).

Influence of large joint involvement on patient-physician discordance in global assessment of rheumatoid arthritis disease activity analyzed by a novel joint index.

Objectives: The discordance between patient global assessment (PGA) and physician global assessment (PhGA) of rheumatoid arthritis (RA) disease activity may be problematic in clinical practice. The aim of this study was to identify determinants of this discordance using a nationwide RA database in Japan (NinJa) with special attention to large joint involvement.

Methods: We investigated 12 043 adults with RA and used a discordance cutoff of 3 cm.

Clinical practice guideline for Sjögren's syndrome 2017.

Objectives: The objective of this study is to develop clinical practice guideline (CPG) for Sjögren's syndrome (SS) based on recently available clinical and therapeutic evidences.

Methods: The CPG committee for SS was organized by the Research Team for Autoimmune Diseases, Research Program for Intractable Disease of the Ministry of Health, Labor and Welfare (MHLW), Japan. The committee completed a systematic review of evidences for several clinical questions and developed CPG for SS 2017 according to the procedure proposed by the Medical Information Network Distribution Service (Minds).

Anticentromere antibody-positive primary Sjögren's syndrome: Epitope analysis of a subset of anticentromere antibody-positive patients.

Objectives: Anticentromere antibody (ACA) is generally considered to be a serological marker for systemic sclerosis (SSc). ACA-positive patients with primary Sjögren's syndrome (pSS) have also been reported. ACA often recognizes centromere proteins (CENPs): CENP-A, CENP-B, and CENP-C, and sometimes reacts to heterochromatin protein 1 (HP1)α.

Validation of EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient indexes (ESSPRI).

Objectives: To validate the two recently developed disease activity indexes for assessment of primary Sjögren's syndrome (SS): the European League Against Rheumatism (EULAR) SS Patient Reported Index (ESSPRI) and the EULAR SS Disease Activity Index (ESSDAI).

Methods: A prospective international 6-month duration validation study was conducted in 15 countries. At each visit, physicians completed ESSDAI, SS disease activity index (SSDAI), Sjögren's Systemic Clinical Activity Index (SCAI) and physician global assessment (PhGA); and patients completed ESSPRI, Sicca Symptoms Inventory (SSI), Profile of Fatigue and Discomfort (PROFAD) and patient global assessment (PGA).

The effect of methotrexate on improving serological abnormalities of patients with systemic lupus erythematosus.

Objective: This was an open-labeled, prospective, control study to determine the efficacy of methotrexate (MTX) for improving serological abnormalities in patients with systemic lupus erythematosus (SLE).

Methods: Thirty patients with a low serum complement and/or high anti-double-stranded DNA (dsDNA) antibody levels during a prednisolone taper received MTX orally at a dose of 7.5 mg/week over 12-18 months (MTX group).

Proposing a method of regional assessment and a novel outcome measure in rheumatoid arthritis.

We proposed a method of regional assessment in patients with rheumatoid arthritis. The utility of this method was demonstrated by assessing drug efficacy in patients who received infliximab (n = 31) or tocilizumab (n = 6). Joints were divided into four regions: upper/large, upper/small, lower/large, and lower/small.

Efficacy and safety of rebamipide for the treatment of dry mouth symptoms in patients with Sjögren's syndrome: a double-blind placebo-controlled multicenter trial.

The effects of rebamipide on dry mouth and salivary secretion in Sjögren's syndrome patients were investigated in a double-blind placebo-controlled study. Rebamipide (100 mg TID) or placebo was administered for eight weeks and patient-assessed improvement of dry mouth and increase in salivary secretion measured by the Saxon test were evaluated. At two, four, and eight weeks, dry mouth improvement rates were, respectively, 26.

Left ventricular aneurysms developed in a patient with systemic sclerosis.

A 60-year-old woman with systemic sclerosis, lung fibrosis, and primary biliary cirrhosis was admitted to our hospital because of heart failure. Ventricular aneurysms were found in the apex and the posterior wall of the left ventricle by angiocardiography; however, there was no sign of coronary artery stenosis. A myocardial biopsy specimen revealed diffuse focal myocardial fibrosis.

A study to standardize quantitative evaluation of parotid gland scintigraphy in patients with Sjögren's syndrome.

Objective: To standardize quantitative parotid gland scintigraphy for diagnosing Sjögren's syndrome (SS).

Methods: Forty-five patients with SS and 23 controls were studied. Dynamic images were obtained up to 50 min after the injection of 185 MBq 99mTc-pertechnetate and salivary excretion was stimulated with lemon juice orally at 40 min after the injection.

Clinical and serological heterogeneity in patients with anticentromere antibodies.

Objective: To evaluate the clinical and serological heterogeneity in patients with anticentromere antibodies (ACA).

Methods: One hundred twenty patients with ACA were analyzed retrospectively. ACA were detected initially on the basis of indirect immunofluorescence on HEp-2 cells, and then antibodies to CENP-B were measured by ELISA.

Cyclosporin A inhibits the early phase of NF-kappaB/RelA activation induced by CD28 costimulatory signaling to reduce the IL-2 expression in human peripheral T cells.

Cyclosporin A (CsA) inhibits interleukin (IL)-2 production, activation and proliferation of human peripheral T cells (HPTC) costimulated with simultaneous engagement of T cell receptor (TCR)/CD3 and CD28. We demonstrated that 10 ng/ml CsA, which reduced the proliferation of HPTC costimulated with anti-CD3 and anti-CD28 by half, prevented NF-AT and NF-kappaB from migrating into the nucleus. Whereas CsA added even 30 min after the costimulation caused NF-AT to remain in the cytoplasm, the delayed addition of CsA could not prevent NF-kappaB from translocating into the nucleus.