A Case of Visual Hallucination With Frontal Lobe Infarction in a Patient With Giant Cell Arteritis.

Giant cell arteritis (GCA) can produce a variety of visual symptoms. Among these, visual hallucinations are rare and are usually accompanied by visual loss. We encountered a 79-year-old female with GCA who presented with visual hallucinations without visual loss. Magnetic resonance imaging (MRI) of the head revealed a stroke in the right frontal lobe, probably caused by GCA, resulting in visual hallucinations. Visual hallucinations are not well recognized by clinicians as a presentation of GCA. However, as shown in the present case, visual hallucinations are an important symptom because they are suggestive of cerebral ischemia or visual loss.

Current evidence and practical knowledge for ultrasound-guided procedures in rheumatology: Joint aspiration, injection, and other applications.

Ultrasound (US)-guided procedures have increasingly gained their role in the daily practice of rheumatology, owing to the growing evidence supporting their utility. The utilization of US guidance in procedures may enhance their accuracy, efficacy, and safety. This article presents a comprehensive review of the current evidence and practical knowledge pertaining to US-guided procedures in rheumatology, encompassing joint aspirations, injections, and other applications such as tendon sheath injections. We provide a detailed description of the US-guided procedure process and compare the in-plane and out-of-plane view methods, along with practical techniques based on existing evidence or our own expertise. For each joint, we summarize how to perform procedures with figures to facilitate a better understanding. Additionally, we introduce other applications of US-guided procedures for tendons, enthesis, bursae, and nerves as well as emerging therapies such as US-guided fascia hydrorelease. By utilizing these US techniques, rheumatologists can achieve the ability to manage a wider range of musculoskeletal conditions.

Antimicrobial therapy for nontuberculous mycobacterial pulmonary disease improved hearing loss and normalized myeloperoxidase-anti-neutrophil cytoplasmic antibody level: A case report.

Several case reports have indicated that nontuberculous mycobacterial pulmonary disease is associated with anti-neutrophil cytoplasmic antibody-associated vasculitides. However, the effect of the treatment for nontuberculous mycobacterial pulmonary disease on anti-neutrophil cytoplasmic antibody-associated vasculitides remains unclear. An asymptomatic 80-year-old woman presented with nodular bronchiectasis. After 1 year, she developed a productive cough. Mycobacterial culture of the respiratory specimen revealed Mycobacterium avium. She was diagnosed with nontuberculous mycobacterial pulmonary disease based on the criteria proposed by the American Thoracic Society. Concurrently, she had hearing loss, tinnitus, and weight loss. A blood test showed an elevated level of myeloperoxidase-anti-neutrophil cytoplasmic antibody (107 IU/mL, normal level: <3.5 IU/mL). Bilateral otitis media with anti-neutrophil cytoplasmic antibody-associated vasculitis was diagnosed based on the diagnostic criteria proposed by the Japan Otological Society. After starting antimicrobial agents for the nontuberculous mycobacterial pulmonary disease, her pulmonary symptoms and hearing loss improved, and the level of myeloperoxidase-anti-neutrophil cytoplasmic antibody normalized. No immunosuppressive treatment was administered. The present case suggests that nontuberculous mycobacterial pulmonary disease can cause otitis media with anti-neutrophil cytoplasmic antibody-associated vasculitides, and antimicrobial treatment for the nontuberculous mycobacterial pulmonary disease may resolve otitis media with anti-neutrophil cytoplasmic antibody-associated vasculitides.

A case report of two systemic lupus erythematosus pregnancies with early placental exposure to belimumab: Case report with review.

Since its approval for the management of systemic lupus erythematosus (SLE), belimumab has been widely used. However, its pregnancy safety profile has been underinvestigated. We present the pregnancy outcomes of two cases of early placental exposure to belimumab and summarise the pregnancy outcomes in previous reports regarding placental exposure to belimumab. Case 1 describes a 27-year-old woman with an 18-year history of SLE and lupus nephritis class III. We introduced belimumab 19 months prior to conception to control her proteinuria and discontinued its use at 5 weeks and 5 days of gestation. Her lupus activity was stable throughout pregnancy, and at 37 weeks and 1 day of gestation, she delivered a healthy girl with no anomaly. At delivery, the girl was small for gestational age, but at the 1-year follow-up, there was no delay in her growth or any serious infection. Case 2 describes a 32-year-old woman with a 15-year history of SLE. We introduced belimumab 9 months prior to conception and discontinued its use at 7 weeks and 1 day of gestation. Although her lupus was well controlled without belimumab, a missed abortion occurred, which was possibly due to foetal factors. Although there is accumulating data on the safety of belimumab use during pregnancy, it seems necessary to cautiously use this medication in pregnant women, until further analyses are conducted.

Effectiveness of ultrasound-guided fascia hydrorelease on the coracohumeral ligament in patients with global limitation of the shoulder range of motion: a pilot study.

We conducted a prospective single-arm interventional study of the treatment efficacy of ultrasound-guided fascia hydrorelease (US-FHR) on the coracohumeral ligament (CHL) of patients with global limitation of shoulder range of motion (ROM) without local inflammation. The primary outcome was the change in passive ROM (pROM) of external rotation (ER) after first US-FHR. Secondary outcomes included the change in pROM of other directions from baseline, the pain visual analogue scale (pVAS) at the timepoints after each procedure (first, second US-FHR and rehabilitation) as well as the change in the Shoulder Pain and Disability Index (SPADI) from the first to the second visit. Eleven patients underwent US-FHR. The pROM of ER after the 1st US-FHR changed by a median of 7.1° (p < 0.01). There was a statistically significant improvement in the pROM of flexion, extension, abduction, external rotation, and internal rotation from baseline to each timepoints. The pVAS at rest showed no significant improvement, although the pVAS at maximal ER showed a trend towards improvement. The SPADI score decreased by a median of 13.4 (p < 0.01). No adverse events were observed. US-FHR on the CHL with or without rehabilitation might be an effective, less invasive treatment for patients with global limitation of shoulder ROM.

Advantages of an alternate-day glucocorticoid treatment strategy for the treatment of IgG4-related disease: A preliminary retrospective cohort study.

Alternate-day glucocorticoid (GC) therapy is a treatment option that can reduce GC-associated adverse events. We investigated the safety and efficacy of alternate-day GC therapy in patients with immunoglobulin G4-related disease (IgG4-RD). Medical records of patients with IgG4-RD who were followed for at least one year at St. Luke's International Hospital, Tokyo, Japan, from 2004 to 2020 were reviewed. Patients who fulfilled comprehensive IgG4-RD diagnostic criteria were divided into alternate-day or daily GC treatment groups based on their treatment protocol. The effect of alternate-day GC therapy on glucocorticoid toxicity index (GTI) score was evaluated using multilinear analysis with adjustments for cumulative GC doses until each assessment point and propensity scores (PS) for alternate-day GC therapy. Kaplan-Meier curves and Cox proportional hazard models were used to assess the efficacy of alternate-day GC therapy for disease control. Among the 67 patients with IgG4-RD, patients with alternate-day (n = 13) and daily (n = 31) GC treatments were analyzed after excluding 23 ineligible patients. The median (interquartile range) age was 64 (60-70) years, 29 (65.9%) were male patients, 26 (59.1%) patients had positive biopsy results, and the median follow-up period was 1643 days. Significantly more patients with alternate-day GC treatment used concomitant immunosuppressants (11 [84.6%] vs 11 [35.5%]; P = .007). The alternate-day strategy significantly lowered the GTI score after adjusting for cumulative GC dose until the assessment and PS (adjusted coefficient: -29.5 [-54.3, -4.8], P = .021 at 12 months; -20.0 [-39.8, -0.1], P = .049 at 24 months). Serious infections were numerically less frequent in the alternate-day group (incidence ratio [95% confidence interval [CI]: 0.45 [0.05, 3.63], P = .45). Most patients (92.3%) in the alternate-day GC treatment group and all patients in the daily GC treatment group showed treatment responses in the remission induction therapy. The PS-adjusted hazard ratio of alternate-day GC treatment for disease flares was not significant (1.55 [0.53, 4.51]; P = .43). The alternate-day treatment strategy significantly reduced GC-related adverse events regardless of the cumulative GC dose. Alternate-day GC treatment is a feasible option for patients with IgG4-RD, without a significant increase in disease flares particularly when combined with immunosuppressants.

Glucocorticoid discontinuation in patients with SLE with prior severe organ involvement: a single-center retrospective analysis.

OBJECTIVE: Long-term glucocorticoid use in SLE may have significant side effects; however, glucocorticoid discontinuation is occasionally associated with disease flare-ups. Therefore, we evaluated the risk factors for disease flares and the flare rate on glucocorticoid tapering in patients with prior severe organ involvement. METHODS: Data of patients with SLE with glucocorticoid tapering at our institution were retrospectively analysed. We divided the patients by the presence of prior severe organ involvement and compared flare rates after glucocorticoid discontinuation. Furthermore, we determined risk factors for flares after glucocorticoid discontinuation. RESULTS: In total, 309 patients with SLE were screened, 73 of whom met the inclusion criteria; 49 were classified as SLE with prior severe organ involvement. No significant differences were noted in the 52-week flare rate after glucocorticoid discontinuation between patients with and without prior severe organ involvement (16.7% vs 18.2%, p=1.0). Hypocomplementaemia, elevated anti-dsDNA antibody titres more than twice the upper limit of the laboratory reference range, positive anti-Smith/anti-ribonucleoprotein antibody, and use of any immunosuppressant on the day of glucocorticoid discontinuation were negatively associated with flare-free remission. CONCLUSIONS: Glucocorticoid discontinuation after gradual tapering can often be achieved in patients with SLE, even with prior severe organ involvement, especially when the disease is clinically and serologically stable.

Performance of a pre-administration infection screening questionnaire in patients with rheumatoid arthritis administered biological disease-modifying antirheumatic drugs.

AIM: Pre-administration screening of active infections is imperative for the safe use of biological disease-modifying antirheumatic drugs (bDMARDs) in patients with rheumatoid arthritis (RA). However, a standardized screening method is lacking. We therefore implemented a novel systematic screening method with a simple predetermined questionnaire on infections and assessed its effectiveness. METHODS: We retrospectively reviewed medical records of individuals for whom intravenous bDMARDs were administered for RA from January 2016 to April 2019. We evaluated the performance of the new screening method based on physicians' assessments. In addition, a survey was administered to nurses, regarding their assessment of the usefulness of this new screening. The incidence of infections was also assessed. RESULTS: A total of 1636 cases underwent this new screening. The new screening method showed high sensitivity (0.97) and specificity (0.89) with a negative predictive value of 99.9%, as determined based on the physician's decision. Administration of bDMARDs was postponed in 37 (2.5%) patients, and there was only one case in which the screening failed to note an active infection. The nurses' survey demonstrated high agreement (87.5%) about the usefulness of this screening on the grounds of clarity, simplicity, ease, and time-saving effects. There was no significant increase in infections after implementation of this method. CONCLUSIONS: Systematic screening with a predetermined simple questionnaire is effective as an infection screening method, with a high negative predictive value. This approach contributes to high satisfaction of nurses and a time-efficient practice by focusing on screen-positive cases without increasing infections.

Effectiveness and safety of mizoribine for the treatment of IgG4-related disease: a retrospective cohort study.

OBJECTIVE: Patients with IgG4-related disease (IgG4RD) usually require steroid-sparing agents due to relapse with tapering glucocorticoids (GC). We aimed to determine the efficacy and safety of mizoribine (MZR) among IgG4RD patients. METHODS: We retrospectively reviewed records of IgG4RD patients at Immuno-Rheumatology Center in St. Luke's International Hospital, Tokyo, Japan. Patients treated with MZR were classified into the MZR group, and those treated with GC alone or with other immunosuppressants were included in the control group. Disease exacerbation, GC dose, IgG-IgG4 titre and adverse events were evaluated using univariate analyses, including the Kaplan-Meier method. The Cox proportional hazard model was used to evaluate risk factors for disease exacerbation. RESULTS: A total of 14 and 29 cases were included in the MZR and control group. Multiple organ involvement (three or more organs) was significantly more frequent in the MZR group [10 (71.4%) vs 9 (31.0%), P= 0.021]. Kaplan-Meier analysis revealed a significant reduction inexacerbation in patients with multiple organ involvement (P< 0.001) but not in total (P= 0.42). The adjusted hazard ratios of MZR use and multiple organ involvement for exacerbation were 0.34 (95%CI 0.12-1.01; P = 0.052) and 3.51 (95%CI 1.29-9.51; P= 0.014). The cumulative GC dose (mg per year, interquartile range) tended to be lower in the MZR group [1448 (1003-1642) vs 2179 (1264-3425); P= 0.09]. CONCLUSION: MZR decreased disease exacerbation among IgG4RD patients with multi-organ involvement and showed a steroid-sparing effect. MZR could be a treatment option for IgG4RD.

Validation of the 2019 ACR/EULAR classification criteria of systemic lupus erythematosus in 100 Japanese patients: a real-world setting analysis.

The objective of the study is to evaluate the sensitivity of the new criteria for the classification of systemic lupus erythematosus (SLE), when applied to real SLE cases. We retrospectively reviewed the electronic medical records of 100 consecutive patients who visited St. Luke's International Hospital. Patients were included if they were clinically diagnosed as having SLE and excluded if they had other autoimmune disease or if they were less than 18 years old. Each patient was evaluated if they satisfied the American College of Rheumatology (ACR) 1997 classification criteria (1997 criteria), 2012 Systemic Lupus International Collaborating Clinics criteria (2012 criteria), or 2019 ACR/European League Against Rheumatism (EULAR) criteria. Among the 100 patients, the sensitivity of the 1997, 2012, and 2019 criteria was, 97, 99, and 92%, respectively. The total patient score with the 2019 criteria ranged from 12 to 44 (mean, 27.3). All patients who were classified as non-SLE with the 2019 criteria had an anti-nuclear antibody (ANA) titre of < 1:80. The 2019 criteria for SLE accomplished modestly high sensitivity in the real-world practice, but not as high as the 1997 and 2012 criteria. They possibly misclassify the real SLE cases as non-SLE, especially if patients have a low titre (< 1:80) of ANA.

Clinical Characteristics of Patients with Spondyloarthritis in Japan in Comparison with Other Regions of the World.

OBJECTIVE: To delineate clinical characteristics of patients with spondyloarthritis (SpA) in Japan in comparison to other areas of the world. METHODS: Using the ASAS-COMOSPA (Assessment of Spondyloarthritis international Society-COMOrbidities in SPondyloArthritis) data, an international cross-sectional observational study of patients with SpA, we analyzed information on demographics, disease characteristics, comorbidities, and risk factors. Patients were classified by region: Japan, other Asian countries (China, Singapore, South Korea, Taiwan), and non-Asian countries (Europe, the Americas, Africa). Patient characteristics, including diagnosis and treatment, were compared. RESULTS: Among 3984 patients included in the study, 161 were from centers in Japan, 933 from other Asian countries, and 2890 from other regions. Of patients with SpA in Japan, 42 (26.1%) had peripheral SpA, substantially more than in other countries. This trend was explained by the predominance of psoriatic arthritis (PsA) among Japanese patients with SpA. In contrast to the relatively low number in Japan, 54% of patients from other Asian countries had pure axial SpA (axSpA) without peripheral features. HLA-B27 testing, considered an integral part of the classification of axSpA, was performed in only 63.6% of Japanese patients with axSpA. More than half of Japanese patients with axSpA were classified using imaging criteria. CONCLUSION: In our study, there was a more substantial number of peripheral SpA cases observed in Japan compared to other parts of Asia and other regions of the world. Aside from ethnic differences, increasing recognition of PsA in Japan, as well as a potential underdiagnosis of axSpA due to the insufficient use of HLA-B27 testing, may partly explain regional discrepancies.

Concomitant use of intravenous methylprednisolone to increase retention rate of abatacept in rheumatoid arthritis.

To investigate whether concomitant use of intravenous methylprednisolone (IVMP) with Abatacept (ABA) contributes to earlier remission and higher drug retention rates. This was a retrospective cohort study to assess the retention rate of ABA in rheumatoid arthritis (RA) patients treated at a single center in Japan from January 2010 to May 2017. Patients were divided into an ABA monotherapy group and ABA IVMP group. IVMP (40 mg) was administered with the first three consecutive doses of ABA. ABA retention rates were evaluated using Kaplan-Meier analysis. Hazard ratios for the drug retention rate were also calculated as the sensitivity analysis. A total of 59 seropositive RA patients were analyzed. Twelve patients were treated with ABA IVMP, and 47 patients were treated with ABA monotherapy. The overall ABA retention rate was 76.3% at 24 weeks. The retention rates were 91.7 and 72.3% in the ABA IVMP and ABA monotherapy groups, respectively. Log-rank analysis revealed no statistical difference between the two groups (p = 0.17). The sensitivity analysis showed that the hazard ratio of IVMP was 2.9-3.7 in three models, although there was no statistical significance. Safety analysis revealed that no patients discontinued ABA because of adverse events in the ABA IVMP group, while 7/47 (14.9%) discontinued the drug in the ABA monotherapy group. In this real life study, ABA, concomitantly used with IVMP, showed numerically higher retention rates without additional safety signals, although there was no statistical significance. Concomitant use of IVMP may help RA patients achieve earlier remission.

Effects of quality indicator monitoring for glucocorticoid-induced osteoporosis and trends of drug treatment in a Japanese hospital.

AIMS: Globally, the appropriate prescription rate for glucocorticoid-induced osteoporosis (GIOP) is low. Thus, we aimed to examine the improvement in real-world GIOP care using a hospital-wide systematic approach with quality indicator (QI) monitoring. METHODS: We defined a novel QI for GIOP care for the prescription rate of anti-osteoporotic drugs according to 2010 American College of Rheumatology GIOP management recommendations, with the target being patients prescribed ≥7.5 mg prednisolone daily or its equivalent for ≥3 months. We monitored the glucocorticoid and osteoporotic medication dose for all patients who visited our hospital. From May 2011, we began interventions to improve QI: monthly QI monitoring providing QI-trend feedback to each department in a hospital-wide QI meeting every 3 months and organizing lectures on GIOP. We retrospectively analyzed QI trends from 2010 to 2013. We categorized groups by sex and age for subanalyses: group A, men; group B, women, aged <50 years; group C, women, aged ≥50 years. RESULTS: The numbers of participants were 401, 420, 520 and 513 in 2010, 2011, 2012 and 2013, respectively, with pooled QI rates of 45.8%, 51.3%, 55.0% and 54.8%, respectively. Changes in QI between each consecutive 2 years were statistically significant. Subanalyses showed statistically significant QI improvements in groups A and C. We observed a decreasing trend of daily bisphosphonate use throughout the study period, especially at the Immuno-Rheumatology Center. CONCLUSIONS: Quality indicator monitoring for GIOP significantly improved appropriate anti-osteoporotic drug prescriptions, especially in men and postmenopausal women.

Safety and efficacy of alternate-day corticosteroid treatment as adjunctive therapy for rheumatoid arthritis: a comparative study.

Corticosteroids (CSs), used to treat rheumatoid arthritis (RA), confer a risk of adverse events (AEs). This study investigated the safety and efficacy of alternate-day (QOD) CS therapy for RA. All patients (> 18 years) who started oral CS therapy for RA, between 2005 and 2014, at our hospital were retrospectively analysed. The patients were divided into the daily (QD) and QOD CS therapy groups to investigate the rates of CS-related major AEs (infection, diabetes, hypertension, cardiovascular events and fragility fractures) within the first year of treatment. The number of patients free from CS treatment at 1 year and the mean decreases in C-reactive protein (CRP) levels at 1 month were also investigated. In total, 138 patients were analysed (QD group, 68; QOD group, 70). The maximum daily CS dose was not significantly different between the two groups, but the annual cumulative dose was significantly lower in the QOD group (P < 0.01). The infection rate was significantly lower in the QOD group (24.3%) than in the QD group (50.0%; P < 0.01), whereas the other AE rates were similar between the groups. The CS-free rate at 1 year was significantly higher in the QOD group (58.6%) than in the QD group (26.5%; P < 0.01). The mean CRP decreases over 1 month of CS therapy were not significantly different between the groups. QOD CS treatment leads to a lower infection rate and less CS dependence than does daily treatment; both RA treatments are equally effective.

Mizoribine is as Effective as Methotrexate for the Treatment of Polymyalgia Rheumatica: A Retrospective Case Series Analysis.

OBJECTIVES: This study aims to evaluate the efficacy and safety of mizoribine (MZR) as a steroid-sparing agent compared to methotrexate (MTX) in the treatment of polymyalgia rheumatica in elderly patients. PATIENTS AND METHODS: Twenty-four patients (9 males, 15 females; mean age 71.7 years; range 50 to 86 years) diagnosed with polymyalgia rheumatica between April 1998 and August 2014, who received prednisone in combination with either MTX or MZR, were included. We collected the data on the cumulative prednisone dose that patients received within 48 weeks after MTX or MZR and its side effect profile. RESULTS: There were 10 patients in the MTX group and 14 in the MZR group. The cumulative prednisone dose over 0-48 weeks was 2272±396 mg in the MTX group and 1907±241 mg in the MZR group, which was not significantly different (p=0.41). In terms of side effects, in the MTX group, three patients experienced a transient elevation in liver enzymes, and one patient developed gastrointestinal symptoms that led to MTX withdrawal. In the MZR group, one patient was hospitalized due to pneumonia that led to MZR withdrawal. CONCLUSION: Mizoribine was non-inferior to MTX in terms of steroid-sparing effects on polymyalgia rheumatica. Also, MZR tended to have fewer side effects than MTX.