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BACKGROUND: The characteristics of patients with advanced chronic kidney disease (CKD) who are recipients of public assistance in Japan, and the adequacy of their medical care have not been reported previously. METHODS: The records of patients with CKD stage G5 who visited nine facilities in Japan from April to June 2013 were retrospectively reviewed to compare the characteristics and care of recipients of public assistance with those of non-recipients. Receiving a presentation of kidney replacement therapy (KRT) options and polypharmacy were used as indicators of suboptimal medical care. RESULTS: Of the 592 patients included in this analysis (mean age, 69.6 years; male, 59.3%), 56 (9.5%) were recipients of public assistance and 536 (90.5%) were non-recipients of public assistance. The prevalence of diabetes mellitus, unmarried status, and living alone were higher in recipients of public assistance. In multivariable logistic regression analysis, compared with non-recipients of public assistance, recipients of public assistance were less likely to receive a presentation of KRT options (adjusted odds ratio [aOR], 0.31; 95% confidence interval [CI], 0.17-0.56), and were more likely to receive ≥ 10 (aOR, 1.92; 95% CI, 1.05-3.51), and ≥ 15 (aOR, 2.78; 95% CI, 1.23-6.26) types of medication. CONCLUSIONS: Patients with advanced CKD receiving public assistance were less likely to receive a presentation of KRT options and more likely to receive ≥ 10 and ≥ 15 types of medication, suggesting that recipients of public assistance are more likely to receive suboptimal medical care.
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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. Baseline characteristics were compared between the RTX and non-RTX groups. We performed multivariate logistic regression analysis and one-to-one propensity score matching analysis as a sensitivity analysis. Totally, 100 patients were enrolled: 52 in the RTX group and 48 in the non-RTX group. Baseline characteristics were comparable between the two groups, except for age, AAV subtype and ANCA serotype. The median age was 71 vs. 75 years, and the PR3-ANCA positivity rate was 44.2% vs. 18.8% in the RTX and non-RTX groups, respectively. No significant difference was observed in CR proportions at week 24 between the two groups (79.2% vs. 68.1%, p = 0.321), with an adjusted odds ratio of 1.27 (95% confidence interval [CI] 0.47-3.51). At week 48, CR proportions were significantly higher in the RTX group (91.7% vs. 64.9%, p = 0.005), with an adjusted odds ratio of 2.95 (95% CI 0.97-9.91). Serious infection rates were lower in the RTX group than in the non-RTX group, with no statistically significant difference. RTX was not superior to conventional immunosuppressive therapies at week 24 but showed significantly favourable results at week 48 for severely relapsed AAV.
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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-randomised into three groups using propensity score-based overlap weighting as follows: non-IVMP, IVMP 0.5 g/day, and IVMP 1.0 g/day. The primary outcome was all-cause death, and the secondary outcomes were composite all-cause death and kidney failure, severe relapse, and serious infection from 2 to 48 weeks after treatment initiation. To estimate the treatment effects, the Cox proportional hazard model and Fine-Gray subdistribution hazard model were used. RESULTS: In this emulated target trial, of 201 eligible patients (MPA, 175; GPA, 26), 6 (2.8%) died, 4 (2.0%) had kidney failure, 11 (5.3%) had severe relapse, and 40 (19.8%) had severe infections. Hazard ratios (HR) for IVMP 0.5 g/day and IVMP 1.0 g/day pulse groups compared with non-IVMP pulse were as follows: all-cause death = 0.46 (95% confidence interval [95%CI]: 0.07-2.81) and 0.07 (95%CI: 0.01-0.41); all-cause death/kidney failure = 1.18 (95%CI: 0.26-5.31) and 0.59 (95%CI: 0.08-4.52); subdistribution HRs for severe relapse = 1.26 (95%CI: 0.12-13.70) and 3.36 (95%CI: 0.49-23.29); and serious infection = 1.88 (95%CI: 0.76-4.65) and 0.94 (95%CI: 0.28-3.13). CONCLUSIONS: IVMP 1.0 g/day pulse may improve 48-week mortality in patients with severe MPA/GPA.
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OBJECTIVE: To clarify seasonal and other environmental effects on the onset of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: We enrolled patients with new-onset eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA), and granulomatosis with polyangiitis (GPA) registered in the database of a Japanese multicenter cohort study. We investigated the relationship between environmental factors and clinical characteristics. Seasons were divided into 4 (spring, summer, autumn, and winter), and the seasonal differences in AAV onset were analyzed using Pearson chi-square test, with an expected probability of 25% for each season. RESULTS: A total of 454 patients were enrolled, with a mean age of 70.9 years and a female proportion of 55.5%. Overall, 74, 291, and 89 patients were classified as having EGPA, MPA, and GPA, respectively. Positivity for myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA was observed in 355 and 46 patients, respectively. Overall, the seasonality of AAV onset significantly deviated from the expected 25% for each season (P = 0.001), and its onset was less frequently observed in autumn. In ANCA serotypes, seasonality was significant in patients with MPO-ANCA (P < 0.001), but not in those with PR3-ANCA (P = 0.97). Additionally, rural residency of patients with AAV was associated with PR3-ANCA positivity and biopsy-proven pulmonary vasculitis. CONCLUSION: The onset of AAV was influenced by seasonal variations and was less frequently observed in autumn. In contrast, the occurrence of PR3-ANCA was triggered, not by season, but by rural residency.
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Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Poliangiite Microscópica , Humanos , Feminino , Idoso , Granulomatose com Poliangiite/complicações , Anticorpos Anticitoplasma de Neutrófilos , Estações do Ano , Síndrome de Churg-Strauss/complicações , Estudos Retrospectivos , Estudos de Coortes , Japão/epidemiologia , Mieloblastina , Poliangiite Microscópica/complicações , PeroxidaseRESUMO
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. After multiple imputations for all explanatory variables, we performed the multivariate analysis using a Fine-Gray model to assess the association between low IgG (the minimum IgG levels <500 mg/dl) and severe infections. In addition, the association was expressed as a restricted cubic spline (RCS) and analysed by treatment subgroups. RESULTS: Of 657 included patients (microscopic polyangiitis, 392; granulomatosis with polyangiitis, 139; eosinophilic granulomatosis with polyangiitis, 126), 111 (16.9%) developed severe infections. The minimum serum IgG levels were measured in 510 patients, of whom 77 (15.1%) had low IgG. After multiple imputations, the confounder-adjusted hazard ratio of low IgG for the incidence of severe infections was 1.75 (95% confidence interval: 1.03-3.00). The RCS revealed a U-shaped association between serum IgG levels and the incidence of severe infection with serum IgG 946 mg/dl as the lowest point. Subgroup analysis showed no obvious heterogeneity between treatment regimens. CONCLUSION: Regardless of treatment regimens, low IgG after remission-induction treatment was associated with the development of severe infections up to 48 weeks after treatment initiation.
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Agamaglobulinemia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Poliangiite Microscópica , Humanos , Granulomatose com Poliangiite/tratamento farmacológico , Estudos Retrospectivos , Agamaglobulinemia/induzido quimicamente , Quimioterapia de Indução , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Poliangiite Microscópica/tratamento farmacológico , Imunoglobulina G/uso terapêutico , Anticorpos Anticitoplasma de NeutrófilosRESUMO
BACKGROUND: This study investigated the characteristics of hypertrophic pachymeningitis (HP) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), using information from a multicenter study in Japan. METHODS: We analyzed the clinical information of 663 Asian patients with AAV (total AAV), including 558 patients with newly diagnosed AAV and 105 with relapsed AAV. Clinical findings were compared between patients with and without HP. To elucidate the relevant manifestations for HP development, multivariable logistic regression analyses were additionally performed. RESULTS: Of the patients with AAV (mean age, 70.2 ± 13.5 years), HP was noted in 30 (4.52%), including 20 (3.58%) with newly diagnosed AAV and 10 (9.52%) with relapsed AAV. Granulomatosis with polyangiitis (GPA) was classified in 50% of patients with HP. A higher prevalence of GPA was significantly observed in patients with HP than in those without HP in total AAV and newly diagnosed AAV (p < 0.001). In newly diagnosed AAV, serum proteinase 3 (PR3)-ANCA positivity was significantly higher in patients with HP than in those without HP (p = 0.030). Patients with HP significantly had ear, nose, and throat (ENT) (odds ratio [OR] 1.48, 95% confidence interval [CI] 1.03-2.14, p = 0.033) and mucous membrane/eye manifestations (OR 5.99, 95% CI 2.59-13.86, p < 0.0001) in total AAV. Moreover, they significantly had conductive hearing loss (OR 11.6, 95% CI 4.51-29.57, p < 0.0001) and sudden visual loss (OR 20.9, 95% CI 5.24-85.03, p < 0.0001). CONCLUSION: GPA was predominantly observed in patients with HP. Furthermore, in newly diagnosed AAV, patients with HP showed significantly higher PR3-ANCA positivity than those without HP. The ear and eye manifestations may be implicated in HP development.
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Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Granulomatose com Poliangiite , Meningite , Idoso , Idoso de 80 Anos ou mais , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/epidemiologia , Anticorpos Anticitoplasma de Neutrófilos , Estudos Transversais , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/epidemiologia , Humanos , Hipertrofia , Japão/epidemiologia , Meningite/epidemiologia , Pessoa de Meia-Idade , Mieloblastina , Peroxidase , Estudos RetrospectivosRESUMO
We herein report a case of a combined crystalline light chain tubulopathy, podocytopathy, histiocytosis, and cast nephropathy in a patient with monoclonal gammopathy of renal significance (MGRS). A 66-year-old female with impaired renal function was referred to our department. Despite intravenous fluid resuscitation, the kidney function worsened progressively; thus, a kidney biopsy was performed. The kidney biopsy revealed light chain proximal tubulopathy (LCPT) with crystals, light chain crystal podocytopathy (LCCP), crystal-storing histiocytosis (CSH), and light chain cast nephropathy (LCCN). Of note, LCCP and CSH were diagnosed via electron microscopy. Serum and urine immunoelectrophoresis (IEP) revealed the presence of monoclonal Bence-Jones protein and free κ light chains. Bone marrow aspiration showed < 10% plasma cell proliferation. Thus, we had encountered a rare case in which a variety of kidney lesions were combined with MGRS. Most of the LCPT, LCCP, and CSH cases show monoclonal IgG κ, while our case showed Bence-Jones protein κ.
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Proteína de Bence Jones/isolamento & purificação , Histiocitose/complicações , Nefropatias/diagnóstico , Idoso , Feminino , Humanos , Cadeias kappa de Imunoglobulina , Nefropatias/etiologia , Túbulos Renais Proximais/patologia , Microscopia Imunoeletrônica , Podócitos/patologiaRESUMO
INTRODUCTION: Anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) is an autoantigen associated with dermatomyositis (DM). Anti-MDA5 Ab-positive DM patients frequently exhibit clinically amyopathic dermatomyositis (CADM), and develop rapidly progressive interstitial lung disease (RPILD). Even with early detection and potent combination immunosuppressive therapy, anti-MDA5 Ab-positive DM patients have a poor prognosis. In the present case report, we present a rare autopsy case of a patient with anti-MDA5 Ab DM with RPILD who exhibited diffuse alveolar damage (DAD) patterning in lung specimens, and extensive hemorrhages in multiple organs. PATIENT CONCERNS: An 82-year-old Japanese man admitted with bacterial pneumonia was subsequently diagnosed with anti-MDA5 Ab-positive DM based on skin manifestations (mechanic's hand, ulcerated palmar papules, and flagellate erythema), myositis, interstitial pneumonia, and elevation of anti-MDA5 Ab titer. DIAGNOSIS: The patient was diagnosed with anti-MDA5 Ab DM, complicated with RPILD. INTERVENTIONS: The patient received potent immunosuppressive therapy consisting of pulse methylpredonisolone at a dose of 1000âmg for 3 days, followed by prednisolone at 60âmg/d, a 1000âmg pulse of intravenous cyclophosphamide (IVCY), and oral tacrolimus at 6âmg/d. Intravenous immunoglobulin (IVIG) at a dose of 400âmg/kg/d for 5 days was subsequently administered. OUTCOMES: Despite triple immunosuppressive therapy and IVIG, the patients' respiratory status deteriorated, and the patient died of respiratory failure on the twelfth day after admission. An autopsy revealed pulmonary DAD and multiorgan hemorrhages, including the left iliopsoas muscle, gastric and bowl mucosa, spleen, and left adrenal gland. LESSONS: Multiorgan hemorrhages may be a fatal complication in anti-MDA5 Ab DM patients.
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Dermatomiosite/complicações , Dermatomiosite/imunologia , Hemorragia/etiologia , Helicase IFIH1 Induzida por Interferon/imunologia , Idoso de 80 Anos ou mais , Autoanticorpos/imunologia , Autopsia , Dermatomiosite/tratamento farmacológico , Humanos , Imunossupressores/uso terapêutico , Pulmão/fisiopatologia , MasculinoRESUMO
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.
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Antígeno HLA-B27/sangue , Espondilartrite/diagnóstico , Adulto , Estudos Transversais , Feminino , Pesquisas sobre Atenção à Saúde , Humanos , Japão , Masculino , Pessoa de Meia-Idade , Espondilartrite/sangue , Espondilartrite/diagnóstico por imagem , Adulto JovemAssuntos
Artrite Reumatoide/complicações , Articulação Atlantoaxial/patologia , Calcinose/complicações , Ligamentos Articulares/patologia , Cervicalgia/etiologia , Adulto , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/tratamento farmacológico , Articulação Atlantoaxial/diagnóstico por imagem , Calcinose/diagnóstico , Feminino , Seguimentos , Humanos , Imageamento por Ressonância Magnética/métodos , Cervicalgia/diagnóstico , Cervicalgia/tratamento farmacológico , Medição da Dor , Prednisolona/administração & dosagem , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X/métodosRESUMO
OBJECTIVE: To investigate the prevalence of psoriatic arthritis (PsA) in Japanese patients with psoriasis. METHODS: A multicenter, noninterventional, retrospective cross-sectional study was conducted at 3 tertiary care centers in Japan. PsA was diagnosed by rheumatologists based on clinical findings. Prevalence of PsA, clinical characteristics, comorbidities, and treatment patterns were examined. RESULTS: PsA was identified in 431 of 3021 patients with psoriasis, with a mean prevalence of 14.3% (range, 8.8-20.4%). No large differences between these results and previous reports from Western countries were observed in arthritis distribution, skin disease type, or treatment selection. CONCLUSION: The prevalence of PsA in patients with psoriasis in Japan approaches 20% in some areas, similar to that observed in Western countries, and is higher than previously reported in Asia. Clinical features including age, sex, age at onset, and manifestation patterns were also similar to those reported in the West.