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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. The secondary endpoints included the changes in the Saxon's test, Schirmer's test, ESSDAI and ESSPRI. Adverse events and adherence rates were also analyzed., Results: 68 patients (36 in ROSE and 32 in ROSE II, all women) were enrolled. SDAI decreased significantly from 23.6 ± 13.2 at baseline to 9.9 ± 9.5 at 52 weeks. Patients with SDAI remission increased from 0 (0 weeks) to 19 patients (27.9%) at 52 weeks. Saliva volume increased significantly at 24 weeks. Tear volume increased significantly at 52 weeks. Both ESSDAI and ESSPRI were significantly decreased at 12 weeks, and these responses were maintained up to 52 weeks. The rate of adherence to abatacept over the 52-week period was 83.8%. Twenty-two adverse events occurred in 15 patients., Conclusion: Abatacept ameliorated both glandular and extraglandular involvements, as well as the systemic disease activities and patient-reported outcomes based on composite measures, in SS associated with RA., (© Japan College of Rheumatology 2022. Published by Oxford University Press.)

Objectives: Nontuberculous mycobacterial pulmonary disease (NTM-PD) is a rare but important comorbidity of rheumatoid arthritis (RA). Our objective was to investigate the association between NTM-PD and RA, especially regarding the immunosuppressive treatment of RA such as biological disease-modifying antirheumatic drugs (bDMARDs)., Methods: We conducted a retrospective, single-centre cohort study. All RA patients regularly followed up at our rheumatology division in December 2012 were included in the study, and followed for 5 years., Results: At baseline, 26 of 1639 RA patients had NTM-PD. During the observation period, 14 were newly diagnosed with NTM-PD. For new diagnosis of NTM-PD, bDMARD use at baseline was not a significant risk factor. Among the 40 patients with NTM-PD, 16 were treated with a total of 27 bDMARDs after NTM-PD diagnosis. They did not present with a greater exacerbation of NTM-PD than those not treated with bDMARDs (25 vs. 17%, p = .52). A total of 55 patients died, but nobody died of NTM-PD. NTM-PD was not associated with worse mortality in multivariate analysis (hazard ratio, 2.0; 95% CI, 0.6-6.4; p = .26)., Conclusions: Biological DMARD was not associated with worse prognosis of NTM-PD. Careful use of bDMARDs could be tolerated in RA patients with NTM-PD., (© Japan College of Rheumatology 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

An 83-year-old woman with a history of asthma complained of left abdominal pain and was admitted to our hospital. She was treated with tocilizumab, an anti-interleukin (IL)-6 receptor antibody, with a prolonged interval for rheumatoid arthritis (RA). Laboratory tests revealed a remarkable increase in eosinophil count and inflammatory markers with negative antineutrophil cytoplasmic antibodies. Echocardiography revealed pericardial fluid retention, and contrast-enhanced computed tomography revealed the thickening of the gastric antrum wall. Upper gastrointestinal endoscopy and biopsy revealed eosinophilic infiltration into the gastric mucosal epithelium. She was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) with pericarditis and eosinophilic gastroenteritis. High-dose glucocorticoids with intermittent intravenous cyclophosphamide (IVCY) were initiated, resulting in remission. As IL-6 is involved in the pathogenesis of allergic diseases such as asthma, our case can provide insights into the pathogenic role of IL-6 in EGPA as the development of EGPA in our case may have been triggered by IL-6 signals enhanced with tocilizumab interval prolongation., (© Japan College of Rheumatology 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: To identify biomarkers for assessing myositis-associated interstitial lung disease (ILD)., Methods: We reviewed consecutive patients from our institution who had been newly diagnosed with PM, DM, or clinically amyopathic DM during the years 2002-2017. The patients were divided into two groups according to the presence of ILD, and the ILD group was further subdivided into three groups according to the clinical courses of induction failure, relapse and non-relapse. Baseline and time-course changes in the parameters were compared between groups., Results: Among 110 patients enrolled, 75 (68%) had ILD. Baseline serum Krebs von den Lungen-6 (KL-6) was significantly higher in the ILD group than in the non-ILD group (1120 vs 236 U/ml; P < 0.001). In the ILD group consisting of the induction failure cases (n = 3), the relapse group (n = 24) and the non-relapse group (n = 48), baseline serum KL-6 was significantly different between the three groups [1971 vs 1870 vs 935 U/ml, respectively; P = 0.003 (relapse group vs non-relapse group)]. The time-course changes in serum KL-6 revealed that KL-6 significantly increased along with relapse, with the increase of 625 U/ml relevant to relapse., Conclusion: Serum KL-6 is a useful biomarker for assessing the disease activity of myositis-associated ILD., (© The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: To identify initial predictors of poor survival in patients with PM/DM-associated interstitial lung disease (ILD)., Methods: We established a multicentre retrospective cohort of incident cases of PM/DM-associated ILD from 44 institutions across Japan (Multicentre Retrospective Cohort of Japanese Patients with Myositis-associated ILD, JAMI). Inclusion criteria were an onset age ⩾16 years; PM/DM or clinically amyopathic DM according to the published criteria; imaging evidence of ILD; and availability of serum samples for assays of autoantibodies such as anti-melanoma differentiation-associated gene 5 and anti-aminoacyl tRNA synthetase. We collected demographic data and clinical characteristics recorded at the time of diagnosis, as well as follow-up survival data. Predictors of ILD-related mortality were identified by univariate and multivariate analyses., Results: JAMI enrolled a cohort of 497 patients with PM (15%), classic DM (32%) and clinically amyopathic DM (53%). During the observation period (median 20 months), 76 died of respiratory insufficiency directly related to ILD. Univariate analysis revealed several initial parameters associated with ILD mortality, including demographic, clinical, laboratory, imaging and autoantibody variables. We used multivariate analysis with a stepwise selection of parameters to generate an appropriate predictive model, and identified the following independent risk factors for ILD mortality: age at onset ⩾60 years [hazard ratio (HR) = 4.3, 95% CI: 2.4, 7.5], CRP ⩾1 mg/dl (HR = 2.6, 95% CI: 1.5, 4.8), peripheral capillary oxygen saturation <95% (HR = 2.0, 95% CI: 1.2, 3.4) and anti-melanoma differentiation-associated gene 5 antibody (HR = 7.5, 95% CI: 2.8, 20.2)., Conclusion: We established a large cohort of incident cases of PM/DM-associated ILD, and successfully identified independent predictors of short-term ILD mortality., (© The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: To examine the relationship between MRI structural damage and repair and plasma inflammatory cytokines in patients with RA., Methods: A total of 88 newly diagnosed, untreated RA patients were enrolled. Contrast MRI of the dominant hand and X-rays of the hands and feet were performed at baseline and 1 year later. MR images were evaluated using RA MRI scoring, and X-ray., Results: Progression of bone erosion and repair were observed more frequently in MRI than in X-rays (erosion, 52% vs 26%, P < 0.001; repair, 26% vs 15%, P = 0.003, respectively). Baseline IL-6 levels and seropositivity were independent relevant factors for MRI erosion progression, with IL-6 having stronger effect than seropositivity. A receiver operating characteristic curve identified the baseline IL-6 level of 7.6 pg/ml for predicting erosion progression during 1 year, with an area under the curve of 0.82; higher IL-6 levels resulted in more erosion progression. Baseline low IL-6 was also an independent predictor for MRI erosion repair., Conclusion: In newly diagnosed, untreated RA patients, baseline plasma IL-6 levels are responsible for 1-year MRI bone erosion progression and repair., (© The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com)

Objectives: To investigate the clinical effectiveness of tocilizumab (TCZ), a monoclonal antibody against the interleukin-6 receptor, in preventing relapse in patients with adult-onset Still's disease (AOSD)., Methods: This retrospective study included clinical data from 40 patients who underwent regular follow-up at our institution in June 2013. Among these patients, 10 received TCZ. The relapse-free rate was analyzed by the Kaplan-Meier method., Results: The age at disease onset (median, interquartile range [IQR]) was 39 (29-52) years. The duration of disease in June 2013 (median, IQR) was 86 (41-193) months. A total of 87 relapses were documented in 27 patients. Ten patients with refractory or relapsing disease received 8 mg/kg of TCZ every 2-4 weeks. After 6 months of TCZ treatment, the median levels of C-reactive protein significantly decreased from 6.3 to 0.01 mg/dl (p < 0.01). Among these 10 patients, 11 relapses were observed before TCZ treatment, and none were observed after the treatment. The relapse-free rate of the 10 patients after starting TCZ was significantly higher than that of all 40 patients after the initial treatments (100% and 67% at 12 months, respectively; p = 0.03)., Conclusions: TCZ effectively prevented relapses of AOSD, and it resolved the disease activity.