Objectives: Calcineurin inhibitors are commonly used to treat idiopathic inflammatory myopathies associated interstitial lung disease (IIM-ILD) in Asia but not in the U.S. Here, we evaluate the efficacy of tacrolimus (TAC) as first-line immunosuppressive therapy in a U.S cohort of IIM-ILD.
Methods: This retrospective, single-centre cohort study evaluated the change in absolute forced vital capacity (FVCabs) in IIM-ILD participants after 12 months of treatment with mycophenolate mofetil (MMF), azathioprine (AZA), or TAC. Participants were naïve to immunosuppressive agents other than glucocorticoids and/or intravenous immunoglobulin. Treatment with MMF, AZA, or TAC was at the discretion of their primary clinician. Additional outcomes were transplant free survival, prednisone use, and supplemental oxygen use at 12 months.
Results: Thirty-one participants were included in this study. The TAC group was younger and had a shorter disease duration than the MMF/AZA group. All MDA5 patients were treated with TAC. After 12 months of therapy, the FVCabs was unchanged in the MMF/AZA group (2.4 l [95%CI 2.0, 3.2 l] vs 2.3 l [95%CI 2.0, 2.8 l]) and improved in TAC group (2.5 l [95%CI 2.0, 3.0 l] vs.1.9L [95%CI 1.0, 2.6 l]). Transplant free survival was 100% in both groups. All patients in the MMF/AZA group remained on supplemental oxygen at 12 months, but 4/5 patients in the TAC group resolved their oxygen requirement.
Conclusion: TAC is effective as a first-line agent in a small, non-randomized U.S. cohort of IIM-ILD enriched for MDA5+ participants. Further work is needed to investigate the relative efficacy of calcineurin inhibitors compared with MMF/AZA in the U.S. population.
Objectives: To describe the characteristics and outcome of patients with the association of large vessel vasculitis (LVV, Takayasu arteritis [TA] or giant cell arteritis [GCA]) and inflammatory bowel disease (IBD).
Methods: An observational, multicentre, retrospective case-control study. Cases were LVV-IBD patients from European countries, whereas controls had isolated LVV (iLVV).
Results: 39 TA-IBD and 12 GCA-IBD cases were enrolled, compared with 52 isolated GCA (iGCA) and 93 isolated TA (iTA) controls. LVV occurred after IBD in 56% in TA-IBD and 75% in GCA-IBD, with a median interval of 1 year (IQR 1-7) in TA-IBD and 8.6 years (IQR 1-17.7) in GCA-IBD. Crohn's disease was more common in TA-IBD (67%), whereas ulcerative colitis was more common in GCA-IBD (58%). Compared with iTA, TA-IBD were significantly younger at diagnosis of TA (median age 27 vs 37 years, p< 0.001) and had more upper limb claudication (36% vs 12%, p= 0.006). GCA-IBD patients had more frequent arterial thickening or stenosis than controls (75% vs 30%, respectively, p= 0.044), and tended to more frequently involve gastrointestinal arteries (20% vs 0%, respectively, p= 0.06). LVV occurred in IBD patients despite treatment with glucocorticoids (36%), azathioprine (25%), or TNF-alpha blockers (29%). The presence of the IBD was not associated with a higher LVV relapse rate in multivariate analysis (adjusted hazard ratio [aHR] 0.62 [0.13-2.83] for GCA and aHR 0.92 [0.44-1.89] for TA).
Conclusion: This study identifies specific clinical and imaging characteristics of LVV-IBD patients, in particular a more severe vascular presentation of GCA-IBD patients compared with iGCA patients.
Objectives: Despite growing interest in musculoskeletal ultrasound (MSUS), training opportunities are often limited due to staff shortages and disbalances of expertise between rural and urban areas. Teledidactic approaches have the potential to expand access to training opportunities. This study aims to compare the effectiveness of teledidactic peer-tutored MSUS training to a conventional approach.
Methods: A teledidactic course was held by a student tutor following a validated MSUS curriculum. An on-campus MSUS training taught by physician lecturers served as a control. Students were randomly assigned to one of both study groups. Objective structured clinical examinations (OSCE) were conducted before and after the training to objectively measure the learning outcome of the participants. Handheld ultrasound devices (ButterflyIQ®) and iPads (Apple Inc., 8th generation) were provided to the students for the MSUS course.
Results: Thirty medical students participated in the study. Prior to the course, baseline OSCE scores were recorded as 13.03/63 (SD ± 4.20) for the on-campus cohort and 13.00/63 (SD ± 6.04) for the teledidactic group. In the post-training OSCE evaluation, the on-campus cohort attained an average score of 56.80/63 (SD ± 4.22), while the TELMUS group averagely achieved 58.53/63 points (SD ± 3.52). While all students' skills increased over time, there was no significant difference between the two cohorts either before or after the course.
Conclusion: Peer-tutored, teledidactic MSUS training showed to be non-inferior to the conventional approach and is a promising approach to reduce local and global disparities in educational opportunities regarding MSUS.
Objectives: To investigate clinical features associated with lack of response to MTX in juvenile idiopathic arthritis associated uveitis (JIA-U).
Methods: Clinical records of JIA-U patients were retrospectively reviewed. Differences among variables were assessed by Mann-Whitney and χ2 or Fisher's exact tests as appropriate. Association between predictors and requirement of a biological disease-modifying antirheumatic drug (bDMARD) was evaluated by univariate Cox regression analysis and Kaplan-Meier curves. A multivariable logistic model was applied to estimate strength of association, adjusting for potential confounders.
Results: Data from 99 JIA-U patients treated with MTX were analysed (82.8% female), with a mean follow up of 9.2 years and a mean age at uveitis onset of 5.7 years. In 65 patients (65.7%) at least one bDMARD to control uveitis was required. Children requiring a bDMARD for uveitis had lower age at JIA and uveitis onset, more frequent polyarticular course, higher frequency of bilateral uveitis at onset and higher prevalence of systemic steroids' use. Despite similar frequency of ocular damage at onset, MTX non-responders showed a higher percentage of ocular damage at last visit. Younger age at JIA onset, polyarticular course and a history of systemic steroids' use resulted independent factors associated to lack of response to MTX at Cox regression analysis. Kaplan-Meier curves and the multivariate model confirm the independent role of both polyarticular course and systemic steroids' use.
Conclusions: Younger age at JIA onset, polyarticular course and a history of systemic steroids' use are predictors of a worse response to MTX in JIA-U.
Objectives: To evaluate damage and clinical characteristics associated with damage in Takayasu's arteritis (TAK).
Methods: Patients with TAK enrolled in a multicentre, prospective, observational study underwent standardized damage assessment every 6 months using the Vasculitis Damage Index (VDI) and the Large-Vessel Vasculitis Index of Damage (LVVID).
Results: The study included 236 patients with TAK: 92% female, 81% Caucasian; median (25th, 75th percentile) disease duration = 2.6 (0.12, 6.9) years. Eighty-four percent had follow-up: median (25th, 75th) duration 4.1 (1.9, 7.5) years. Items of damage were present in 89% on VDI, 87% on LVVID, in the peripheral vascular (76% VDI, 74% LVVID) and cardiac (40% VDI, 45% LVVID) systems. During follow-up, 42% patients had new damage, including major vessel stenosis/arterial occlusion (8%), limb claudication (6%), hypertension (7%), aortic aneurysm (4%) and bypass surgery (4%). Disease-specific damage accounted for >90% of new items. Older age, relapse and longer duration of follow-up were associated with new damage items; a higher proportion of patients without new damage were on MTX (P <0.05). Among 48 patients diagnosed with TAK within 180 days of enrolment, new damage occurred in 31% on VDI and 52% on LVVID. History of relapse was associated with new damage in the entire cohort while in patients with a recent diagnosis, older age at diagnosis was associated with new damage.
Conclusion: Damage is present in >80% of patients with TAK even with recent diagnosis and >40% of patients accrue new, mainly disease-specific damage. Therapies for TAK that better control disease activity and prevent damage should be prioritized.