Tracheobronchomalacia due to amyloidosis in a patient with rheumatoid arthritis

In this case report, we describe a patient with longstanding rheumatoid arthritis who developed tracheobronchomalacia with fatal outcome. Despite negative antemortem biopsies of abdominal fat and tongue, amyloid was found postmortem in the trachea and appeared to be associated with tracheobronchomalacia

When it looks like Behçet's syndrome but is something else: Differential diagnosis of Behcet's syndrome: a two-centre retrospective analysis.

OBJECTIVE To investigate the differential diagnostic spectrum in patients with suspected Behçet's syndrome (BS) in low prevalence regions. In addition, the number of patients fulfilling the ICBD criteria despite not having BS was evaluated. METHODS This retrospective analysis was performed in two referral centers for BS. Patients with confirmed BS (clinical diagnosis with fulfilment of ISG criteria or a score of ≥ 5 points in the ICBD criteria) were excluded. The remaining patients were divided into eleven differential diagnosis categories. If no definitive alternative diagnosis could be established, patients were termed 'probable BS' in case of (1) relapsing orogenital aphthosis in the absence of other causes and either HLA-B51 positivity, origin from an endemic area or presence of an additional typical BS symptom that is not part of the classification criteria or (2) with 3-4 points scored in the ICBD criteria. RESULTS In total 202 patients were included and categorized as follows: 58 patients (28.7%) as 'probable BS', 57 (28.2%) skin disease, 26 (12.9%) chronic pain syndrome, 14 (6.9%) eye disease, 11 (5.4%) spondyloarthropathy, 9 (4.5%) gastrointestinal disease, 7 (3.5%) neurological disease, 4 (2%) arthritis, 3 (1.5%) auto-inflammation, 3 (1.5%) connective tissue disease, 10 (5.0%) miscellaneous disease. HLA-B51 was positive in 55/132 (41.6%); 75/202 (37.1%) of the patients fulfilled the ICBD criteria. CONCLUSION In a low disease prevalence setting the straightforward application of the ICBD criteria may lead to overdiagnosis of BS. The differential diagnosis of BS is enormously broad. Clinicians should be aware that HLA-B51 positivity is still not considered as a diagnostic feature in BS

Adherence to guidelines for the treatment of Behçet's syndrome in New York and Amsterdam

Objective. To assess adherence to published guidelines for the treatment of Behçet's syndrome (BS) in two geographic areas. Methods. We extracted guideline statements from the 2008 EULAR recommendations. Adherence to these statements was evaluated retrospectively in both New York (USA) and Amsterdam (The Netherlands), by reviewing records from patients fulfilling the ISG criteria. We analysed data per statement and event, and divided data according to the year in which an event occurred. We compared events prior to 2009 to those after publication of the EULAR recommendations (2009 and later). Results. 474 patients were evaluated, 24 of whom were from Amsterdam. Treatment adherence varied substantially across various Behçet's manifestations, ranging from 21% vs. 31% in posterior uveitis, 50% vs. 25% in arterial disease, 29% vs. 29% in arthritis and 38% vs. 55% in erythema nodosum to 65% vs. 67% in deep venous thrombosis (DVT), before and after publication of the guidelines respectively. Topical treatment of mucocutaneous disease was only 2% vs. 8%, whereas adherence in neuro-Behçet was ≥94% and 100% in gastrointestinal disease. Conclusion. Adherence to treatment guidelines varies substantially by Behçet manifestation. Lack of adherence in manifestations such as eye disease and arthritis suggests that current recommendations are not sufficient or other concurrent manifestations require more aggressive treatment. The extensive use of anti-TNF agents might indicate a shift towards more aggressive treatment. Thus, our results suggest the 2008 guidelines were not in line with treatment in clinical practice over the past years and the recent revision of the recommendations was indeed needed

Feasibility of Self-Monitoring Rheumatoid Arthritis With a Smartphone App: Results of Two Mixed-Methods Pilot Studies

Background: Several mobile apps that monitor symptoms of rheumatoid arthritis (RA) exist, but a recent systematic review indicated that high-quality apps are lacking. When patients self-monitor their own disease with patient-reported outcomes (PROs) and self-initiate care at the right moment, it may be possible to reduce the frequency of their clinic visits, which would reduce health care burden and costs. We developed an app, that is, the MijnReuma Reade app, for this purpose and performed 2 pilot tests with weekly self-monitoring. Objective: The primary objective of this study was to design, develop, and evaluate the usability, satisfaction, and usage of the MijnReuma Reade app-an app that allows patients with RA to monitor their own disease. The secondary objective was to review the patients' perspectives on app usage and its intended purpose. Methods: This app was designed in collaboration with patients with RA, rheumatologists, and information technology experts. Two 1-month pilot studies were performed, after which satisfaction (0-10 scale), usability (system usability scale, 0-100), and usage (proportion of completed questionnaires) of this app were assessed. After the second pilot study, semistructured interviews were performed to determine patients' perspectives and the promoters and barriers of app usage. Results: In the first and second pilot study, 42 and 27 patients were included, respectively. Overall, the patients were satisfied (medians, 8 and 7) and found the app usable (mean system usability scores, 76 and 71) in pilot studies 1 and 2, respectively. App usage declined over time in both the pilot studies; 61% (17/28) and 37% (10/27) of the patients who disclosed their usage statistics completed the final weekly questionnaire in pilot study 1 and pilot study 2, respectively. Approximately 81% (25/31) of the patients indicated they would like to skip hospital visits if the self-monitored disease activity is low. In the semistructured interviews, technical problems, internal resistance (respondent fatigue, the app reminded them of their disease), and a lack of symptoms were identified as barriers for usage. Patients reported that "experiencing more grip on their disease" and "improved communication with their physician" were promoters for usage. Patients reported that pain positively mediated usage, that is, more pain promoted and less pain discouraged app usage. Conclusions: This study illustrates the feasibility of the MijnReuma Reade app that enables self-monitoring of the disease activity in patients with RA with the overarching aim to allocate clinical consultations according to need. Satisfaction with the app and usability of the app were found to be high; however, app usage declined over time. Patients acknowledged the potential of the app to self-monitor their own disease and would like to be able to skip clinic visits if the monitored disease activity is low. To evaluate this strategy, a randomized controlled trial is underway

The value of joint ultrasonography in predicting arthritis in seropositive patients with arthralgia: A prospective cohort study

Background: The value of joint ultrasonography (US) in the prediction of clinical arthritis in individuals at risk of developing rheumatoid arthritis (RA) is still a point of debate, due to varying scanning protocols and different populations. We investigated whether US abnormalities assessed with a standard joint protocol can predict development of arthritis in seropositive patients with arthralgia. Methods: Anti-citrullinated protein antibodies and/or rheumatoid factor positive patients with arthralgia, but without clinical arthritis were included. US was performed at baseline in 16 joints: bilateral metacarpophalangeal 2-3, proximal interphalangeal 2-3, wrist and metatarsophalangeal (MTP) joints 2-3 and 5. Images were scored semi-quantitatively for synovial thickening and for positive signs on power Doppler (PD). Association between US abnormalities and arthritis development at the joint and at the patient level was evaluated. Also, we investigated the added value of US over clinical parameters. Results: Out of 163 patients who underwent US examination, 51 (31%) developed clinical arthritis after a median follow-up time of 12 (interquartile range 5-24) months, of which 44 (86%) satisfied the 2010 American College of Rheumatology/European League Against Rheumatism classification criteria for RA. US revealed synovial thickening and PD in at least one joint in 49 patients (30%) and 7 patients (4%), respectively. Synovial thickening was associated with both development and timing of clinical arthritis in any joint (patient level) when MTP joints were excluded from the US assessment (odds ratio 6.6, confidence interval (CI) 1.9-22), and hazard ratio 3.4, CI 1.6-6.8, respectively, with a mean time to arthritis of 23 versus 45 months when synovial thickening was present versus not present). There was no association between US and arthritis development at the joint level. Predictive capacity was highest in the groups with an intermediate and high risk of developing arthritis based on a prediction rule with clinical parameters. Conclusions: Synovial thickening on US predicted clinical arthritis development at the patient level in seropositive patients with arthralgia when MTPs were excluded from the US assessment. Positive PD signs were infrequently seen in these at-risk individuals and was not predictive. In patients at intermediate risk of RA, US may help to identify those at higher risk of developing arthritis

A multidisciplinary lifestyle program for rheumatoid arthritis: the 'Plants for Joints' randomized controlled trial

Objective To determine the effect of a multidisciplinary lifestyle program in patients with RA with low–moderate disease activity. Methods In the ‘Plants for Joints’ (PFJ) parallel-arm, assessor-blind randomized controlled trial, patients with RA and 28-joint DAS (DAS28) ≥2.6 and ≤5.1 were randomized to the PFJ or control group. The PFJ group followed a 16-week lifestyle program based on a whole-food plant-based diet, physical activity and stress management. The control group received usual care. Medication was kept stable 3 months before and during the trial whenever possible. We hypothesized that PFJ would lower disease activity (DAS28). Secondary outcomes included anthropometric, metabolic and patient-reported measures. An intention-to-treat analysis with a linear mixed model adjusted for baseline values was used to analyse between-group differences. Results Of the 83 people randomized, 77 completed the study. Participants were 92% female with mean (S.D.) age of 55 (12) years, BMI of 26 (4) kg/m2 and mean DAS28 of 3.8 (0.7). After 16 weeks the PFJ group had a mean 0.9-point greater improvement of DAS28 vs the control group (95% CI 0.4, 1.3; P < 0.0001). The PFJ intervention led to greater decreases in body weight (difference –3.9 kg), fat mass (–2.8 kg), waist circumference (–3 cm), HbA1c (–1.3 mmol/mol) and low-density lipoprotein (–0.32 mmol/l), whereas patient-reported outcome measures, blood pressure, glucose and other lipids did not change. Conclusion The 16-week PFJ multidisciplinary lifestyle program substantially decreased disease activity and improved metabolic status in people with RA with low–moderate disease activity. Trial Registration International Clinical Trials Registry Platform; https://www.who.int/clinical-trials-registry-platform; NL7800

Results of a 6-week treatment with 10 mg prednisolone in patients with hand osteoarthritis (HOPE):a double-blind, randomised, placebo-controlled trial

Background Hand osteoarthritis is a prevalent joint condition that has a high burden of disease and an unmet medical need for effective therapeutic options. Since local inflammation is recognised as contributing to osteoarthritic complaints, the Hand Osteoarthritis Prednisolone Efficacy (HOPE) study aimed to investigate the efficacy and safety of short-term prednisolone in patients with painful hand osteoarthritis and synovial inflammation.Methods The HOPE study is a double-blind, randomised, placebo-controlled trial. We recruited eligible adults from rheumatology outpatient clinics at two sites in the Netherlands. Patients were considered eligible if they had symptomatic hand osteoarthritis and signs of inflammation in their distal and proximal interphalangeal (DIP/PIP) joints. For inclusion, patients were required to have four or more DIP/PIP joints with osteoarthritic nodes; at least one DIP/PIP joint with soft swelling or erythema; at least one DIP/PIP joint with a positive power Doppler signal or synovial thickening of at least grade 2 on ultrasound; and finger pain of at least 30 mm on a 100-mm visual analogue scale (VAS) that flared up during a 48-h non-steroidal anti-inflammatory drug (NSAID) washout (defined as worsening of finger pain by at least 20 mm on the VAS). Eligible patients were randomly assigned (1:1) to receive 10 mg prednisolone or placebo orally once daily for 6 weeks, followed by a 2-week tapering scheme, and a 6-week follow-up without study medication. The patients and study team were masked to treatment assignment. The primary endpoint was finger pain, assessed on a VAS, at 6 weeks in participants who had been randomly assigned to groups and attended the baseline visit. This study is registered with the Netherlands Trial Registry, number NTR5263.Findings We screened patients for enrolment between Dec 3, 2015, and May 31, 2018. Patients completed baseline visits and started treatment between Dec 14, 2015, and July 2, 2018, and the last study visit of the last patient was Oct 4, 2018. Of 149 patients assessed for eligibility, 57 (38%) patients were excluded (predominantly because they did not meet one or several inclusion criteria, most often because of an absence of synovial inflammation or of flare-ups after NSAID washout) and 92 (62%) patients were eligible for inclusion. We randomly assigned 46 (50%) patients to receive prednisolone and 46 (50%) patients to receive placebo, all of whom were included in the modified intention-to-treat analysis of the primary endpoint. 42 (91%) patients in the prednisolone group and 42 (91%) in the placebo group completed the 14-week study. The mean change between baseline and week 6 on VAS-reported finger pain was -21.5 (SD 21.7) in the prednisolone group and -5.2 (24.3) in the placebo group, with a mean between-group difference (of prednisolone vs placebo) of -16.5 (95% CI -26.1 to -6.9; p=0.0007). The number of non-serious adverse events was similar between the groups. Five serious adverse events were reported during our study: one serious adverse event in the prednisolone group (a myocardial infarction) and four serious adverse events in the placebo group (an infected traumatic leg haematoma that required surgery, bowel surgery, atrial fibrillation that required a pacemaker implantation, and symptomatic uterine myomas that required a hysterectomy). Four (4%) patients discontinued the study because of an adverse event: one (2%) patient receiving prednisolone (for a myocardial infarction) and three (7%) patients receiving placebo (for surgery of the bowel and for an infected leg haematoma and for Lyme disease arthritis of the knee).Interpretation Treatment with 10 mg prednisolone for 6 weeks is efficacious and safe for the treatment of patients with painful hand osteoarthritis and signs of inflammation. The results of our study provide clinicians with a new short-term treatment option for patients with hand osteoarthritis who report a flare-up of their disease. Copyright (C) 2019 Elsevier Ltd. All rights reserved.Clinical epidemiolog