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Tezcan, Mehmet Engin/0000-0002-1753-4936; TUFAN, Abdurrahman/0000-0001-6244-9362; SARI, Ismail/0000-0001-7737-4180; YILMAZ, Sedat/0000-0002-4691-3417 WOS: 000436403100017 PubMed: 28992387 ObjectiveApproximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients. MethodsIn all, 21 centers from different geographical regions of Turkey were included in the current study. The medical records of all FMF patients who had used anti-IL-1 treatment for at least 6 months were reviewed. ResultsIn total, 172 FMF patients (83 [48%] female, mean age 36.2 years [range 18-68]) were included in the analysis; mean age at symptom onset was 12.6 years (range 1-48), and the mean colchicine dose was 1.7 mg/day (range 0.5-4.0). Of these patients, 151 were treated with anakinra and 21 with canakinumab. Anti-IL-1 treatment was used because of colchicine-resistant disease in 84% and amyloidosis in 12% of subjects. During the mean 19.6 months of treatment (range 6-98), the yearly attack frequency was significantly reduced (from 16.8 to 2.4; P < 0.001), and 42.1% of colchicine-resistant FMF patients were attack free. Serum levels of C-reactive protein, erythrocyte sedimentation rate, and 24-hour urinary protein excretion (5,458.7 mg/24 hours before and 3,557.3 mg/24 hours after) were significantly reduced. ConclusionAnti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients.

Objective Patient-physician discordance in global assessment of disease activity concerns one-third of patients, but what does it reflect? We aimed to assess patient-physician discordance in psoriatic arthritis (PsA) and patient-reported domains of health (physical and psychological) associated with discordance. Methods We analyzed the PsAID (Psoriatic Arthritis Impact of Disease), a cross-sectional, multicenter European study of patients with PsA according to expert opinion. Patient global assessment (PGA) and physician global assessment (PhGA) were rated on a 0–10 numeric rating scale. Discordance was defined as the difference (PGA−PhGA) and as the absolute difference |PGA−PhGA| ≥3 points. Determinants of PGA−PhGA were assessed by a stepwise multivariate linear regression among 12 physical and psychological aspects of impact: pain, skin problems, fatigue, ability to work/leisure, functional incapacity, feeling of discomfort, sleep disturbance, anxiety/fear, coping, embarrassment/shame, social participation, and depressive affects. Results In 460 patients (mean ± SD age 50.6 ± 12.9 years, 52.2% female, mean ± SD disease duration 9.5 ± 9.5 years, mean ± SD Disease Activity Index for Psoriatic Arthritis score 30.8 ± 32.4, and 40.4% undergoing treatment with biologic agents), the mean ± SD PGA was higher than the mean PhGA, with a mean absolute difference of 1.9 ± 1.8 points. Discordance defined by |PGA−PhGA| ≥3 of 10 concerned 134 patients (29.1%), and 115 patients (85.8% of the patients with discordance) had PGA>PhGA. Higher fatigue (β = 0.14), lower self-perceived coping (β = 0.23), and impaired social participation (β = 0.16) were independently associated with a higher difference (PGA−PhGA). Conclusion Discordance concerned 29.1% of these patient/physician dyads, mainly by PGA>PhGA. Factors associated with discordance were psychological rather than physical domains of health. Discordance was more frequent in patients in remission, indicating more work is needed on the patient perspective regarding disease activity.

Objective: The six minute walk test (6MWT) is used for the assessment of functional capacity in pulmonary and cardiovascular diseases. Left ventricular diastolic dysfunction (LVDD) is the most common cardiac abnormality in systemic sclerosis (SSc). The aim of this study was to define the effect of LVDD on 6MWT parameters in patients with SSc. Methods: We studied 45 (female : male 40 : 5) SSc patients. Patients with obvious conditions that can affect 6MWT distance (6MWD) were excluded. All subjects were evaluated by 6MWT. Additionally, 6MWD of the participants was calculated as the percentage of normal predicted values. LVDD was assessed by using echocardiographic findings and classified into three categories: impaired relaxation, pseudonormal or restrictive. Results: There were 12 (27%) patients with LVDD. SSc patients with LVDD were older than SSc patients without LVDD (50 ± 12 years vs. 41 ± 10 years; P = 0.017). In all, the mean 6MWD was 487.9 ± 98.3 m. The 6MWD was shorter in SSc patients with LVDD as compared to those without LVDD (438.0 ± 94.7 m vs. 506.0 ± 94.5 m; P = 0.039). There was significant difference between the groups regarding the percentage of the predicted 6MWD (74.1 ± 10.1%vs. 82.8 ± 13.1%; P = 0.041). Conclusion: The presence of LVDD alters 6MWD in SSc patients. Reduction of 6MWD in a patient with SSc should prompt the investigation of LVDD.

BACKGROUND Cardiac involvement is one of the major problems in systemic sclerosis (SSc). Subclinical cardiac involvement has a higher frequency than thought previously. In this study we investigated whether subclinical cardiac involvement can be detected by using echocardiographic strain imaging in SSc patients without pulmonary hypertension. METHODS Echocardiographic examinations were performed to 27 SSc patients and 26 healthy controls. Left ventricular strain parameters were obtained from apical views and average strain value was calculated from these measurements. RESULTS There were no significant differences between patients and controls regarding two-dimensional (2D), conventional Doppler and tissue Doppler velocity measurements. Strain was reduced in 6 of 12 segments of the left ventricle (LV) and in 1 of 2 segments of the right ventricle (RV). Strain rate (SR) was reduced in 2 of 12 segments of the LV and 1 of 2 segments of the RV in SSc patients as compared to controls (P < 0.05 for all). These involvements did not match any particular coronary artery distribution. More important differences were detected by average strain and SR values of the LV between patients and controls (19.78 +/- 3.00% vs 23.41 +/- 2.73%, P < 0.001; 2.01 +/- 0.41 vs 2.23 +/- 0.27/sec, P = 0.026, respectively). Furthermore, carbon monoxide diffusion capacity (DLCO) in scleroderma patients significantly correlated with LV average strain (r = 0.59; P = 0.001). CONCLUSION Evaluation of ventricular function by using echocardiographic strain imaging appears to be useful to detect subclinical cardiac involvement in SSc patients with normal standard echocardiographic and tissue Doppler velocity findings.

Aim To assess the appropriateness of utilization of beds in the internal medicine department of a university hospital. Methods The appropriateness of hospital stay was evaluated using the Appropriateness Evaluation Protocol. A random sample of 402 days of stay was assessed. Results One hundred and thirty-nine days of stay (34.6%) were classified as inappropriate. The inappropriate stays were mostly secondary to hospital-related factors. The two major factors for inappropriate stays were ‘inappropriate timing/delay in diagnostic procedures/consultations’ (27%) and ‘delay in obtaining test results’ (27%). None of the factors including age, gender, residence and inpatient period was significantly related to inappropriate stay in univariate analysis. Conclusion This study indicated that a significant portion of stays were inappropriate. Efforts to decrease particularly hospital-related factors associated with inappropriate stay are needed.

Objectives: Subclinical cardiac involvement may occur in patients with Behcet's disease (BD). The purpose of our study was to assess the noninvasive parameters of biventricular function derived from Doppler tissue imaging (DTI) of the tricuspid and mitral annular motion in BD. Methods: Twenty- one patients with BD and 20 control subjects were enrolled in this study. All subjects were selected to exclude those with cardiovascular risk factors. Standard echocardiography and pulsed DTI were obtained in every patient. Results: Peak systolic (13.71 ± 2.09 vs 20.01 ± 1.57, P < 0.001), peak early diastolic (11.26 ± 2.52 vs 15.35 ± 2.06, P < 0.001) tricuspid annular velocities were significantly lower in patients than controls. Peak systolic (8.68 ± 1.4 vs 12.25 ± 1.7, P < 0.001), peak early diastolic (7.89 ± 1.07 vs 9.94 ± 1.12, P < 0.001), and peak end diastolic (8.30 ± 1.32 vs 9.23 ± 0.91, P = 0.013) lateral mitral annular velocities were significantly lower in patients than controls. Conclusions: We demonstrated that myocardial velocities, were affected in patients with BD. Therefore, we conclude that right and left ventricular function is impaired in patients with BD. (ECHOCARDIOGRAPHY, Volume 23, October 2006)