A national, multicenter, retrospective study evaluating retention rate and efficacy of tocilizumab treatment in patients with active rheumatoid arthritis who had an inadequate response to csDMARDs and/or TNF inhibitors.

BACKGROUND: To describe the disease activity and retention rate in rheumatoid arthritis (RA) patients with inadequate response (IR) to conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) and/or tumor necrosis factor inhibitors (TNFis) who were prescribed tocilizumab (TCZ) as first-line or second-line biologic treatment in real-world setting. METHODS: Data gathered from patients' files was used in a multicenter and retrospective context. Retention rates and the Disease Activity Score in 28 joints with CRP (DAS28-CRP) were evaluated at time points. The relationship of drug efficacy with factors such as smoking, obesity, and previous use of TNFis was also examined. RESULTS: One hundred and twenty-four patients with a median (IQR) RA duration of 3.7 (7.4) years were included. Mean (SD) age was52.9 (12.9) and 75% of the patients were female. TCZ retention rates in the 6th and 12th months were 94.1% and 86.6%, respectively. In all patients, DAS28-CRP level decreased significantly from baseline to Months 3 and 6. There was an increase in patients with remission and/or low disease activity and a decrease in patients with high disease activity at Month 3 and Month 6 (p < 0.001 for both). Disease activity was similar between subgroups based on body mass index, smoking status, and previous use of TNFis at any time point. Regression analysis showed that absence of concomitant corticosteroid treatment independently was associated with remission/LDA achievement at Month 6 [OR = 0.31, 95% CI (0.14- 0.72), p = 0.006], and Month 12 [OR = 0.35, 95% CI (0.13-0.94), p = 0.037]. Overall, 25 mild adverse events were reported. DISCUSSION: TCZ was found to be effective and safe in RA patients with IR to csDMARDs and/or TNFis. The drug retention rate was considered satisfactory with more than half of the patients continuing TCZ treatment at Month 12.

Plasma thiopurine S-methyltransferase levels and azathioprine-related adverse events in patients with Behçet's disease.

OBJECTIVES: Thiopurine S-methyltransferase (TPMT) is the key enzyme inactivating azathioprine (AZA), an immunosuppressive agent commonly used for treating inflammatory diseases including Behçet's disease (BD), systemic lupus erythematosus (SLE) and systemic vasculitis. Low TPMT levels facilitate occurrence of AZA-related adverse effects. We investigated TPMT levels in patients with BD, compared to healthy controls and patients with SLE or systemic vasculitis. METHODS: This cross-sectional study included 101 BD (77 using AZA), 74 SLE (35 using AZA), and 44 vasculitis (18 using AZA) patients and 101 healthy controls. Plasma TPMT levels were measured using ELISA. Student's t- and Kruskal-Wallis tests were used to compare TPMT levels according to possible risk factors. Receiver operating characteristic (ROC) analysis was used to determine whether a cut-off TPMT level could be found to predict AZA-related adverse effects. RESULTS: Plasma TPMT levels (mean± SD ng/mL) in BD (22.80±13.81) were comparable with healthy controls (22.71±13.49), but significantly lower than in SLE group (29.37±11.39) (p<0.001). TPMT levels in 130 patients receiving AZA were similar to the rest of the group. AZA-related adverse effects were identified in only 8 patients (5 with BD and 3 with SLE). TPMT levels were significantly lower in those 8 patients (14.08±9.49 vs. 25.62±12.68) (p=0.013), besides a cut-off value for predicting adverse effects was determined for the BD group with ROC analysis (area under the curve: 0.813). CONCLUSIONS: This is the first study to evaluate TPMT activity in a Turkish adult population. Although low plasma TPMT level is not the only factor determining AZA toxicity, a TPMT cut-off value may help to predict AZA-related adverse effects in BD.

Frequency of pulmonary hypertension in transthoracic echocardiography screening is not increased in Takayasu arteritis: Experience from a single center in Turkey.

OBJECTIVE: Pulmonary hypertension (PH) may occur in Takayasu arteritis (TA), mostly due to pulmonary arteritis, but also due to left heart disease and/or chronic thromboembolism (CTE). Using transthoracic echocardiography (TTE), we investigated the frequency of PH caused by pulmonary arteritis. METHODS: This cross-sectional study included 70 patients with TA fulfilling the 1990 ACR criteria, 68 healthy controls, and 67 patients with systemic sclerosis (SSc) fulfilling the 1980 ACR criteria representing the disease control group. Patients with severe left heart disease or CTE were excluded. The ESC-ERS guideline definition was considered for diagnosis of PH. RESULTS: The mean systolic pulmonary artery pressure (SPAP) values in TA, SSc, and healthy control groups were 20.93±6.06, 31.57±12.75, and 18.88±5.39 mmHg, respectively. While the SPAP values were similar between TA and healthy groups, the SPAP values in the SSc group were significantly higher than in other groups. Based on conventional and/or magnetic resonance angiography findings, pulmonary arteritis was present in 4 out of 70 TA patients; however, PH was not detected in any patients with TA, including those with pulmonary arteritis. CONCLUSION: The TTE findings suggested that the frequency of PH was not increased in TA. However, a low frequency of pulmonary arteritis in our series might have affected our results.

Segmental arterial mediolysis mimics systemic vasculitis.

Segmental arterial mediolysis (SAM) is a rare, nonarteriosclerotic, noninflammatory vascular disease and mostly affects medium-to-large sized abdominal arteries as well as presents with hemorrhages in the abdominal cavity. We report the case of a patient with SAM of the celiac, right renal, jejunal branch of the superior mesenteric, left gastric, and splenic arteries who was diagnosed by excluding other causes and in whom transcatheter embolization was performed in two different sessions, but he died because of an undefined reason. SAM mimics systemic vasculitis and causes abdominal pain; it should be considered because abdominal hemorrhage or arterial infarction can result in death.