Biosimilar infliximab for Behçet's syndrome: a case series.

OBJECTIVES: The efficacy and safety of biosimilar infliximab (bio-IFX) was shown in randomised controlled trials and it was approved for all indications of the reference product in several countries. However, a previous case series of 3 patients with Behçet's syndrome (BS) reported disappointing results. We aimed to share our experience with bio-IFX treatment in different types of organ involvement in patients with BS. METHODS: We reviewed the charts of all BS patients who were prescribed reference infliximab (ref-IFX) or bio-IFX in our BS clinic. Among the 181 BS patients who were prescribed IFX since 2003, 6 (3%) were prescribed bio-IFX due to refractory disease despite conventional immunosuppressives. RESULTS: A total of 6 patients (mean age: 32.1±6.2, mean disease duration: 5.3±1.8 years, 5 men and 1 woman) received bio-IFX for uveitis, nervous system, vascular and joint involvement. Four of the 6 patients obtained remission and stayed in remission during the 16±6.5 months they used bio-IFX. Among the 4 patients who obtained remission, 2 were switched to ref-IFX due to unavailability of bio-IFX infusion set and did not experience adverse events or loss of efficacy. However, relapses occurred during tapering. The other 2 patients are still in remission with bio- IFX. Among the remaining 2 patients, one had to be switched to ref-IFX after the first infusion, due to a change in the reimbursement policy and the other was non-responsive. CONCLUSIONS: Our limited experience showed that bio-IFX may be a safe and effective alternative for patients with BS, refractory to conventional immunosuppressives.

Outcome of invasive procedures for venous thrombosis in Behçet's syndrome: case series and systematic literature review.

OBJECTIVES: Systemic therapy aimed at suppressing the diffuse inflammation in the vessel wall is the major treatment modality for venous thrombosis in Behçet's syndrome (BS). Endovascular and/or surgical interventions are also used. We here report five patients who were referred to our clinic after having such interventions and also present a literature review to assess the outcome of invasive procedures for venous thrombosis in BS. METHODS: Our patients were presented and a literature search for endovascular and/or surgical interventions in Pub-Med was performed. Recanalisation, reocclusion or other complications were assessed as outcomes. RESULTS: Five BS patients with lower extremity thrombosis were referred to our clinic with post thrombotic syndrome due to incomplete recanalisation or infectious complication after endovascular interventions. Twenty-one articles reporting on 36 patients were found suitable for review. There were totally 21 lower extremity venous intervention cases, 14 of which had failure such as complication, reocclusion or incomplete recanalisation. Reocclusions occurred in 10 patients and reinterventions to 8 of them could restore flow only in 4 cases. Ileal infarct and vena cava wall-duodenal perforation were major complications. Invasive procedures of 8 abdominal thrombosis cases resulted with death due to ileus in one patient, and reocclusion in another. Seven of the 12 upper extremity/superior vena cava thrombosis cases resulted with reocclusions. CONCLUSIONS: Endovascular and surgical interventions seemed to be unsuccessful because of recurrent infectious and vascular complications in 22 (53.6%) of 41 patients with venous thrombosis. The indication of these procedures is controversial. Their economic burden on the healthcare system must be considered.

Acknowledged statistical help and a better use of p values: a proposal.

OBJECTIVES: The p-value is commonly misused. We hypothesised that a close cooperation with a statistician would go along with a more proper use of p-values. We considered a close cooperation present, when a statistician was a coauthor, or a formal statistical help was acknowledged in a study report. METHODS: Randomised controlled trials published in 2015-16 in 4 widely read rheumatology journals were searched for a close cooperation with a statistician, the inclusion of effect sizes, confidence intervals, exact rather than relative p-values and the omission of p-values in tables depicting trial entry data. RESULTS: There were only 28/133 (21%) articles in which a formal statistical help was acknowledged (Group I). The rest (Group II) gave no acknowledgement of a close cooperation. Reporting of effect sizes (96% vs. 71%) and exact p-values (88% vs. 69%) were more in Group I (p=0.01, and p=0.08, respectively). CONCLUSIONS: While a formal acknowledgement of a close cooperation was notably infrequent at 21%, this went along with improvement in some aspects of p-value reporting. If substantiated by further studies, we propose that a formally acknowledged statistical help should improve p-value reporting. Like all professionals, statisticians would like their name/office to be formally associated with their good work.

Secukinumab induced Behçet's syndrome: a report of two cases.

Secukinumab is a human monoclonal antibody against IL-17A that has been shown to be effective in psoriasis, psoriatic arthritis and ankylosing spondylitis (AS). On the other hand, in randomized controlled trials among patients with Crohn's disease (CD) and uveitis due to Behçet's syndrome (BS) treated with secukinumab, primary end points were not met and the drug caused more exacerbations compared to placebo. The drug fact sheet states that secukinumab should be used with caution in patients with CD; however, there are no warnings for those with BS. Here, we present two patients with AS treated with secukinumab; we observed exacerbation of BS in one and emergence of de novo BS in another. Although IL-17A is thought to contribute to the pathogenesis of BS, our observations suggest that it might have a protective role. Finally, we suggest caution is required with the inhibition of IL-17 in BS.

Miliary Tuberculosis Induced Acute Liver Failure.

Hepatobiliary tuberculosis is uncommon even in endemic countries. It is associated with a high mortality and is even diagnosed early in the disease course. Acute liver failure (ALF) caused by tuberculosis bacilli has been reported in only a few reports. All previous cases have been diagnosed by postmortem examination. Time to antituberculosis treatment is very critical. In case of suggestive findings on clinical and radiologic examination, antituberculosis treatment should be initiated immediately. Drug use can be a challenge in patients with ALF. However, as long as the other possible causes of ALF can be excluded and hepatotoxic drugs were avoided during the early course of treatment, such a highly fatal presentation of tuberculosis can be treated safely. Here, we report a case of acute liver failure as a presentation of miliary tuberculosis. He was treated successfully with antituberculosis treatment.

Diagnostic usefulness of FibroMeter VCTE for hepatic fibrosis in patients with nonalcoholic fatty liver disease.

BACKGROUND: Liver fibrosis is an important prognostic determinant in patients with nonalcoholic fatty liver disease (NAFLD). The FibroMeter VCTE is a diagnostic tool comprising both biochemical markers and transient elastography (TE) originally developed for the diagnosis of fibrosis in patients with chronic viral hepatitis. In this pilot study, we investigated the diagnostic performance of the FibroMeter VCTE tool for determining fibrosis in patients with biopsy-proven NAFLD. Its diagnostic accuracy was also compared with those of the NAFLD fibrosis score (NFSA) and TE alone. PATIENTS AND METHODS: FibroMeter VCTE, NFSA, and TE were determined in 52 patients with NAFLD. The results of liver biopsies were considered the gold standard. Areas under the receiver operating characteristic curve were used to express the diagnostic accuracy of each test. RESULTS: Significant (F≥2) and severe (F≥3) fibrosis were detected in 20 (38%) and 10 (19%) patients, respectively. The sensitivity of FibroMeter VCTE, NFSA, and TE for detecting significant fibrosis was 70, 65, and 75%, respectively, whereas specificity was 88, 81, and 78%. The sensitivity of FibroMeter VCTE, NFSA, and TE for diagnosing severe fibrosis was 90, 90, and 100%, respectively, whereas specificity was 93, 78, and 76%. Receiver operating characteristic analysis showed that FibroMeter VCTE had a significantly larger areas under the receiver operating characteristic curve (0.968) compared with both NFSA (0.833, P<0.001) and TE (0.922, P<0.05) for the detection of severe fibrosis. CONCLUSION: Our preliminary findings indicate that FibroMeter VCTE is superior to both NFSA and TE for the diagnosis of severe fibrosis in patients with NAFLD.