Artificial liver support in patients with liver failure: a modified DELPHI consensus of international experts

International audienceThe present narrative review on albumin dialysis provides evidence-based and expert opinion guidelines for clinicians caring for adult patients with different types of liver failure. The review was prepared by an expert panel of 13 members with liver and ntensive care expertise in extracorporeal liver support therapies for the management of patients with liver failure. The coordinating committee developed the questions according to their importance in the management of patients with liver failure. For each indication, experts conducted a comprehensive review of the literature aiming to identify the best available evidence and assessed the quality of evidence based on the literature and their experience. Summary statements and expert’s recommendations covered all indications of albumin dialysis therapy in patients with liver failure, timing and intensity of treatment, efficacy, technical issues related to the device and safety. The panel supports the data from the literature that albumin dialysis showed a beneficial effect on hepatic encephalopathy, refractory pruritus, renal function, reduction of cholestasis and jaundice. However, the trials lacked to show a clear beneficial effect on overall survival. A short-term survival benefit at 15 and 21 days respectively in acute and acute-on-chronic liver failure has been reported in recent studies. The technique should be limited to patients with a transplant project, to centers experienced in the management of advanced liver disease. The use of extracorporeal albumin dialysis could be beneficial in selected patients with advanced liver diseases listed for transplant or with a transplant project. Waiting future large randomized controlled trials, this panel experts’ statements may help careful patient selection and better treatment modalities

Routine detection of serum anti-desmocollin autoantibodies is only useful in patients with atypical pemphigus

Autoantibodies against the 3 desmocollin (Dsc; Dsc1-Dsc3) isoforms have been described in different pemphigus variants. Here, we developed state-of-the-art detection systems for serum anti-Dsc1, Dsc2 and Dsc1 IgG and IgA. These assays were applied in 5 different cohorts including pemphigus vulgaris (PV) patients with compatible direct immunofluorescence (IF) microscopy but no reactivity against desmogleins 1 and 3 (n = 24) and sera from patients with autoimmune blistering diseases with positive direct IF microscopy taken at the time of diagnosis (n = 749). We found that detection of anti-Dsc serum reactivity is not helpful in the routine diagnosis of PV, pemphigus foliaceus and paraneoplastic pemphigus but may be valuable in pemphigus vegetans

Multicenter prospective study on multivariant diagnostics of autoimmune bullous dermatoses using the BIOCHIPTM technology.

BACKGROUND The current standard in the serological diagnosis of autoimmune bullous diseases (AIBD) is a multistep procedure sequentially applying different assays. In contrast, the BIOCHIPTM mosaic technology combines multiple substrates for parallel analysis by indirect immunofluorescence (IF). METHODS Sera from 749 consecutive, prospectively recruited, direct IF positive AIBD patients from 13 international study centers were analyzed independently and blinded using (i) a BIOCHIPTM mosaic including primate esophagus, salt-split skin, recombinant BP180 NC16A and gliadin GAF3x as well as HEK293 cells expressing recombinant desmoglein1, desmoglein3, type VII collagen, and BP230 C-terminus and (ii) the conventional multistep approach of the Department of Dermatology, University of Lübeck. RESULTS In 731 of 749 sera (97.6%) specific autoantibodies could be detected using the BIOCHIPTM mosaic, similar to the conventional procedure (725 cases, 96.8%). Cohens κ for both serological approaches ranged from 0.84 to 1.00. In 6.5% of sera, differences between the two approaches occurred and were mainly attributed to autoantigen fragments not present on the BIOCHIPTM mosaic. LIMITATIONS Laminin 332 and laminin γ1 are not represented on the BIOCHIPTM mosaic. CONCLUSIONS The BIOCHIPTM mosaic is a standardized, time- and serum-saving approach that further facilitates the serological diagnosis of AIBD