Canakinumab treat-to target strategies increase complete response rate in CAPS

نویسندگان

  • J Kuemmerle-Deschner
  • F Hofer
  • T Endres
  • B Kortus-Goetze
  • N Blank
  • E Weißbarth-Riedel
  • C Schuetz
  • T Kallinich
  • K Krause
  • C Rietschel
  • G Horneff
  • SM Benseler
چکیده

Objective Cryopyrin-associated periodic syndrome (CAPS) is a heterogeneous group of diseases characterized by excessive Interleukin-1b (IL-1 b) release resulting in severe systemic and organ inflammation. Canakinumab targets IL-1b and is approved at standard dose for children and adults with all CAPS phenotypes. Limited data are available regarding real-life effectiveness of canakinumab in patients living with CAPS. Therefore the aim of the study was to evaluate the real-life dosing practice and effectiveness of canakinumab in CAPS.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Dosing patterns of canakinumab in patients with Cryopyrin-Associated Periodic Syndromes (CAPS): A comparative analysis of a study in Western versus Japanese patients

Background CAPS is an orphan auto-inflammatory disease, generally diagnosed in childhood that requires life-long treatment. Canakinumab, a fully human anti-IL-1b antibody, has previously demonstrated rapid, complete and sustained response in CAPS patients. Aim To compare dosing patterns of canakinumab in pediatric and adult CAPS patients of a predominantly Western population (WP) vs Japanese pa...

متن کامل

Canakinumab (ACZ885, a fully human IgG1 anti-IL-1β mAb) induces sustained remission in pediatric patients with cryopyrin-associated periodic syndrome (CAPS)

INTRODUCTION Cryopyrin-associated periodic syndrome (CAPS) represents a spectrum of three auto-inflammatory syndromes, familial cold auto-inflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease/chronic infantile neurological cutaneous and articular syndrome (NOMID/CINCA) with etiology linked to mutations in the NLRP3 gene resulting in eleva...

متن کامل

Canakinumab (ILARIS®) in cryopyrin-associated periodic syndrome (CAPS) patients below 2 years of age: Review of four cases and literature.

Curr Pediatr Res 2017 Volume 21 Issue 4 646 Cryopyrin-Associated Periodic Syndrome (CAPS) is combination of different auto-inflammatory hereditary disorders with same genetic basis. CAPS caused mostly due to mutation of NLRP3 gene (nucleotide-binding domain, leucine-rich family (NLR), pyrin domain containing 3), which is an early onset in children. There are many anti-INF agents, anti-IL blocke...

متن کامل

In vivo regulation of interleukin 1β in patients with cryopyrin-associated periodic syndromes

The investigation of interleukin 1beta (IL-1beta) in human inflammatory diseases is hampered by the fact that it is virtually undetectable in human plasma. We demonstrate that by administering the anti-human IL-1beta antibody canakinumab (ACZ885) to humans, the resulting formation of IL-1beta-antibody complexes allowed the detection of in vivo-produced IL-1beta. A two-compartment mathematical m...

متن کامل

A decade of anti-IL-1 therapy in CAPS - a spectrum of efficacy in this spectrum of diseases

Results Patients 1 (male; age at treatment: 40; mutation in NLRP3: T436I; phenotype: CINCA) and 2 (female; 43; R260W; MWS) experienced modest and transient reductions of SAA and symptoms when treated with canakinumab. Both patients developed marked inflammation with morphea-like rash shortly afterwards. Both were switched to anakinra with excellent effect. Patient 3 (male; 47; A439V; FCAS) weig...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:

دوره 13  شماره 

صفحات  -

تاریخ انتشار 2015