A5051230084- Autoimmune and Inflammatory Disorders Research
- Inflammasome and immune disorders
- Adolescent and Pediatric Healthcare
- IL-33, ST2, and ILC Pathways
- Immunodeficiency and Autoimmune Disorders
- Acute Lymphoblastic Leukemia research
- Systemic Lupus Erythematosus Research
- Kawasaki Disease and Coronary Complications
- Rheumatoid Arthritis Research and Therapies
- Toxin Mechanisms and Immunotoxins
- Eosinophilic Disorders and Syndromes
- Dermatology and Skin Diseases
- Urticaria and Related Conditions
- Childhood Cancer Survivors' Quality of Life
- interferon and immune responses
- Ocular Diseases and Behçet’s Syndrome
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Gout, Hyperuricemia, Uric Acid
- Inflammatory Myopathies and Dermatomyositis
- Otitis Media and Relapsing Polychondritis
- Osteomyelitis and Bone Disorders Research
- Hematological disorders and diagnostics
- Immune Response and Inflammation
- Streptococcal Infections and Treatments
- Lymphoma Diagnosis and Treatment
University Children's Hospital Tübingen
2015-2024
University of Tübingen
2015-2024
Universitätsklinikum Tübingen
2009-2024
Universitätskinderklinik
2021-2023
Center for Rheumatology
2022
University Children’s Hospital Basel
2021
University of Basel
2021
Klinik und Poliklinik für Kinder- und Jugendmedizin
2011-2021
Charité - Universitätsmedizin Berlin
2007-2020
Medizinische Hochschule Hannover
2020
The cryopyrin-associated periodic syndrome (CAPS) is a rare inherited inflammatory disease associated with overproduction of interleukin-1. Canakinumab human anti-interleukin-1beta monoclonal antibody.We performed three-part, 48-week, double-blind, placebo-controlled, randomized withdrawal study canakinumab in patients CAPS. In part 1, 35 received 150 mg subcutaneously. Those complete response to treatment entered 2 and were randomly assigned receive either or placebo every 8 weeks for up 24...
The objective of this work was to develop and validate a set clinical criteria for the classification patients affected by periodic fevers. Patients with inherited fevers (familial Mediterranean fever (FMF); mevalonate kinase deficiency (MKD); tumour necrosis factor receptor-associated syndrome (TRAPS); cryopyrin-associated syndromes (CAPS)) enrolled in Eurofever Registry up until March 2013 were evaluated. fever, aphthosis, pharyngitis adenitis (PFAPA) used as negative controls. For each...
<h3>Background</h3> We aimed to describe the main features of Behçet9s disease (BD) in children largest prospective cohort date and propose a classification. <h3>Methods</h3> An international expert consensus group was formed define data set minimal symptoms for inclusion patients. Patients were entered prospectively during 66 months. Experts classified patients on basis. The concordance two classifications analysed confirmed with BD. Comparisons subgroups helped criteria. BD-associated...
<h3>Background</h3> The interleukin-1 (IL-1) mediated systemic autoinflammatory diseases, including the cryopyrin-associated periodic syndromes (CAPS), tumour necrosis factor receptor-associated syndrome (TRAPS), mevalonate kinase deficiency (MKD) and of IL-1 receptor antagonist (DIRA), belong to a group rare immunodysregulatory diseases that primarily present in early childhood with variable multiorgan involvement. When untreated, patients severe clinical phenotypes have poor prognosis,...
Chronic anterior uveitis in children often takes a serious course. Despite various immunosuppressive drugs some do not respond sufficiently and there is high risk of them becoming seriously disabled. Anti-TNF alpha therapy has been shown by our group others to be mostly ineffective (Etanercept) or partly effective (Infliximab) with the anaphylactic reactions. Here we report on 18 young patients treated Adalimumab (Humira), complete humanised anti-TNF antibody.
Fever of unknown origin (FUO) in children presents a diagnostic challenge. The differential diagnosis includes systemic-onset juvenile idiopathic arthritis (JIA), an autoinflammatory syndrome associated with activation phagocytic cells that, at presentation, is difficult to differentiate from severe systemic infections. aim this study was investigate whether serum concentrations the proinflammatory protein S100A12 may help deciding treat patients FUO antibiotics or immunosuppressive...
Muckle-Wells syndrome (MWS) is an inherited autoinflammatory disease caused by mutations in the NLRP3 gene that result excessive interleukin-1 (IL-1) release. It characterized severe fevers, rashes, arthralgia, and conjunctivitis, leading to sensorineural deafness amyloidosis. The recombinant IL-1 receptor antagonist anakinra blocks biologic activity of IL-1. aim this study was determine short- long-term efficacy safety therapy children adults with MWS.A single-center observational...
Chronic non-bacterial osteomyelitis (CNO) or chronic recurrent multifocal (CRMO) is an autoinflammatory disorder characterized by sterile bone osteolytic lesions. The aim of this study was to evaluate the demographic data and clinical, instrumental therapeutic features at baseline in a large series CNO/CRMO patients enrolled Eurofever registry. A web-based registry collected retrospective on affected CRMO/CNO. Both paediatric adult centres were involved. Complete information 486 available...
<h3>Objectives</h3> To validate the Auto-Inflammatory Diseases Activity Index (AIDAI) in four major hereditary recurrent fever syndromes (HRFs): familial Mediterranean (FMF), mevalonate kinase deficiency (MKD), tumour necrosis factor receptor-associated periodic syndrome (TRAPS) and cryopyrin-associated (CAPS). <h3>Methods</h3> In 2010, an international collaboration established content of a disease activity tool for HRFs. Patients completed 1-month prospective diary with 12 yes/no items...
To assess the effect of canakinumab, a fully human anti-interleukin-1β antibody, on symptoms and health-related quality life (HRQoL) in patients with cryopyrin-associated periodic syndrome (CAPS). In this 48-week, phase 3 study, CAPS received canakinumab 150 mg subcutaneously at 8-week intervals. All (n = 35) during weeks 1 through 8; 9 24 constituted double-blind placebo-controlled withdrawal phase, 48 an open-label which all canakinumab. Patient physician assessments symptoms, levels...
Cryopyrin-associated periodic syndrome (CAPS) represents a spectrum of three auto-inflammatory syndromes, familial cold (FCAS), Muckle-Wells (MWS), and neonatal-onset multisystem inflammatory disease/chronic infantile neurological cutaneous articular (NOMID/CINCA) with etiology linked to mutations in the NLRP3 gene resulting elevated interleukin-1β (IL-1β) release. CAPS is rare hereditary disease, which may start early childhood requires life-long treatment. Canakinumab, fully human...
To provide outcome data concerning pregnancies exposed to the Interleukin-1 (IL-1) inhibitors prior conception in both men and women, during pregnancy breast feeding.Retrospective were collected from members of International Society for Systemic Autoinflammatory diseases collated a single centre. A uniform collection sheet was used obtain standardized including maternal age diagnosis, type, duration response IL-1 blockade, duration, delivery, mode feeding neonatal development.There 31...
Natural CD4+CD25+FoxP3+ Treg cells play a crucial role in maintaining immune homeostasis and controlling autoimmunity. In patients with juvenile idiopathic arthritis (JIA), inflammation occurs despite the increased total numbers of synovial fluid (SF) compared to peripheral blood (PB). This study was undertaken investigate phenotype CD4+ T PB SF from JIA patients, function cells, sensitivity CD4+CD25- effector immunoregulatory properties suppression cytokine secretion by cells.The phenotypes...
Objective Periodic fever syndrome (PFS) conditions are characterized by recurrent attacks of and localized inflammation. This study examined the diagnostic pathway treatments at tertiary centers for familial Mediterranean (FMF), tumor necrosis factor receptor–associated periodic (TRAPS), mevalonate kinase deficiency (MKD)/hyperimmunoglobulinemia D (HIDS). Methods PFS specialists medical in US, European Union, eastern participated a retrospective chart review, providing de‐identified data an...
Abstract Introduction While adalimumab is licensed for ankylosing spondylitis (AS), open uncontrolled studies suggest therapeutic efficacy of TNF-inhibitors in juvenile onset AS (JoAS). Methods A total 32 patients aged 12 to 17 years with severe, active and refractory JoAS were enrolled a multicenter, randomized, double-blind, placebo-controlled parallel study weeks, followed by open-label until week 24 all patients. ASAS40 was used as the primary, ASAS20, PedACR single items secondary...