Jasmin B Kuemmerle-Deschner
- Methodological challenges in monitoring new treatments for rare diseases: lessons from the cryopyrin-associated periodic syndrome registryHugh Tilson
Novartis Pharma AG, Basel, Switzerland
Orphanet J Rare Dis 8:139. 2013..Creative approaches to observational methodology are needed, harnessing novel registry strategies to ensure Health Care Provider reporting and patient monitoring...
- Abatacept in difficult-to-treat juvenile idiopathic arthritisJasmin B Kuemmerle-Deschner
Pediatric Rheumatology Clinics, Dept of Pediatrics, University Hospital Tubingen, Germany
Biologics 2:865-74. 2008..Although information on ABA in JIA is still limited, available data suggest a potential role in difficult to treat JIA patients previously treated with other biologic agents and for non-responders to TNF-blockade...
- [Cryopyrin-associated periodic syndrome]J B Kümmerle-Deschner
Klinik fur Kinder und Jugendmedizin, Abteilung für pädiatrische Rheumatologie, Autoinflammation Reference Center Tübingen, Universitatsklinikum Tubingen, Hoppe Seyler Str 1, 72076, Tubingen, Deutschland
Z Rheumatol 71:199-208. 2012..To prevent severe and possible life-threatening disease sequelae, early and correct diagnosis and immediate initiation of therapy are mandatory...
- NLRP3 E311K mutation in a large family with Muckle-Wells syndrome--description of a heterogeneous phenotype and response to treatmentJasmin B Kuemmerle-Deschner
Division of Pediatric Rheumatology, Dept, of Pediatrics, University Hospital Tubingen, Hoppe Seyler Straße 1, 72076 Tuebingen, Germany
Arthritis Res Ther 13:R196. 2011..The NLRP3/CIAS1 E311K mutation caused a heterogeneous phenotype of MWS in a large family. This study analyzes the clinical spectrum, patterns of inflammatory parameters and reports on response to treatment...
- Safety and efficacy of once-weekly application of Etanercept in children with juvenile idiopathic arthritisJasmin B Kuemmerle-Deschner
Division of Pediatric Rheumatology, University Children s Hospital Tubingen, Tubingen, Germany
Rheumatol Int 28:153-6. 2007..At last observation 10/12 patients achieved an ACR-JRA 30 and 8/12 achieved an ACR-JRA70 response. These data indicate that once weekly application of etanercept is safe and efficacous in children...
- Two-year results from an open-label, multicentre, phase III study evaluating the safety and efficacy of canakinumab in patients with cryopyrin-associated periodic syndrome across different severity phenotypesJasmin B Kuemmerle-Deschner
Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tubingen, Germany
Ann Rheum Dis 70:2095-102. 2011..Longer-term effects of prolonged selective interleukin-1β blockade with canakinumab were evaluated in the largest cohort of cryopyrin-associated periodic syndrome (CAPS) patients studied to date...
- Efficacy and safety of anakinra therapy in pediatric and adult patients with the autoinflammatory Muckle-Wells syndromeJasmin B Kuemmerle-Deschner
University Hospital Tubingen, Tubingen, Germany
Arthritis Rheum 63:840-9. 2011..The recombinant IL-1 receptor antagonist anakinra blocks the biologic activity of IL-1. The aim of this study was to determine the short- and long-term efficacy and safety of anakinra therapy in children and adults with severe MWS...
- Canakinumab (ACZ885, a fully human IgG1 anti-IL-1β mAb) induces sustained remission in pediatric patients with cryopyrin-associated periodic syndrome (CAPS)Jasmin B Kuemmerle-Deschner
Division of Pediatric Rheumatology, Department of Pediatrics, University Children s Hospital Tuebingen, Hoppe Seyler Strasse 1, 72076 Tubingen, Tuebingen, Germany
Arthritis Res Ther 13:R34. 2011..Canakinumab, a fully human anti-IL-1β antibody, produces sustained selective inhibition of IL-1β. This study was conducted to assess the efficacy, safety, and pharmacokinetics of canakinumab in the treatment of pediatric CAPS patients...
- Interferon-alpha--a new therapeutic option in refractory juvenile Behçet's disease with CNS involvementJ B Kuemmerle-Deschner
Division of Paediatric Rheumatology, University Children s Hospital Tuebingen, Germany
Rheumatology (Oxford) 47:1051-3. 2008..To report the successful treatment with recombinant human IFN- alpha 2a (rhIFN-alpha2a) in two male adolescents suffering from severe treatment-resistant Behçet's disease (BD) with central nervous system (CNS) involvement...
- Hearing loss in Muckle-Wells syndromeJasmin B Kuemmerle-Deschner
University Hospital Tuebingen, Tuebingen, Germany
Arthritis Rheum 65:824-31. 2013..The aims of this study were to determine the otologic characteristics of MWS, define trajectories of hearing loss, and explore the association with distinct NLRP3 genotypes...
- Challenges in diagnosing Muckle-Wells syndrome: identifying two distinct phenotypesJasmin B Kuemmerle-Deschner
University Hospital Tubingen, Tubingen, Germany
Arthritis Care Res (Hoboken) 66:765-72. 2014..The aims of this study were to describe key elements of the diagnostic evaluation process in MWS and compare identified variables between patients diagnosed in childhood and adulthood...
- Progressive familial hearing loss in Muckle-Wells syndromeAssen Koitschev
Department of Otorhinolaryngology, Klinikum Stuttgart, University Hospital Tubingen, Germany
Acta Otolaryngol 132:756-62. 2012..The design of further clinical trials should focus on hearing in order to document the long-term effect of anti-interleukin (IL)-1 drugs on hearing preservation...
- Inflammasome and cytokine blocking strategies in autoinflammatory disordersMonika Moll
Division of Pediatric Rheumatology, Department of Pediatrics, University Children s Hospital Tuebingen, Germany
Clin Immunol 147:242-75. 2013..IL-1 inhibition to date is the most successful specific therapy in autoinflammatory disorders. Here, current treatment strategies in autoinflammatory disorders are reviewed with a focus on inflammasome and cytokine inhibition...