Search results for "canakinumab"

Familial Mediterranean Fever: an unusual cause of liver disease

2019

Abstract Background Familial Mediterranean Fever is an autoinflammatory disease typically expressed with recurrent attacks of fever, serositis, aphthous stomatitis, rash. Only a few reports describe the association with hepatic involvement. Case presentation We describe the clinical case of a child affected, since the age of 1 year, by recurrent fever, aphthous stomatitis, rash, arthralgia, associated with abdominal pain, vomiting, lymphadenopathy. The diagnosis of Familial Mediterranean Fever was confirmed by the genetic study of MEFV gene; the homozygous mutation M694 V in exon was documented. A partial control of attacks was obtained with colchicine. The child continued to manifest only …

Trouble always comes in threes: three mutations for three auto inflammatory genes in a child and in his father

2014

The coexistence of mutations in more than one gene, linked to Autoinflammatory Diesases, can confuse and make difficult the diagnosis and management of these patients, especially in childhood, when the clinical history is still brief.

VALUTAZIONE DELLA CRESCITA IN PAZIENTI CON ARTRITE IDIOPATICA GIOVANILE SISTEMICA

2021

IL-1 BLOCKADE IN PAEDIATRIC RECURRENT PERICARDITIS: A MULTICENTRIC RETROSPECTIVE STUDY OF THE ITALIAN COHORT

2019

Introduction: Acute pericarditis is an inflammatory condition causing the occurrence of pericardial effusion. In a third of patients, the disease is recurrent. First line treatment of idiopathic pericarditis consists in non-steroidal anti-inflammatory drugs (NSAIDs) and colchicine; glucocorticoids represent the second line treatment in resistant or intolerant cases. A recent clinical trial has enlightened the effectiveness of anakinra in adults and paediatric patients with colchicine-resistant recurrent pericarditis. Objectives: To describe the clinical characteristics and response to treatment in a cohort of paediatric patients with recurrent pericarditis treated with IL inhibitors. Method…

Canakinumab in systemic juvenile idiopathic arthritis: clinical inactive disease rate and safety in italian patients

2019

Introduction: Systemic juvenile idiopathic arthritis (sJIA) is a polygenic autoinflammatory disease. The pathophysiology is still unclear, it is now well known that innate immune mechanisms play a central role with overproduction of inflammatory cytokines. The increased knowledge on the role of these cytokines has provided a change in the natural history of the disease with the introduction of the targeted treatments. Remarkable results has been observed with canakinumab, an anti-interleukin-1β monoclonal antibody, in two clinical trials but little information are available in real life. Objectives: To evaluate clinical inactive disease rate and safety of canakinumab in Italian patients wit…

Tasso di persistenza in terapia di anakinra e canakinumab nelle malattie autoinfiammatorie monogeniche: studio osservazionale dal registro internazio…

2021

SAT0511 CANAKINUMAB IN SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS: CLINICAL INACTIVE DISEASE RATE AND SAFETY IN ITALIAN PATIENTS

2019

Background: Systemic juvenile idiopathic arthritis (sJIA) is a polygenic autoinflammatory disease. The innate immune mechanisms play a central role with overproduction of inflammatory cytokines. The increased knowledge on the role of these cytokines has provided a change in the natural history of the disease with the introduction of the targeted treatments. Remarkable results has been observed with canakinumab, an anti-interleukin-1β monoclonal antibody, in two clinical trials but little information are available in real life. Objectives: To evaluate clinical inactive disease rate and safety of canakinumab in Italian patients with sJIA. Methods: We have collected retrospectively clinical an…

Canakinumab in systemic juvenile idiopathic arthritis: real-world data from a retrospective Italian cohort

2021

Abstract Objective The objective of this study was to use real-world data to evaluate the effectiveness and safety of canakinumab in Italian patients with systemic JIA (sJIA). Methods A retrospective multicentre study of children with sJIA was performed. Clinical features, laboratory parameters and adverse events were collected at baseline, and 6 and 12 months after starting canakinumab. The primary outcome measure of effectiveness was clinically inactive disease (CID) off glucocorticoids (GCs) treatment at 6 months. Results A total of 80 children from 15 Italian centres were analysed. Of the 12 patients who started canakinumab in CID while receiving anakinra, all maintained CID. Of the 68 …

A Snapshot on the On-Label and Off-Label Use of the Interleukin-1 Inhibitors in Italy among Rheumatologists and Pediatric Rheumatologists: A Nationwi…

2016

Background: Interleukin (IL)-1 inhibitors have been suggested as possible therapeutic options in a large number of old and new clinical entities characterized by an IL-1 driven pathogenesis. Objectives: To perform a nationwide snapshot of the on-label and off-label use of anakinra (ANA) and canakinumab (CAN) for different conditions both in children and adults. Methods: We retrospectively collected demographic, clinical, and therapeutic data from both adult and pediatric patients treated with IL-1 inhibitors from January 2008 to July 2016. Results: Five hundred and twenty-six treatment courses given to 475 patients (195 males, 280 females; 111 children and 364 adults) were evaluated. ANA wa…

Early Treatment of Systemic Juvenile Idiopathic Arthritis with Canakinumab and Complete Remission After 2 Years of Treatment Suspension: Case Report …

2019

Systemic juvenile idiopathic arthritis (sJIA) is an autoinflammatory disease characterised by fever and arthritis. We describe the case of a 14-year-old girl hospitalised with fever associated with rash, myalgia, arthralgia and polyarticular involvement. Examinations revealed increased levels of C-reactive protein, erythrocyte sedimentation rate, ferritin, triglycerides, leukocytes, neutrophils, lactate dehydrogenase, fibrinogen, aspartate aminotransferase (AST), alanine aminotransferase (ALT) and γ-glutamyl transferase (GGT). Bone marrow biopsy showed polyclonal leukocyte activation. A genetic study revealed a heterozygous mutation of the MEFV gene, c.442G>C (E148Q), which is typical of…