Efficacy of canakinumab in patients with Still's disease across different lines of biologic therapy: real-life data from the International AIDA Network Registry for Still's Disease

Introduction: The effectiveness of canakinumab may change according to the different times it is used after Still's disease onset. This study aimed to investigate whether canakinumab (CAN) shows differences in short- and long-term therapeutic outcomes, according to its use as different lines of biologic treatment.Methods: Patients included in this study were retrospectively enrolled from the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Still's disease. Seventy-seven (51 females and 26 males) patients with Still's disease were included in the present study. In total, 39 (50.6%) patients underwent CAN as a first-line biologic agent, and the remaining 38 (49.4%) patients were treated with CAN as a second-line biologic agent or subsequent biologic agent.Results: No statistically significant differences were found between patients treated with CAN as a first-line biologic agent and those previously treated with other biologic agents in terms of the frequency of complete response (p =0.62), partial response (p =0.61), treatment failure (p >0.99), and frequency of patients discontinuing CAN due to lack or loss of efficacy (p =0.2). Of all the patients, 18 (23.4%) patients experienced disease relapse during canakinumab treatment, 9 patients were treated with canakinumab as a first-line biologic agent, and nine patients were treated with a second-line or subsequent biologic agent. No differences were found in the frequency of glucocorticoid use (p =0.34), daily glucocorticoid dosage (p =0.47), or concomitant methotrexate dosage (p =0.43) at the last assessment during CAN treatment.Conclusion: Canakinumab has proved to be effective in patients with Still's disease, regardless of its line of biologic treatment

Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry

: To characterize clinical and laboratory signs of patients with still's disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. patients with still's disease classified according to internationally accepted criteria were enrolled in the autoInflammatory disease alliance (AIDA) still's disease registry. clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still's disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). at multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9-52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9-97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still's disease onset (OR 0.6, 95% CI 0.4-0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01-0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0-0.2, p = 0.008) resulted to be protective. clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data

Örgüt kültürü, örgüt yapısı ve bilgi yönetim süreçleri arasındaki ilişkiler : ilaç sektöründe bir araştırma

Bilgi yönetimi dijitalleşen dünyamızda örgütler için rekabet avantajı sağlayan önemli bir faktördür. Bilgiyi elde eden, kullanılabilir hale getiren, kullanan ve koruyabilen örgütler rakiplerinden farklılaşmaktadırlar. Örgütlerde bilgi yönetimini etkileyen çeşitli faktörler karşımıza çıkmaktadır. Bunlardan başlıcaları; kültür, yapı, teknoloji, liderlik ve strateji unsurlarıdır. Bu çalışmada ise bu unsurlardan kültür ve yapı ele alınmıştır. Bu çalışmanın amacı örgüt kültürü ve örgüt yapısının bilgi yönetim süreçleri üzerindeki etkisini araştırmaktır. Araştırmada veri toplama yöntemi olarak anket tekniği kullanılmıştır. Elde edilen veriler SPSS 22 programı kullanılarak analiz edilmiştir. Araştırma ilaç sektöründe faaliyet gösteren 12 işletmenin 148 çalışanı üzerinde gerçekleştirilmiştir. Anket çalışmasına katılım gösteren bireyler gönüllülük esasına göre belirlenmiştir. Araştırma sonucunda; örgüt kültürünün klan ve adhokrasi kültürü ve hiyerarşi kültürü olarak adlandırılan iki boyutu ile bilgi yönetim süreçleri arasında pozitif yönlü bir ilişki bulunmuştur. Örgüt yapısı ile bilgi yönetim süreçleri arasındaki ilişkide ise; örgüt yapısı alt boyutlarından formalleşme ile bilgi yönetim süreçleri arasında pozitif yönlü bir ilişki bulunurken; merkezileşme ile bilgi yönetim süreçleri arasında negatif yönlü bir ilişki bulunmuştur.--------------------Knowledge management is a crucial factor that gain competitive advantage for organisations in digitised world. Organisations which have knowledge, use that knowledge correctly and preserved well become different from their competitors. There are many factors that affects knowledge management in organisations. These are primarily culture, structure, technology, leadership and strategical factors. In this study, those which culture and structure was addressed from among these factors. The aim of this study is analysing the effects organisational culture and organisational structure on the process of knowledge management. In this study, survey method was used as a data collection method. Data that are obtained was analysed with the use of SPSS 22 program. The study was conducted on 148 employees of 12 companies operating in pharmaceutical industry. Individuals who participated in the survey was based on a volunteer basis. At the end of the study, there is a positive relation between knowledge management process and clan & adhocracy and hieararchy cultures that are subdimensions of the organizational cultures. In the relationship between organizational structure and information management processes; while there is a positive relationship between formalization, that is one of the subdimensions of the organizational structure, and knowledge management processes; there is a negative relationship between centralization and knowledge management processes

Örgüt kültürü, örgüt yapısı ve bilgi yönetim süreçleri arasındaki ilişkiler : ilaç sektöründe bir araştırma

Bilgi yönetimi dijitalleşen dünyamızda örgütler için rekabet avantajı sağlayan önemli bir faktördür. Bilgiyi elde eden, kullanılabilir hale getiren, kullanan ve koruyabilen örgütler rakiplerinden farklılaşmaktadırlar. Örgütlerde bilgi yönetimini etkileyen çeşitli faktörler karşımıza çıkmaktadır. Bunlardan başlıcaları; kültür, yapı, teknoloji, liderlik ve strateji unsurlarıdır. Bu çalışmada ise bu unsurlardan kültür ve yapı ele alınmıştır. Bu çalışmanın amacı örgüt kültürü ve örgüt yapısının bilgi yönetim süreçleri üzerindeki etkisini araştırmaktır. Araştırmada veri toplama yöntemi olarak anket tekniği kullanılmıştır. Elde edilen veriler SPSS 22 programı kullanılarak analiz edilmiştir. Araştırma ilaç sektöründe faaliyet gösteren 12 işletmenin 148 çalışanı üzerinde gerçekleştirilmiştir. Anket çalışmasına katılım gösteren bireyler gönüllülük esasına göre belirlenmiştir. Araştırma sonucunda; örgüt kültürünün klan ve adhokrasi kültürü ve hiyerarşi kültürü olarak adlandırılan iki boyutu ile bilgi yönetim süreçleri arasında pozitif yönlü bir ilişki bulunmuştur. Örgüt yapısı ile bilgi yönetim süreçleri arasındaki ilişkide ise; örgüt yapısı alt boyutlarından formalleşme ile bilgi yönetim süreçleri arasında pozitif yönlü bir ilişki bulunurken; merkezileşme ile bilgi yönetim süreçleri arasında negatif yönlü bir ilişki bulunmuştur. -------------------- Knowledge management is a crucial factor that gain competitive advantage for organisations in digitised world. Organisations which have knowledge, use that knowledge correctly and preserved well become different from their competitors. There are many factors that affects knowledge management in organisations. These are primarily culture, structure, technology, leadership and strategical factors. In this study, those which culture and structure was addressed from among these factors. The aim of this study is analysing the effects organisational culture and organisational structure on the process of knowledge management. In this study, survey method was used as a data collection method. Data that are obtained was analysed with the use of SPSS 22 program. The study was conducted on 148 employees of 12 companies operating in pharmaceutical industry. Individuals who participated in the survey was based on a volunteer basis. At the end of the study, there is a positive relation between knowledge management process and clan & adhocracy and hieararchy cultures that are subdimensions of the organizational cultures. In the relationship between organizational structure and information management processes; while there is a positive relationship between formalization, that is one of the subdimensions of the organizational structure, and knowledge management processes; there is a negative relationship between centralization and knowledge management processes

Derivation and validation of four patient clusters in Still's disease, results from GIRRCS AOSD-study group and AIDA Network Still Disease Registry

Background: Different patient clusters were preliminarily suggested to dissect the clinical heterogeneity in Still's disease. Thus, we aimed at deriving and validating disease clusters in a multicentre, observational, prospective study to stratify these patients. Methods: Patients included in GIRRCS AOSD-study group and AIDA Network Still Disease Registry were assessed if variables for cluster analysis were available (age, systemic score, erythrocyte sedimentation rate (ESR), C reactive protein (CRP) and ferritin). K-means algorithm with Euclidean metric and Elbow plot were used to derive an adequate number of clusters. Results: K-means clustering assessment provided four clusters based on means standardised according to z-scores on 349 patients. All clusters mainly presented fever, skin rash and joint involvement. Cluster 1 was composed by 115 patients distinguished by lower values of age and characterised by skin rash myalgia, sore throat and splenomegaly. Cluster 2 included 128 patients identified by lower levels of ESR, ferritin and systemic score; multiorgan manifestations were less frequently observed. Cluster 3 comprised 31 patients categorised by higher levels of CRP and ferritin, they were characterised by fever and joint involvement. Cluster 4 contained 75 patients derived by higher values of age and systemic score. Myalgia, sore throat, liver involvement and life-threatening complications, leading to a high mortality rate, were observed in these patients. Conclusions: Four patient clusters in Still's disease may be recognised by a multidimensional characterisation ('Juvenile/Transitional', 'Uncomplicated', 'Hyperferritinemic' and 'Catastrophic'). Of interest, cluster 4 was burdened by an increased rate of life-threatening complications and mortality, suggesting a more severe patient group

Clinical and laboratory features associated with macrophage activation syndrome in Still's disease : data from the international AIDA Network Still's Disease Registry

Abstract: To characterize clinical and laboratory signs of patients with Still's disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. Patients with Still's disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Alliance (AIDA) Still's Disease Registry. Clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still's disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). At multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9-52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9-97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still's disease onset (OR 0.6, 95% CI 0.4-0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01-0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0-0.2, p = 0.008) resulted to be protective. Clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data