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.
Derivation and validation of four patient clusters in Still's disease, results from GIRRCS AOSD-study group and AIDA Network Still Disease Registry / Ruscitti, P.; Masedu, F.; Vitale, A.; Di Cola, I.; Caggiano, V.; Di Muzio, C.; Cipriani, P.; Valenti, M.; Berardicurti, O.; Navarini, L.; Iacono, D.; Pantano, I.; Mauro, D.; Ciccia, F.; Rossi, S.; De Stefano, L.; Monti, S.; Bugatti, S.; Montecucco, C.; Caso, F.; Costa, L.; Prete, M.; Perosa, F.; Iagnocco, A.; Atzeni, F.; Guggino, G.; Giardini, H.; Antonelli, I. P. D. B.; Almaghlouth, I. A.; Asfina, K.; Direskeneli, H.; Alibaz-Oner, F.; Sevik, G.; Tufan, A.; Sfikakis, P. P.; La Torre, F.; Hinojosa-Azaola, A.; Martin-Nares, E.; Torres-Ruiz, J.; Ragab, G.; Maggio, M. C.; Makowska, J.; Del Giudice, E.; Bartoloni, E.; Emmi, G.; Govoni, M.; Lo Gullo, A.; Lopalco, G.; Simonini, G.; Fotis, L.; Ogunjimi, B.; Tharwat, S.; Frediani, B.; Maier, A.; Carubbi, F.; Dagna, L.; Erten, S.; Gidaro, A.; Hernandez-Rodriguez, J.; Sfriso, P.; Fabiani, C.; Giacomelli, R.; Cantarini, L.. - In: RMD OPEN. - ISSN 2056-5933. - 9:4(2023). [10.1136/rmdopen-2023-003419]
Derivation and validation of four patient clusters in Still's disease, results from GIRRCS AOSD-study group and AIDA Network Still Disease Registry
Caso F.;Costa L.;
2023
Abstract
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
