Background Different patient clusters were preliminarily suggested to dissect the clinica) 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 Stili Disease Registry were assessed if variables for cluster analysis were available (age, systemic score, erythrocyte sedimentation rate (ESR), C reattive protein (CRP) and ferritin). K-means algorithm with Euclidean metric and Elbow plot were used to derive an adeguate number of clusters. Results K-means clustering assessment provided four clusters based on means standardised according to zscores on 349 patients. Ali clusters mainly presented fever, skin rash and joint involvement. Cluster 1 was conriposed 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/Transitiong, 'Uncomplicated', 'Hyperferritinemic' and 'Catastrophic'). Of interest, cluster 4 was burdened by an increased rate of ) ife-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.

Marcella Prete;Federico Perosa;Giuseppe Lopalco;
2023-01-01

Abstract

Background Different patient clusters were preliminarily suggested to dissect the clinica) 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 Stili Disease Registry were assessed if variables for cluster analysis were available (age, systemic score, erythrocyte sedimentation rate (ESR), C reattive protein (CRP) and ferritin). K-means algorithm with Euclidean metric and Elbow plot were used to derive an adeguate number of clusters. Results K-means clustering assessment provided four clusters based on means standardised according to zscores on 349 patients. Ali clusters mainly presented fever, skin rash and joint involvement. Cluster 1 was conriposed 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/Transitiong, 'Uncomplicated', 'Hyperferritinemic' and 'Catastrophic'). Of interest, cluster 4 was burdened by an increased rate of ) ife-threatening complications and mortality, suggesting a more severe patient group.
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11586/459420
 Attenzione

Attenzione! I dati visualizzati non sono stati sottoposti a validazione da parte dell'ateneo

Citazioni
  • ???jsp.display-item.citation.pmc??? 0
  • Scopus 2
  • ???jsp.display-item.citation.isi??? ND
social impact