Pregled bibliografske jedinice broj: 673223
Comparison of sensitivity and specificity of MAS and HLH diagnostic guidelines in 362 children with MAS complicating systemic JIA
Comparison of sensitivity and specificity of MAS and HLH diagnostic guidelines in 362 children with MAS complicating systemic JIA // European Paediatric Rheumatology 20th Congress
Ljubljana, Slovenija, 2013. (poster, međunarodna recenzija, sažetak, znanstveni)
CROSBI ID: 673223 Za ispravke kontaktirajte CROSBI podršku putem web obrasca
Naslov
Comparison of sensitivity and specificity of MAS and HLH diagnostic guidelines in 362 children with MAS complicating systemic JIA
Autori
Davì, Sergio ; Minoia, Francesca ; Horne, Anna Carin ; Demirkaya, Erkan ; Ilowite, Norman ; Insalaco , Antonella ; Ioseliani, Maka ; Jelušić-Dražić , Marija ; Kapović, Agneza Marija ; Kasapcopur, Ozgur ; Kone-Paut, Isabelle ; Lepore, Loredana ; Li, Caifeng ; Magni-Manzoni, Silvia ; Maritsi, Despoina ; McCurdy, Deborah ; Merino, Rosa ; Miettunen, Paivi ; Ruperto, Nicolino ; Martini, Alberto ; Cron, Randy ; Ravelli, Angelo
Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni
Skup
European Paediatric Rheumatology 20th Congress
Mjesto i datum
Ljubljana, Slovenija, 25.09.2013. - 29.09.2013
Vrsta sudjelovanja
Poster
Vrsta recenzije
Međunarodna recenzija
Ključne riječi
sensitivity; specificity; diagnostic guidelines; macrophage activation syndrome; juvenile idiopathic arthritis; systemic;
Sažetak
Introduction: Early diagnosis of macrophage activations syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA) may be challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication. However, the diagnostic value of the guidelines for hemophagocytic lymphohistiocytosis (HLH) or sJIA-associated MAS has seldom been examined. Objectives: To investigate the sensitivity and specificity of diagnostic guidelines for HLH and sJIA-associated MAS in patients with sJIA who developed MAS. Methods: The study sample included 362 children with sJIA who had MAS (diagnosed and treated as such by the attending physician) and 2 control groups with potentially “confusable” conditions, including active sJIA without MAS (n=404) and a systemic febrile infection requiring hospitalization (n=345). Diagnostic guidelines for HLH and sJIA-associated MAS were applied to all MAS and control patients. Because no patient had NK-cell activity and soluble CD25 determination available and bone marrow aspirate was performed in only a few patients, these 3 criteria were excluded from HLH guidelines. HLH criteria were, therefore, met when at least 4 of the 5 remaining variables were present. sJIA-associated MAS criteria were met when at least 2 laboratory criteria or at least 1 laboratory criterion and 1 clinical criterion were present. Sensitivity and specificity of guidelines in discriminating patients with MAS from control patients were assessed. Results: The table shows the comparison of sensitivity and specificity of diagnostic guidelines. Conclusion: The diagnostic guidelines for sJIA-associated MAS revealed strong sensitivity and specificity, whereas HLH guidelines were highly specific, but lacked sensitivity. Sensitivity of HLH was mostly hampered by the excessive stringent threshold for cytopenia and hypofibrinogenemia, and the infrequent occurrence of splenomegaly in patients with MAS.
Izvorni jezik
Engleski
Znanstvena područja
Kliničke medicinske znanosti
POVEZANOST RADA
Projekti:
072-1080229-0337 - Modulacija funkcije ljudskih regulacijskih T-limfocita (Gagro, Alenka, MZOS ) ( CroRIS)
Ustanove:
Klinika za dječje bolesti Medicinskog fakulteta,
Medicinski fakultet, Zagreb,
Klinički bolnički centar Zagreb