Performances of the "MS-score" And "HScore" in the diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis patients.
Hscore
MS-score
Macrophage activation syndrome
Systemic JIA
Journal
Rheumatology international
ISSN: 1437-160X
Titre abrégé: Rheumatol Int
Pays: Germany
ID NLM: 8206885
Informations de publication
Date de publication:
Jan 2021
Jan 2021
Historique:
received:
10
10
2020
accepted:
03
11
2020
pubmed:
21
11
2020
medline:
10
11
2021
entrez:
20
11
2020
Statut:
ppublish
Résumé
Macrophage activation syndrome (MAS) is a devastating complication of systemic JIA (sJIA), seen in approximately 10-25% of the sJIA patients. A number of criteria have been proposed to differentiate between activation of sJIA and MAS, including HScore and the recently proposed MS-score. This is the first study comparing the performances of MS-score and HScore for the diagnosis of MAS in sJIA patients. Systemic JIA patients followed at Hacettepe University Pediatric Rheumatology Unit were included in the study. Clinical features and laboratory findings at the time when the disease was most active or patients were diagnosed with MAS were recorded retrospectively. HScore and MS-score were calculated and the diagnostic performance for MAS was compared by receiver operating characteristic (ROC) curve analysis. Seventy-one sJIA patients were included (23 MAS, 48 activation). There was no difference in age of onset (median 4.7 vs. 5.0 years) and gender (73.9% vs. 54.2%) between patients who had MAS and sJIA activation. Median MS-score and HScore were higher in the MAS group. ROC curve analysis revealed that the HScore performed slightly better in diagnosing MAS, compared with the MS-score (AUC = 0.965 and 0.901 for HScore and MS-score respectively, P < 0.001). In our cohort, the optimal cut-off for the MS score was ≥ - 1.64 (sensitivity: 91.3%; specificity: 83.8%) and for the HScore it was ≥ 162.5 (sensitivity: 91.3%; specificity: 90.2%). HScore performed slightly better than MS-score for the diagnosis of MAS in our cohort.
Identifiants
pubmed: 33215249
doi: 10.1007/s00296-020-04750-x
pii: 10.1007/s00296-020-04750-x
doi:
Types de publication
Comparative Study
Journal Article
Observational Study
Validation Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
87-93Références
Petty RE, Southwood TR, Manners P, Baum J, Glass DN, Goldenberg J, He X, Maldonado-Cocco J, Orozco-Alcala J, Prieur AM, Suarez-Almazor ME, Woo P, International League of Associations for R (2004) International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton 2001. J Rheumatol 31(2):390–392
pubmed: 14760812
Minoia F, Davi S, Horne A, Demirkaya E, Bovis F, Li C, Lehmberg K, Weitzman S, Insalaco A, Wouters C, Shenoi S, Espada G, Ozen S, Anton J, Khubchandani R, Russo R, Pal P, Kasapcopur O, Miettunen P, Maritsi D, Merino R, Shakoory B, Alessio M, Chasnyk V, Sanner H, Gao YJ, Huasong Z, Kitoh T, Avcin T, Fischbach M, Frosch M, Grom A, Huber A, Jelusic M, Sawhney S, Uziel Y, Ruperto N, Martini A, Cron RQ, Ravelli A, Pediatric Rheumatology International Trials O, Childhood A, Rheumatology Research A, Pediatric Rheumatology Collaborative Study G, Histiocyte S (2014) Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol 66(11):3160–3169. https://doi.org/10.1002/art.38802
doi: 10.1002/art.38802
pubmed: 25077692
Ravelli A, Grom AA, Behrens EM, Cron RQ (2012) Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment. Genes Immun 13(4):289–298. https://doi.org/10.1038/gene.2012.3
doi: 10.1038/gene.2012.3
pubmed: 22418018
Sag E, Uzunoglu B, Bal F, Sonmez HE, Demir S, Bilginer Y, Ozen S (2019) Systemic onset juvenile idiopathic arthritis: a single center experience. Turk J Pediatr 61(6):852–858. https://doi.org/10.24953/turkjped.2019.06.005
doi: 10.24953/turkjped.2019.06.005
pubmed: 32134578
Ravelli A, Minoia F, Davi S, Horne A, Bovis F, Pistorio A, Arico M, Avcin T, Behrens EM, De Benedetti F, Filipovic L, Grom AA, Henter JI, Ilowite NT, Jordan MB, Khubchandani R, Kitoh T, Lehmberg K, Lovell DJ, Miettunen P, Nichols KE, Ozen S, PachlopnikSchmid J, Ramanan AV, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner HI, Martini A, Ruperto N, Cron RQ, Paediatric Rheumatology International Trials O, Childhood A, Rheumatology Research A, Pediatric Rheumatology Collaborative Study G, Histiocyte S (2016) 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol 68(3):566–576. https://doi.org/10.1002/art.39332
doi: 10.1002/art.39332
pubmed: 26314788
Behrens EM, Beukelman T, Paessler M, Cron RQ (2007) Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis. J Rheumatol 34(5):1133–1138
pubmed: 17343315
Borgia RE, Gerstein M, Levy DM, Silverman ED, Hiraki LT (2018) Features, treatment, and outcomes of macrophage activation syndrome in childhood-onset systemic lupus erythematosus. Arthritis Rheumatol 70(4):616–624. https://doi.org/10.1002/art.40417
doi: 10.1002/art.40417
pubmed: 29342508
Poddighe D, Dauyey K (2020) Macrophage activation syndrome in juvenile dermatomyositis: a systematic review. RheumatolInt 40(5):695–702. https://doi.org/10.1007/s00296-019-04442-1
doi: 10.1007/s00296-019-04442-1
Garcia-Pavon S, Yamazaki-Nakashimada MA, Baez M, Borjas-Aguilar KL, Murata C (2017) Kawasaki disease complicated with macrophage activation syndrome: a systematic review. J PediatrHematolOncol 39(6):445–451. https://doi.org/10.1097/MPH.0000000000000872
doi: 10.1097/MPH.0000000000000872
Grom AA, Horne A, De Benedetti F (2016) Macrophage activation syndrome in the era of biologic therapy. Nat Rev Rheumatol 12(5):259–268. https://doi.org/10.1038/nrrheum.2015.179
doi: 10.1038/nrrheum.2015.179
pubmed: 27009539
pmcid: 5851441
Minoia F, Davi S, Horne A, Bovis F, Demirkaya E, Akikusa J, Ayaz NA, Al-Mayouf SM, Barone P, Bica B, Bolt I, Breda L, De Cunto C, Enciso S, Gallizzi R, Griffin T, Hennon T, Horneff G, Jeng M, Kapovic AM, Lipton JM, Magni Manzoni S, Rumba-Rozenfelde I, Magalhaes CS, Sewairi WM, Stine KC, Vougiouka O, Weaver LK, Davidsone Z, De Inocencio J, Ioseliani M, Lattanzi B, Tezer H, Buoncompagni A, Picco P, Ruperto N, Martini A, Cron RQ, Ravelli A, Pediatric Rheumatology International Trials O, Childhood A, Rheumatology Research A, Pediatric Rheumatology Collaborative Study G, Histiocyte S (2015) Dissecting the heterogeneity of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J Rheumatol 42(6):994–1001. https://doi.org/10.3899/jrheum.141261
doi: 10.3899/jrheum.141261
pubmed: 25877504
Sawhney S, Woo P, Murray KJ (2001) Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arch Dis Child 85(5):421–426. https://doi.org/10.1136/adc.85.5.421
doi: 10.1136/adc.85.5.421
pubmed: 11668110
pmcid: 1718981
Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D, Coppo P, Hejblum G (2014) Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol 66(9):2613–2620. https://doi.org/10.1002/art.38690
doi: 10.1002/art.38690
pubmed: 24782338
Batu ED, Erden A, Seyhoglu E, Kilic L, Buyukasik Y, Karadag O, Bilginer Y, Bilgen SA, Akdogan A, Kiraz S, Ertenli AI, Ozen S, Kalyoncu U (2017) Assessment of the HScore for reactive haemophagocytic syndrome in patients with rheumatic diseases. Scand J Rheumatol 46(1):44–48. https://doi.org/10.3109/03009742.2016.1167951
doi: 10.3109/03009742.2016.1167951
pubmed: 27359073
Minoia F, Bovis F, Davi S, Horne A, Fischbach M, Frosch M, Huber A, Jelusic M, Sawhney S, McCurdy DK, Silva CA, Rigante D, Unsal E, Ruperto N, Martini A, Cron RQ, Ravelli A, Pediatric Rheumatology International Trials Organization tCA, Rheumatology Research Alliance tPRCSG, the Histiocyte S (2019) Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Ann Rheum Dis 78(10):1357–1362. https://doi.org/10.1136/annrheumdis-2019-215211
doi: 10.1136/annrheumdis-2019-215211
pubmed: 31296501
Tibaldi J, Pistorio A, Aldera E, Puzone L, El Miedany Y, Pal P, Giri PP, De H, Khubchandani R, Chavan PP, Vilaiyuk S, Lerkvaleekul B, Yamsuwan J, Sabui TK, Datta P, Pardeo M, Bracaglia C, Sawhney S, Mittal S, Hassan WA, Elderiny GF, Abu-Zaid MH, Eissa M, Sztajnbok F, das NevesSztajnbok FC, Russo R, Katsicas MM, Cimaz R, Marrani E, Alexeeva E, Dvoryakovskaya TM, Alsuweiti MO, Alzyoud RM, Kostik M, Chikova I, Minoia F, Filocamo G, Farag Y, Lotfy H, Nasef SI, Al-Mayouf SM, Maggio MC, Magalhaes CS, Gallizzi R, Conti G, Shimizu M, Civino A, Felici E, Giancane G, Ruperto N, Consolaro A, Ravelli A (2020) Development and initial validation of a composite disease activity score for systemic juvenile idiopathic arthritis. Rheumatology 59(11):3505–3514. https://doi.org/10.1093/rheumatology/keaa240
doi: 10.1093/rheumatology/keaa240
pubmed: 32829413
Kelly A, Ramanan AV (2007) Recognition and management of macrophage activation syndrome in juvenile arthritis. CurrOpinRheumatol 19(5):477–481. https://doi.org/10.1097/BOR.0b013e32825a6a79
doi: 10.1097/BOR.0b013e32825a6a79
Ravelli A, Minoia F, Davi S, Horne A, Bovis F, Pistorio A, Arico M, Avcin T, Behrens EM, De Benedetti F, Filipovic A, Grom AA, Henter JI, Ilowite NT, Jordan MB, Khubchandani R, Kitoh T, Lehmberg K, Lovell DJ, Miettunen P, Nichols KE, Ozen S, PachlopnikSchmid J, Ramanan AV, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner HI, Martini A, Ruperto N, Cron RQ (2016) Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. RMD Open 2(1):e000161. https://doi.org/10.1136/rmdopen-2015-000161
doi: 10.1136/rmdopen-2015-000161
pubmed: 26848401
pmcid: 4731834
Wang R, Li T, Ye S, Tan W, Zhao C, Li Y, Bao C, Fu Q (2019) Application of MS score in macrophage activation syndrome patients associated with adult onset Still’s disease. Ann Rheum Dis. https://doi.org/10.1136/annrheumdis-2019-216286
doi: 10.1136/annrheumdis-2019-216286
pubmed: 31848144
pmcid: 7167108
Zhang L, Yang X, Li TF, Liu X, Liu S (2020) Comparison of MS score and HScore for the diagnosis of adult-onset Still’s disease-associated macrophage activation syndrome. Ann Rheum Dis. https://doi.org/10.1136/annrheumdis-2020-217917
doi: 10.1136/annrheumdis-2020-217917
pubmed: 33272962
pmcid: 7705424
Poddighe D, Cavagna L, Brazzelli V, Bruni P, Marseglia GL (2014) A hyper-ferritinemia syndrome evolving in recurrent macrophage activation syndrome, as an onset of amyopathic juvenile dermatomyositis: a challenging clinical case in light of the current diagnostic criteria. Autoimmun Rev 13(11):1142–1148. https://doi.org/10.1016/j.autrev.2014.05.009
doi: 10.1016/j.autrev.2014.05.009
pubmed: 24879966