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

Author

Triggianese P, Vitale A, Lopalco G, Mayrink Giardini HA, Ciccia F, Al-Maghlouth I, Ruscitti P, Sfikakis PP, Iannone F, de Brito Antonelli IP, Patrone M, Asfina KN, Di Cola I, Laskari K, Gaggiano C, Tufan A, Sfriso P, Dagna L, Giacomelli R, Hinojosa-Azaola A, Ragab G, Fotis L, Direskeneli H, Spedicato V, Dagostin MA, Iacono D, Ali HH, Cipriani P, Sota J, Kardas RC, Bindoli S, Campochiaro C, Navarini L, Gentileschi S, Martín-Nares E, Torres-Ruiz J, Saad MA, Kourtesi K, Alibaz-Oner F, Sevik G, Iagnocco A, Makowska J, Govoni M, Monti S, Maggio MC, La Torre F, Del Giudice E, Hernández-Rodríguez J, Bartoloni E, Emmi G, Chimenti MS, Maier A, Simonini G, Conti G, Olivieri AN, Tarsia M, De Paulis A, Lo Gullo A, Więsik-Szewczyk E, Viapiana O, Ogunjimi B, Tharwat S, Erten S, Nuzzolese R, Karamanakos A, Frassi M, Conforti A, Caggiano V, Marino A, Sebastiani GD, Gidaro A, Tombetti E, Carubbi F, Rubegni G, Cartocci A, Balistreri A, Fabiani C, Frediani B, and Cantarini L

Published
2024
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2. Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry.

Author

Triggianese P, Vitale A, Lopalco G, Mayrink Giardini HA, Ciccia F, Al-Maghlouth I, Ruscitti P, Sfikakis PP, Iannone F, de Brito Antonelli IP, Patrone M, Asfina KN, Di Cola I, Laskari K, Gaggiano C, Tufan A, Sfriso P, Dagna L, Giacomelli R, Hinojosa-Azaola A, Ragab G, Fotis L, Direskeneli H, Spedicato V, Dagostin MA, Iacono D, Ali HH, Cipriani P, Sota J, Kardas RC, Bindoli S, Campochiaro C, Navarini L, Gentileschi S, Martín-Nares E, Torres-Ruiz J, Saad MA, Kourtesi K, Alibaz-Oner F, Sevik G, Iagnocco A, Makowska J, Govoni M, Monti S, Maggio MC, La Torre F, Del Giudice E, Hernández-Rodríguez J, Bartoloni E, Emmi G, Chimenti MS, Maier A, Simonini G, Conti G, Olivieri AN, Tarsia M, De Paulis A, Lo Gullo A, Więsik-Szewczyk E, Viapiana O, Ogunjimi B, Tharwat S, Erten S, Nuzzolese R, Karamanakos A, Frassi M, Conforti A, Caggiano V, Marino A, Sebastiani GD, Gidaro A, Tombetti E, Carubbi F, Rubegni G, Cartocci A, Balistreri A, Fabiani C, Frediani B, and Cantarini L

Subjects
Humans, Hepatomegaly complications, Macrophage Activation Syndrome diagnosis, Macrophage Activation Syndrome complications, Still's Disease, Adult-Onset complications, Still's Disease, Adult-Onset diagnosis, Liver Diseases complications
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., (© 2023. The Author(s).)

Published
2023
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