• Paola Triggianese, Antonio Vitale, Giuseppe Lopalco, Henrique Ayres Mayrink Giardini, Francesco Ciccia, Ibrahim Al-Maghlouth, Piero Ruscitti, Petros Paul Sfikakis, Florenzo Iannone, de Brito AntonelliIsabele ParenteIPRheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil., Martina Patrone, Kazi Nur Asfina, Ilenia Di Cola, Katerina Laskari, Carla Gaggiano, Abdurrahman Tufan, Paolo Sfriso, Lorenzo Dagna, Roberto Giacomelli, Andrea Hinojosa-Azaola, Gaafar Ragab, Lampros Fotis, Haner Direskeneli, Veronica Spedicato, Marilia Ambiel Dagostin, Daniela Iacono, Hebatallah Hamed Ali, Paola Cipriani, Jurgen Sota, Riza Can Kardas, Sara Bindoli, Corrado Campochiaro, Luca Navarini, Stefano Gentileschi, Eduardo Martín-Nares, Jiram Torres-Ruiz, Moustafa Ali Saad, Katerina Kourtesi, Fatma Alibaz-Oner, Gizem Sevik, Annamaria Iagnocco, Joanna Makowska, Marcello Govoni, Sara Monti, Maria Cristina Maggio, Francesco La Torre, Emanuela Del Giudice, José Hernández-Rodríguez, Elena Bartoloni, Giacomo Emmi, Maria Sole Chimenti, Armin Maier, Gabriele Simonini, Giovanni Conti, Alma Nunzia Olivieri, Maria Tarsia, Amato De Paulis, Alberto Lo Gullo, Ewa Więsik-Szewczyk, Ombretta Viapiana, Benson Ogunjimi, Samar Tharwat, Sukran Erten, Rossana Nuzzolese, Anastasios Karamanakos, Micol Frassi, Alessandro Conforti, Valeria Caggiano, Achille Marino, Gian Domenico Sebastiani, Antonio Gidaro, Enrico Tombetti, Francesco Carubbi, Giovanni Rubegni, Alessandra Cartocci, Alberto Balistreri, Claudia Fabiani, Bruno Frediani, and Luca Cantarini.
• Rheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
• Intern Emerg Med. 2023 Nov 1; 18 (8): 223122432231-2243.

AbstractTo 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).

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