Clinical and laboratory features associated with macrophage activation syndrome in Still’s disease: data from the international AIDA Network Still’s Disease Registry
Authors: Triggianese Paola , Vitale Antonio , Lopalco Giuseppe , Mayrink Giardini Henrique Ayres , Ciccia Francesco , Al-Maghlouth Ibrahim , Ruscitti Piero , Sfikakis Petros Paul , Iannone Florenzo , de Brito Antonelli Isabele Parente , Patrone Martina , Asfina Kazi Nur , Di Cola Ilenia , Laskari Katerina , Gaggiano Carla , Tufan Abdurrahman , Sfriso Paolo , Dagna Lorenzo , Giacomelli Roberto , Hinojosa-Azaola Andrea , Ragab Gaafar , Fotis Lampros , Direskeneli Haner , Spedicato Veronica , Dagostin Marilia Ambiel , Iacono Daniela , Ali Hebatallah Hamed , Cipriani Paola , Sota Jurgen , Kardas Riza Can , Bindoli Sara , Campochiaro Corrado , Navarini Luca , Gentileschi Stefano , Martín-Nares Eduardo , Torres-Ruiz Jiram , Saad Moustafa Ali , Kourtesi Katerina , Alibaz-Oner Fatma , Sevik Gizem , Iagnocco Annamaria , Makowska Joanna , Govoni Marcello , Monti Sara , Maggio Maria Cristina , La Torre Francesco , Del Giudice Emanuela , Hernández-Rodríguez José , Bartoloni Elena , Emmi Giacomo , Chimenti Maria Sole , Maier Armin , Simonini Gabriele , Conti Giovanni , Olivieri Alma Nunzia , Tarsia Maria , De Paulis Amato , Lo Gullo Alberto , Więsik-Szewczyk Ewa , Viapiana Ombretta , Ogunjimi Benson , Tharwat Samar , Erten Sukran , Nuzzolese Rossana , Karamanakos Anastasios , Frassi Micol , Conforti Alessandro , Caggiano Valeria , Marino Achille , Sebastiani Gian Domenico , Gidaro Antonio , Tombetti Enrico , Carubbi Francesco , Rubegni Giovanni , Cartocci Alessandra , Balistreri Alberto , Fabiani Claudia , Frediani Bruno , Cantarini Luca 
Published: October 2023

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.