Evaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry
Authors: Ruscitti Piero , Di Cola Ilenia , Vitale Antonio , Caggiano Valeria , Palumbo Pierpaolo , Di Cesare Ernesto , Torres-Ruiz Jiram , Guaracha-Basañez Guillermo Arturo , Martín-Nares Eduardo , Ciccia Francesco , Iacono Daniela , Riccio Flavia , Maggio Maria Cristina , Tharwat Samar , Hashad Soad , Rigante Donato , Ortolan Augusta , Mayrink Giardini Henrique A. , de Brito Antonelli Isabele Parente , Cordeiro Rafael Alves , Giacomelli Roberto , Navarini Luca , Berardicurti Onorina , Conforti Alessandro , Opris-Belinski Daniela , Sota Jurgen , Gaggiano Carla , Lopalco Giuseppe , Iannone Fiorenzo , La Torre Francesco , Mastrorilli Violetta , Govoni Marcello , Ruffilli Francesca , Emmi Giacomo , Biancalana Edoardo , Sfikakis Petros P. , Tektonidou Maria , Hernández-Rodríguez José , Gómez-Caverzaschi Verónica , Gündüz Özgül Soysal , Conti Giovanni , Patroniti Serena , Gidaro Antonio , Bartoli Arianna , Olivieri Alma Nunzia , Gicchino M. Francesca , Brucato Antonio Luca , Dagna Lorenzo , Tomelleri Alessandro , Campochiaro Corrado , De Paulis Amato , Mormile Ilaria , Della Casa Francesca , Direskeneli Haner , Alibaz-Oner Fatma , Karamanakos Anastasios , Dimouli Aikaterini , Ragab Gaafar , Mahmoud Ayman Abdelmonem Ahmed , Tufan Abdurrahman , Kucuk Hamit , Kardas Riza , Batu Ezgi D. , Ozen Seza , Wiesik-Szewczyk Ewa , Hinojosa-Azaola Andrea , Balistreri Alberto , Fabiani Claudia , Frediani Bruno , Cantarini Luca 
Published: January 2025

Objective

We aimed to (1) evaluate the cardiac involvement, with a focus on myocarditis, in patients with Still disease included in the multicenter Autoinflammatory Disease Alliance (AIDA) Network Still disease registry; and (2) assess the predictive factors for myocarditis by deriving a clinical risk patient profile for this severe manifestation.

Methods

A multicenter observational study was established, in which consecutive patients with Still disease in the AIDA Network Still disease registry were characterized by cardiac involvement. Cardiac involvement was defined according to the presence of pericarditis, tamponade, myocarditis, and/or aseptic endocarditis.

Results

In total, 73 patients with Still disease and cardiac involvement were assessed (mean age 36.3 [SD 19.9] years; male sex, 42.5%), out of which 21.9% were children. The most common cardiac manifestation was pericarditis, occurring in 90.4% of patients; patients also presented with myocarditis (26%), and less frequently endocarditis (2.7%) and tamponade (1.4%). In comparing clinical features of patients with myocarditis to those without, significantly increased frequencies of skin rash and pleuritis, as well as higher systemic scores, were seen. Further, a higher mortality rate was shown in patients with myocarditis. In regression models, skin rash and the systemic score independently predicted the myocarditis.

Conclusion

The characteristics of patients with Still disease and cardiac involvement were assessed in the AIDA Network. The most common feature was the pericarditis, but a more severe clinical picture was also reported in patients with myocarditis. The latter was associated with increased mortality rate and higher systemic score, identifying patients who should be carefully managed.