Browsing by Author "Mayrink Giardini H.A."

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    The Systemic Score May Identify Life-Threatening Evolution in Still Disease: Data from the GIRRCS AOSD-Study Group and the AIDA Network Still Disease Registry
    (John Wiley and Sons Inc, 2024) Ruscitti P.; Masedu F.; Vitale A.; Caggiano V.; Di Cola I.; Cipriani P.; Valenti M.; Mayrink Giardini H.A.; de Brito Antonelli I.P.; Dagostin M.A.; Lopalco G.; Iannone F.; Maria M.; Almaghlouth I.A.; Asfina K.N.; Ali H.H.; Ciccia F.; Iacono D.; Pantano I.; Mauro D.; Sfikakis P.P.; Tektonidou M.; Laskari K.; Berardicurti O.; Dagna L.; Tomelleri A.; Tufan A.; Can Kardas R.; Hinojosa-Azaola A.; Martín-Nares E.; Kawakami-Campos P.A.; Ragab G.; Hegazy M.T.; Direskeneli H.; Alibaz-Oner F.; Fotis L.; Sfriso P.; Govoni M.; La Torre F.; Cristina Maggio M.; Montecucco C.; De Stefano L.; Bugatti S.; Rossi S.; Makowska J.; Del Giudice E.; Emmi G.; Bartoloni E.; Hernández-Rodríguez J.; Conti G.; Nunzia Olivieri A.; Lo Gullo A.; Simonini G.; Viapiana O.; Wiesik-Szewczyk E.; Erten S.; Carubbi F.; De Paulis A.; Maier A.; Tharwat S.; Costi S.; Iagnocco A.; Sebastiani G.D.; Gidaro A.; Brucato A.L.; Karamanakos A.; Akkoç N.; Caso F.; Costa L.; Prete M.; Perosa F.; Atzeni F.; Guggino G.; Fabiani C.; Frediani B.; Giacomelli R.; Cantarini L.
    Objective: We aimed to evaluate the clinical usefulness of the systemic score in the prediction of life-threatening evolution in Still disease. We also aimed to assess the clinical relevance of each component of the systemic score in predicting life-threatening evolution and to derive patient subsets accordingly. Methods: A multicenter, observational, prospective study was designed including patients included in the Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale Adult-Onset Still Disease Study Group and the Autoinflammatory Disease Alliance Network Still Disease Registry. Patients were assessed to see if the variables to derive the systemic score were available. The life-threatening evolution was defined as mortality, whatever the clinical course, and/or macrophage activation syndrome, a secondary hemophagocytic lymphohistiocytosis associated with a poor prognosis. Results: A total of 597 patients with Still disease were assessed (mean ± SD age 36.6 ± 17.3 years; male 44.4%). The systemic score, assessed as a continuous variable, significantly predicted the life-threatening evolution (odds ratio [OR] 1.24; 95% confidence interval [CI] 1.07–1.42; P = 0.004). A systemic score ≥7 also significantly predicted the likelihood of a patient experiencing life-threatening evolution (OR 3.36; 95% CI 1.81–6.25; P < 0.001). Assessing the clinical relevance of each component of the systemic score, liver involvement (OR 1.68; 95% CI 1.48–2.67; P = 0.031) and lung disease (OR 2.12; 95% CI 1.14–4.49; P = 0.042) both significantly predicted life-threatening evolution. The clinical characteristics of patients with liver involvement and lung disease were derived, highlighting their relevance in multiorgan disease manifestations. Conclusion: The clinical utility of the systemic score was shown in identifying Still disease at a higher risk of life-threatening evolution in a large cohort. Furthermore, the clinical relevance of liver involvement and lung disease was highlighted. (Figure presented.). © 2024 American College of Rheumatology.
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    Influence of gender on Behçet's disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry
    (Elsevier Masson s.r.l., 2025) Sota J.; Ragab G.; AlMaglouth I.; Lopalco G.; Tufan A.; Direskeneli H.; Hinojosa-Azaola A.; Mayrink Giardini H.A.; Guerriero S.; Triggianese P.; Sfikakis P.P.; Piga M.; Ruscitti P.; Govoni M.; Iagnocco A.; Carubbi F.; Hernández-Rodríguez J.; Laymouna A.H.; Mahmoud A.A.-M.A.; Ghanema M.; Aboabat A.A.; Asfina K.N.; Alanazi F.; Morrone M.; Spedicato V.; Kucuk H.; Kardas R.; Alibaz Öner F.; Sevik G.; Torres-Ruiz J.; Kawakami-Campos P.A.; Parente de Brito Antonelli I.; Dammacco R.; Chimenti M.S.; Arida K.; Floris A.; Gentile M.; Ruffilli F.; Bellis E.; Alunno A.; Espinosa G.; Gentileschi S.; Gaggiano C.; Vitale A.; Caggiano V.; Lopez R.; Tarsia M.; Monti S.; Hatemi G.; Karakoç A.; Frassi M.; Giacomelli R.; Tharwat S.; Thabet M.; Ciccia F.; Emmi G.; Viapiana O.; Şahin A.; Sebastiani G.D.; Batu E.D.; Ozen S.; Sener S.; Opris-Belinski D.; Costi S.; Conforti A.; Cattalini M.; Bartoloni E.; Akkoç N.; Gunduz O.S.; Conti G.; Maier A.; Giardina A.; Li Gobbi F.; Parronchi P.; Sarzi Puttini P.; Breda L.; De Paulis A.; Carreño E.; La Torre F.; Więsik-Scewczyk E.; de-la Torre A.; Mejía-Salgado G.; Shahram F.; Guiducci S.; Maggio M.C.; Aragona E.; Rigante D.; Ciavarro A.; Önen F.; Erten; Insalaco A.; Del Giudice E.; Barone P.; Gicchino F.; Brucato A.; Lo Gullo A.; Mauro A.; Karamanakos A.; Balistreri A.; Mazzei M.A.; Frediani B.; Fabiani C.; Cantarini L.
    Objectives: Gender impact on phenotypical expression of Behçet's disease (BD) has been specifically investigated only in a few large-scale studies. The main goal of the study was to examine gender differences in a large cohort of patients affected by BD. Methods: Data were retrieved from the International AIDA Network Registry for BD. We assessed differences between males and females in terms of Behçet's syndrome Overall Damage Index (BODI), differences in the disease manifestations at onset and in the cumulative manifestations throughout disease course, as well as differences in the cardiovascular risk. Finally, predictive factors leading to major organ involvement were investigated. Results: In total, 1024 BD patients (567 males, 457 females) were enrolled in the study, with a male-to-female ratio of 1.24/1. Males displayed a significantly higher mean ± SD BODI (1.92 ± 2.09) at the last follow-up, compared to female patients (1.25 ± 1.87) (P < 0.0001). Uveitis (P < 0.0001) and vascular involvement (P = 0.0076) were significantly more frequent among males whereas female patients were significantly over-represented in arthralgia (P < 0.0001), arthritis (P = 0.00025), isolated headache (P < 0.0001), central nervous system (CNS) involvement (P = 0.040), and gastrointestinal involvement (P = 0.00046). Regarding cardiovascular risk, no differences between the two groups emerged (P = 0.617). Four variables were associated with the development of major organ involvement: male gender (OR = 2.104, P = 0.001), current treatment with biologic agents (OR = 2.257, P = 0.0003), origin from endemic countries (OR = 2.661, P = 0.0009), and disease duration (OR = 1.002, P = 0.024). Conclusion: BD displays a more severe course among males. This subgroup develops more irreversible damage and presents more frequently ocular and vascular involvement during disease course. On the other hand, female patients are prone to experience articular involvement, headache, CNS and gastrointestinal involvement. These data suggest the existence of a gender-driven disease expression. © 2024 The Author(s)
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    Evaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry
    (Journal of Rheumatology, 2025) Ruscitti P.; Di Cola I.; Vitale A.; Caggiano V.; Palumbo P.; Di Cesare E.; Torres-Ruiz J.; Guaracha-Basañez G.A.; Martín-Nares E.; Ciccia F.; Iacono D.; Riccio F.; Maggio M.C.; Tharwat S.; Hashad S.; Rigante D.; Ortolan A.; Mayrink Giardini H.A.; de Brito Antonelli I.P.; Cordeiro R.A.; Giacomelli R.; Navarini L.; Berardicurti O.; Conforti A.; Opris-Belinski D.; Sota J.; Gaggiano C.; Lopalco G.; Iannone F.; La Torre F.; Mastrorilli V.; Govoni M.; Ruffilli F.; Emmi G.; Biancalana E.; Sfikakis P.P.; Tektonidou M.; Hernández-Rodríguez J.; Gómez-Caverzaschi V.; Gündüz Ö.S.; Conti G.; Patroniti S.; Gidaro A.; Bartoli A.; Olivieri A.N.; Gicchino M.F.; Brucato A.L.; Dagna L.; Tomelleri A.; Campochiaro C.; De Paulis A.; Mormile I.; Casa F.D.; Direskeneli H.; Alibaz-Oner F.; Karamanakos A.; Dimouli A.; Ragab G.; Ahmed Mahmoud A.A.; Tufan A.; Kucuk H.; Kardas R.; Batu E.D.; Ozen S.; Wiesik-Szewczyk E.; Hinojosa-Azaola A.; Balistreri A.; Fabiani C.; Frediani B.; Cantarini L.
    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. © 2025 The Journal of Rheumatology.