Browsing by Author "Hinojosa-Azaola, A"

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    Musculoskeletal manifestations in children with Behcet's syndrome: data from the AIDA Network Behcet's Syndrome Registry
    Gaggiano, C; Maselli, A; Sfikakis, PP; Laskari, K; Ragab, G; Hegazy, MT; Laymouna, AH; Lopalco, G; Almaghlouth, IA; Asfina, KN; Alahmed, O; Mayrink, HAG; Antonelli, IPD; Cattalini, M; Piga, M; Sota, J; Gentileschi, S; Maggio, MC; Opris-Belinski, D; Hatemi, G; Insalaco, A; Olivieri, AN; Tufan, A; Karadeniz, H; Kardas, RC; La Torre, F; Cardinale, F; Marino, A; Guerriero, S; Ruscitti, P; Tarsia, M; Vitale, A; Caggiano, V; Telesca, S; Iannone, F; Parretti, V; Frassi, M; Aragona, E; Ciccia, F; Wiesik-Szewczyk, E; Ionescu, R; Sahin, A; Akkoç, N; Hinojosa-Azaola, A; Tharwat, S; Hernández-Rodríguez, J; Espinosa, G; Conti, G; Del Giudice, E; Govoni, M; Emmi, G; Fabiani, C; Balistreri, A; Frediani, B; Rigante, D; Cantarini, L
    This study aims to describe musculoskeletal manifestations (MSM) in children with Behcet's syndrome (BS), their association with other disease manifestations, response to therapy, and long-term prognosis. Data were retrieved from the AIDA Network Behcet's Syndrome Registry. Out of a total of 141 patients with juvenile BS, 37 had MSM at disease onset (26.2%). The median age at onset was 10.0 years (IQR 7.7). The median follow-up duration was 21.8 years (IQR 23.3). Recurrent oral (100%) and genital ulcers (67.6%) and pseudofolliculitis (56.8%) were the most common symptoms associated with MSM. At disease onset, 31 subjects had arthritis (83.8%), 33 arthralgia (89.2%), and 14 myalgia (37.8%). Arthritis was monoarticular in 9/31 cases (29%), oligoarticular in 10 (32.3%), polyarticular in 5 (16.1%), axial in 7 (22.6%). Over time, arthritis became chronic-recurrent in 67.7% of cases and 7/31 patients had joint erosions (22.6%). The median Behcet's Syndrome Overall Damage Index was 0 (range 0-4). Colchicine was inefficacious for MSM in 4/14 cases (28.6%), independently from the type of MSM (p = 0.46) or the concomitant therapy (p = 0.30 for cDMARDs, p = 1.00 for glucocorticoids); cDMARDs and bDMARDs were inefficacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefficacy (p = 0.014). To conclude, MSM in children with BS are frequently associated with recurrent ulcers and pseudofolliculitis. Arthritis is mostly mono- or oligoarticular, but sacroiliitis is not unusual. Prognosis of this subset of BS is overall favorable, though the presence of myalgia negatively affects response to biologic therapies. ClinicalTrials.gov Identifier: NCT05200715 (registered on December 18, 2021).
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    The clinical assessment of lung involvement in patients with Still's disease, results from the multicentre international AIDA Network Still's Disease Registry
    Ruscitti, P; Vitale, A; Di Cola, I; Caggiano, V; Palumbo, P; Di Cesare, E; Hinojosa-Azaola, A; Torres-Ruiz, J; Guaracha-Basañez, GA; Martín-Nares, E; Lopalco, G; Morrone, M; Iannone, F; Giardini, HAM; Cordeiro, RA; Antonelli, IPD; Berardicurti, O; Navarini, L; Ciccia, F; Visconti, MC; Iacono, D; Direskeneli, H; Erten, S; Yao, HH; Thabet, M; Tharwat, S; Ragab, G; Gómez-Caverzaschi, V; Sfikakis, PP; Fotis, L; La Torre, F; Maier, A; Karamanakos, A; Almaghlouth, IA; Frassi, M; Tufan, A; Govoni, M; Sota, J; Simonini, G; Emmi, G; Li Gobbi, F; Parronchi, P; Costi, S; Sarzi-Puttini, P; Opris-Belinski, D; Sfriso, P; Tarsia, M; Maggio, MC; Monti, S; Gündüz, OS; Rigante, D; Bartoloni, E; Verrecchia, E; Iagnocco, A; Viapiana, O; Bargagli, E; Batu, ED; Sebastiani, GD; Del Giudice, E; Conti, G; Breda, L; Gidaro, A; Gicchino, MF; Gaggiano, C; Brucato, AL; Triggianese, P; Makowska, J; Carubbi, F; Farina, N; Guggino, G; De Paulis, A; Mazzei, MA; Di Meglio, N; Lo Gullo, A; Conforti, A; Ogunjimi, B; Calabrese, L; Rubegni, P; Giardina, A; Wiesik-Szewczyk, E; Balistreri, A; Fabiani, C; Frediani, B; Dagna, L; Giacomelli, R; Cantarini, L
    Objectives To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.Methods A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry. Still's disease-lung involvement was defined by the presence of pleuritis, parenchymal features, acute respiratory distress syndrome (ARDS) and/or pulmonary arterial hypertension.Results In total, 90 patients with Still's disease and lung involvement were assessed (mean age 36.3 +/- 17.8 years, 35.6% male sex). Among them, 13.3% of patients were paediatrics. These patients with lung involvement mainly showed pleuritis in 72.2% of cases, parenchymal features in 34.4%, ARDS in 9.5% and pulmonary arterial hypertension in 2.3%. After that we focused on patients characterised by parenchymal lung involvement, which is an emergent issue of clinical concern. These patients with parenchymal lung disease were significantly characterized by sore throat, pericarditis and higher values of systemic score than others. Finally, the administration of both IL-1 or IL-6 inhibitors was not associated with the presence of parenchymal lung involvement.Conclusion The clinical characteristics of patients with Still's disease and lung involvement were described in the AIDA network. We also provided a clinical profile of patients with parenchymal lung involvement considering its prognostic relevance. Although providing a clinical landscape of these patients, further studies are needed to fully clarify this issue.
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    Impact of HLA-B51 on Uveitis and Retinal Vasculitis: Data from the AIDA International Network Registries on Ocular Inflammatory Disorders
    Sota, J; Guerriero, S; Lopalco, G; Tufan, A; Ragab, G; Almaglouth, I; Govoni, M; Sfikakis, PP; Frassi, M; Vitale, A; Kardas, RC; Triggianese, P; Chimenti, MS; Aboabat, AA; Piga, M; Monti, S; Sebastiani, GD; Yildirim, D; Conforti, A; Gentileschi, S; Dammacco, R; Hinojosa-Azaola, A; Kawakami-Campos, PA; Ruffilli, F; Torres-Ruiz, J; Thabet, M; Atig, A; Ruscitti, P; Cataldi, G; Viapiana, O; Hatemi, G; Karakoç, A; Costi, S; Iagnocco, A; Crisafulli, F; Fragoulis, G; Del Giudice, E; Hegazy, MT; Paroli, MP; Sahin, A; Morrone, M; Iannone, F; Opris-Belinski, D; Asfina, KN; Barone, P; Gaggiano, C; Kucuk, H; Gicchino, MF; Carubbi, F; Caggiano, V; Laskari, K; Tharwat, S; Direskeneli, H; Alibaz-Oner, F; Sevik, G; Maier, A; Laymouna, AH; Emmi, G; Akkoç, N; Tarsia, M; Sbalchiero, J; Conti, G; Spinella, R; La Torre, F; Tombetti, E; Amin, RH; Mauro, A; Karamanakos, A; Carreño, E; Fonollosa, A; Cattalini, M; Breda, L; de-la-Torre, A; Wiesik-Szewczyk, E; Cifuentes-González, C; Ozen, S; Mazzei, MA; Tosi, GM; Frediani, B; Balistreri, A; Batu, ED; Gupta, V; Cantarini, L; Fabiani, C
    PurposeThe clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Beh & ccedil;et's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown.MethodsData were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time.ResultsRecords of 213 patients (341 eyes) were analyzed. No differences in complications were observed (p = 0.465). With regard to VA, a significant difference was detected in median BCVA (p = 0.046), which was not maintained after Bonferroni correction (p = 0.060). RV was significantly more prevalent in NIU-affected patients who tested positive for HLA-B51, irrespective of the systemic diagnosis of BD (p = 0.025). No differences emerged in the occurrence of macular edema (p = 0.99).ConclusionsPatients with NIU testing positive for HLA-B51 exhibit an increased likelihood of RV throughout disease course, irrespective of a systemic diagnosis of BD. The rate of complications as well as VA are comparable between NIU cases unrelated to BD testing positive for HLA-B51 and uveitis associated with BD. Therefore, it is advisable to perform the HLA-B typing in patients with NIU or retinal vasculitis, even in the absence of typical BD features.
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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
    Ruscitti, P; Masedu, F; Vitale, A; Caggiano, V; Di Cola, I; Cipriani, P; Valenti, M; Giardini, HAM; Antonelli, IPDB; Dagostin, MA; Lopalco, G; Iannone, F; Maria, M; Almaghlouth, IA; Asfina, KN; Ali, HH; Ciccia, F; Iacono, D; Pantano, I; Mauro, D; Sfikakis, PP; Tektonidou, M; Laskari, K; Berardicurti, O; Dagna, L; Tomelleri, A; Tufan, A; Kardas, RC; Hinojosa-Azaola, A; Martín-Nares, E; Kawakami-Campos, PA; Ragab, G; Hegazy, MT; Direskeneli, H; Alibaz-Oner, F; Fotis, L; Sfriso, P; Govoni, M; La Torre, F; Maggio, MC; Montecucco, C; De Stefano, L; Bugatti, S; Rossi, S; Makowska, J; Del Giudice, E; Emmi, G; Bartoloni, E; Hernández-Rodríguez, J; Conti, G; Olivieri, AN; 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, GD; Gidaro, A; Brucato, AL; 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.
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    A patient-driven registry on Behcet's disease: the AIDA for patients pilot project
    Gaggiano, C; Del Bianco, A; Sota, J; Gentileschi, S; Ruscitti, P; Giacomelli, R; Piga, M; Crisafulli, F; Monti, S; Emmi, G; De Paulis, A; Vitale, A; Tarsia, M; Caggiano, V; Nuzzolese, R; Parretti, V; Fabiani, C; Lopalco, G; Maier, A; Cattalini, M; Rigante, D; Govoni, M; Li Gobbi, F; Guiducci, S; Parronchi, P; Marino, A; Ciccia, F; Maggio, MC; Aragona, E; Bartoloni, E; Iagnocco, A; Viapiana, O; Sebastiani, GD; Guerriero, S; Insalaco, A; Del Giudice, E; Conti, G; Barone, P; Olivieri, AN; Brucato, A; Carubbi, F; Triggianese, P; Mauro, A; Tosi, GM; Fonollosa, A; Giardini, HAM; Ragab, G; Tharwat, S; Hernández-Rodríguez, J; Sfikakis, PP; Laskari, K; Karamanakos, A; Espinosa, G; Shahram, F; Direskeneli, H; Hinojosa-Azaola, A; Opris-Belinski, D; AlMaghlouth, IA; Hatemi, G; Eksin, MA; Önen, F; Wiesik-Szewczyk, E; Akkoç, N; Tufan, A; Sahin, A; Erten, S; Ozen, S; Batu, ED; Frediani, B; Balistreri, A; Cantarini, L
    IntroductionThis paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behcet's disease (BD). MethodsThe project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behcet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry. ResultsRespondents were reached via SIMBA communication channels in 167 cases (83.5%) and the AIDA Network affiliated clinical centers in 33 cases (16.5%). The median value of the Behcet's Disease Quality of Life (BDQoL) score was 14 (IQR 11, range 0-30), indicating a medium quality of life, and the median Global Fatigue Index (GFI) was 38.7 (IQR 10.9, range 1-50), expressing a significant level of fatigue. The mean Beliefs about Medicines Questionnaire (BMQ) necessity-concern differential was 0.9 & PLUSMN; 1.1 (range - 1.8-4), showing that the registry participants prioritized necessity belief over concerns to a limited extent. As for the socioeconomic impact of BD, in 104 out of 187 cases (55.6%), patients had to pay from their own pocket for medical exams required to reach the diagnosis. The low family socioeconomic status (p < 0.001), the presence of any major organ involvement (p < 0.031), the presence of gastro-intestinal (p < 0.001), neurological (p = 0.012) and musculoskeletal (p = 0.022) symptoms, recurrent fever (p = 0.002), and headache (p < 0.001) were associated to a higher number of accesses to the healthcare system. Multiple linear regression showed that the BDQoL score could significantly predict the global socioeconomic impact of BD (F = 14.519, OR 1.162 [CI 0.557-1.766], p < 0.001). DiscussionPreliminary results from the AIDA for Patients BD registry were consistent with data available in the literature, confirming that PROs and PREs could be easily provided by the patient remotely to integrate physician-driven registries with complementary and reliable information.
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    Influence of gender on Behcet's disease phenotype and irreversible organ damage: Data from the International AIDA Network Behcet's Disease Registry
    Sota, J; Ragab, G; AlMaglouth, I; Lopalco, G; Tufan, A; Direskeneli, H; Hinojosa-Azaola, A; Giardini, HAM; Guerriero, S; Triggianese, P; Sfikakis, PP; Piga, M; Ruscitti, P; Govoni, M; Iagnocco, A; Carubbi, F; Hernández-Rodríguez, J; Laymouna, AH; Mahmoud, AAMA; Ghanema, M; Aboabat, AA; Asfina, KN; Alanazi, F; Morrone, M; Spedicato, V; Kucuk, H; Kardas, R; Öner, FA; Sevik, G; Torres-Ruiz, J; Kawakami-Campos, PA; Antonelli, IPDB; Dammacco, R; Chimenti, MS; 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; Montis, S; Hatemi, G; Karakoc, A; Frassi, M; Giacomelli, R; Tharwat, S; Thabet, M; Ciccia, F; Emmi, G; Viapiana, O; Sahin, A; Sebastiani, GD; Batu, ED; Ozen, S; Sener, S; Opris-Belinski, D; Costi, S; Conforti, A; Cattalini, M; Bartoloni, E; Akkoç, N; Gunduz, OS; Conti, G; Maier, A; Giardina, A; Li Gobbi, F; Parronchi, P; Puttini, PS; Breda, L; De Paulis, A; Carreño, E; La Torre, F; Wiesik-Scewczyk, E; de-la Torre, A; Mejía-Salgado, G; Shahram, F; Guiducci, S; Maggio, MC; Aragona, E; Rigante, D; Ciavarro, A; Önen, F; Erten, U; Insalaco, A; Del Giudice, E; Barone, P; Gicchino, F; Brucato, A; Lo Gullo, A; Mauro, A; Karamanakos, A; Balistreri, A; Mazzei, MA; Frediani, B; Fabiani, C; Cantarini, L
    Objectives. - Gender impact on phenotypical expression of Behcet'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 Behcet's syndrome Overall Damage Index (BODI), differences in the disease manifestations at onset and in the cumulative manifestations throughout diseasecourse, 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. (c) 2024 Les Auteurs. Publie par Elsevier Masson SAS au nom de Socioto Franoaise de Rhumatologie. Cet article est publie en Open Access sous licence CC BY (http://creativecommons.org/licenses/by/4.0/).