HENRIQUE AYRES MAYRINK GIARDINI

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P ICHC, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/17 - Laboratório de Investigação em Reumatologia, Hospital das Clínicas, Faculdade de Medicina

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Autor: BALISTRERI, Alberto × search.filters.applied.f.dateIssued: 2023 × Revista / Livro: Internal and Emergency Medicine ×

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  • article 2 Citação(ões) na Scopus
    Musculoskeletal manifestations in children with Behcet's syndrome: data from the AIDA Network Behcet's Syndrome Registry
    (2023) GAGGIANO, Carla; MASELLI, Anna; SFIKAKIS, Petros P.; LASKARI, Katerina; RAGAB, Gaafar; HEGAZY, Mohamed Tharwat; LAYMOUNA, Ahmed Hatem; LOPALCO, Giuseppe; ALMAGHLOUTH, Ibrahim A.; ASFINA, Kazi Nur; ALAHMED, Ohoud; MAYRINK, Henrique Ayres Giardini; ANTONELLI, Isabele Parente de Brito; CATTALINI, Marco; PIGA, Matteo; SOTA, Jurgen; GENTILESCHI, Stefano; MAGGIO, Maria Cristina; OPRIS-BELINSKI, Daniela; HATEMI, Gulen; INSALACO, Antonella; OLIVIERI, Alma Nunzia; TUFAN, Abdurrahman; KARADENIZ, Hazan; KARDAS, Riza Can; TORRE, Francesco La; CARDINALE, Fabio; MARINO, Achille; GUERRIERO, Silvana; RUSCITTI, Piero; TARSIA, Maria; VITALE, Antonio; CAGGIANO, Valeria; TELESCA, Salvatore; IANNONE, Florenzo; PARRETTI, Veronica; FRASSI, Micol; ARAGONA, Emma; CICCIA, Francesco; WIESIK-SZEWCZYK, Ewa; IONESCU, Ruxandra; SAHIN, Ali; AKKOC, Nurullah; HINOJOSA-AZAOLA, Andrea; THARWAT, Samar; HERNANDEZ-RODRIGUEZ, Jose; ESPINOSA, Gerard; CONTI, Giovanni; GIUDICE, Emanuela Del; GOVONI, Marcello; EMMI, Giacomo; FABIANI, Claudia; BALISTRERI, Alberto; FREDIANI, Bruno; RIGANTE, Donato; CANTARINI, Luca
    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).
  • article 1 Citação(ões) na Scopus
    Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry
    (2023) TRIGGIANESE, Paola; VITALE, Antonio; LOPALCO, Giuseppe; GIARDINI, Henrique Ayres Mayrink; CICCIA, Francesco; AL-MAGHLOUTH, Ibrahim; RUSCITTI, Piero; SFIKAKIS, Petros Paul; IANNONE, Florenzo; ANTONELLI, Isabele Parente de Brito; PATRONE, Martina; ASFINA, Kazi Nur; COLA, Ilenia Di; 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; MARTIN-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; TORRE, Francesco La; GIUDICE, Emanuela Del; HERNANDEZ-RODRIGUEZ, Jose; BARTOLONI, Elena; EMMI, Giacomo; CHIMENTI, Maria Sole; MAIER, Armin; SIMONINI, Gabriele; CONTI, Giovanni; OLIVIERI, Alma Nunzia; TARSIA, Maria; PAULIS, Amato De; GULLO, Alberto Lo; WIESIK-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
    To 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.