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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search.filters.applied.f.dateIssued: 2023 × Revista / Livro: Frontiers in Medicine ×

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  • article 0 Citação(ões) na Scopus
    A patient-driven registry on Behcet's disease: the AIDA for patients pilot project
    (2023) GAGGIANO, Carla; BIANCO, Alessandra Del; SOTA, Jurgen; GENTILESCHI, Stefano; RUSCITTI, Piero; GIACOMELLI, Roberto; PIGA, Matteo; CRISAFULLI, Francesca; MONTI, Sara; EMMI, Giacomo; PAULIS, Amato De; VITALE, Antonio; TARSIA, Maria; CAGGIANO, Valeria; NUZZOLESE, Rossana; PARRETTI, Veronica; FABIANI, Claudia; LOPALCO, Giuseppe; MAIER, Armin; CATTALINI, Marco; RIGANTE, Donato; GOVONI, Marcello; GOBBI, Francesca Li; GUIDUCCI, Serena; PARRONCHI, Paola; MARINO, Achille; CICCIA, Francesco; MAGGIO, Maria Cristina; ARAGONA, Emma; BARTOLONI, Elena; IAGNOCCO, Annamaria; VIAPIANA, Ombretta; SEBASTIANI, Gian Domenico; GUERRIERO, Silvana; INSALACO, Antonella; GIUDICE, Emanuela Del; CONTI, Giovanni; BARONE, Patrizia; OLIVIERI, Alma Nunzia; BRUCATO, Antonio; CARUBBI, Francesco; TRIGGIANESE, Paola; MAURO, Angela; TOSI, Gian Marco; FONOLLOSA, Alex; GIARDINI, Henrique Ayres Mayrink; RAGAB, Gaafar; THARWAT, Samar; HERNANDEZ-RODRIGUEZ, Jose; SFIKAKIS, Petros P.; LASKARI, Katerina; KARAMANAKOS, Anastasios; ESPINOSA, Gerard; SHAHRAM, Farhad; DIRESKENELI, Haner; HINOJOSA-AZAOLA, Andrea; OPRIS-BELINSKI, Daniela; ALMAGHLOUTH, Ibrahim A.; HATEMI, Gulen; EKSIN, Mehmet Akif; ONEN, Fatos; WIESIK-SZEWCZYK, Ewa; AKKOC, Nurullah; TUFAN, Abdurrahman; SAHIN, Ali; ERTEN, Sukran; OZEN, Seza; BATU, Ezgi Deniz; FREDIANI, Bruno; BALISTRERI, Alberto; CANTARINI, Luca
    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.
  • article 1 Citação(ões) na Scopus
    Preliminary data revealing efficacy of Streptococcus salivarius K12 (SSK12) in Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome: A multicenter study from the AIDA Network PFAPA syndrome registry
    (2023) TORRE, Francesco La; SOTA, Jurgen; INSALACO, Antonella; CONTI, Giovanni; GIUDICE, Emanuela Del; LUBRANO, Riccardo; BREDA, Luciana; MAGGIO, Maria Cristina; CIVINO, Adele; MASTRORILLI, Violetta; LOCONTE, Roberta; NATALE, Marco Francesco; CELANI, Camilla; ROMEO, Mery; PATRONITI, Serena; GENTILE, Cristina; VITALE, Antonio; CAGGIANO, Valeria; GAGGIANO, Carla; DIOMEDA, Federico; CATTALINI, Marco; LOPALCO, Giuseppe; EMMI, Giacomo; PARRONCHI, Paola; GENTILESCHI, Stefano; CARDINALE, Fabio; ARAGONA, Emma; SHAHRAM, Farhad; MARINO, Achille; BARONE, Patrizia; MOSCHEO, Carla; OZKIZILTAS, Burcugul; CARUBBI, Francesco; ALAHMED, Ohoud; IEZZI, Ludovica; OGUNJIMI, Benson; MAURO, Angela; TARSIA, Maria; MAHMOUD, Ayman Abdel-Monem Ahmed; GIARDINI, Henrique Ayres Mayrink; SFIKAKIS, Petros P.; LASKARI, Katerina; WIESIK-SZEWCZYK, Ewa; HERNANDEZ-RODRIGUEZ, Jose; FREDIANI, Bruno; GOMEZ-CAVERZASCHI, Veronica; TUFAN, Abdurrahman; ALMAGHLOUTH, Ibrahim A.; BALISTRERI, Alberto; RAGAB, Gaafar; FABIANI, Claudia; CANTARINI, Luca; RIGANTE, Donato; AIDA Network
    ObjectiveTo evaluate the potential role of Streptococcus salivarius K12 (SSK12) in controlling febrile flares in patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Further aims were to assess the impact of SSK12 on (i) flare duration, (ii) variation in the degree of the highest body temperature during flares, (iii) steroid-sparing effect, and (iv) change of PFAPA accompanying symptoms before and after SSK12 introduction. Patients and methodsThe medical charts from 85 pediatric patients with PFAPA syndrome (49 males and 36 females) enrolled in the AIDA registry and treated with SSK12 for a median period of 6.00 +/- 7.00 months in the period between September 2017 and May 2022 were examined. Children recruited had a median time of disease duration of 19.00 +/- 28.00 months. ResultsThe number of febrile flares significantly decreased comparing the 12 months before [median (IQR), 13.00 (6.00)] and after SSK12 initiation [median (IQR), 5.50 (8.00), p < 0.001]. The duration of fever was significantly reduced from 4.00 (2.00) days to 2.00 (2.00) days [p < 0.001]. Similarly, the highest temperature in degrees C was found significantly lower in the last follow-up assessment [median (IQR), 39.00 (1.00)] compared to the period prior to SSK12 start [median (IQR), 40.00 (1.00), p < 0.001]. Steroid load (mg/year) of betamethasone (or any equivalent steroid) significantly decreased between 12 months before treatment with SSK12 [median (IQR), 5.00 (8.00) mg/year] and the last follow-up visit [median (IQR), 2.00 (4.00) mg/year, p < 0.001]. The number of patients experiencing symptoms including pharyngitis/tonsillitis (p < 0.001), oral aphthae (p < 0.001) and cervical lymphadenopathy (p < 0.001) significantly decreased following SSK12. ConclusionSSK12 prophylaxis given for at least 6.00 months was found to reduce febrile flares of PFAPA syndrome: in particular, it halved the total number per year of fever flares, shortened the duration of the single febrile episode, lowered body temperature by 1 degrees C in the febrile flare, provided a steroid-sparing effect, and significantly reduced the accompanying symptoms related to the syndrome.
  • article 0 Citação(ões) na Scopus
    Axial spondyloarthritis in patients with recurrent fever attacks: data from the AIDA network registry for undifferentiated autoInflammatory diseases (USAIDs)
    (2023) VITALE, Antonio; CAGGIANO, Valeria; SILVA, Isabel; OLIVEIRA, Daniel G.; RUSCITTI, Piero; CICCIA, Francesco; VASI, Ibrahim; TUFAN, Abdurrahman; LOPALCO, Giuseppe; ALMAGHLOUTH, Ibrahim A.; SOTA, Jurgen; WIESIK-SZEWCZYK, Ewa; GAGGIANO, Carla; GIARDINI, Henrique Ayres Mayrink; SPEDICATO, Veronica; RAGAB, Gaafar; IANNONE, Florenzo; BALISTRERI, Alberto; FRASSI, Micol; HERNANDEZ-RODRIGUEZ, Jose; FABIANI, Claudia; FALSETTI, Paolo; MEGLIO, Nunzia Di; FREDIANI, Bruno; MAZZEI, Maria Antonietta; RIGANTE, Donato; FARIA, Raquel; CANTARINI, Luca
    BeckgroundDespite the recent advances in the field of autoinflammatory diseases, most patients with recurrent fever episodes do not have any defined diagnosis. The present study aims at describing a cohort of patients suffering from apparently unexplained recurrent fever, in whom non-radiographic axial spondylarthritis (SpA) represented the unique diagnosis identified after a complete clinical and radiologic assessment. Materials and methodsPatients' data were obtained from the international registry on Undifferentiated Systemic AutoInflammatory Diseases (USAIDs) developed by the AutoInflammatory Disease Alliance (AIDA) network. ResultsA total of 54 patients with recurrent fever episodes were also affected by non-radiographic axial SpA according to the international classification criteria. SpA was diagnosed after the start of fever episodes in all cases; the mean age at the diagnosis of axial SpA was 39.9 +/- 14.8 years with a diagnostic delay of 9.3 years. The highest body temperature reached during flares was 42 degrees C, with a mean temperature of 38.8 +/- 1.1 degrees C. The most frequent manifestations associated to fever were: arthralgia in 33 (61.1%) cases, myalgia in 24 (44.4%) cases, arthritis in 22 (40.7%) cases, headache in 15 (27.8%) cases, diarrhea in 14 (25.9%) cases, abdominal pain in 13 (24.1%) cases, and skin rash in 12 (22.1%) cases. Twenty-four (44.4%) patients have taken daily or on-demand non-steroidal anti-inflammatory drugs (NSAIDs) and 31 (57.4%) patients have been treated with daily or on demand oral glucocorticoids. Colchicine was used in 28 (51.8%) patients, while other conventional disease modifying anti-rheumatic drugs (cDMARDs) were employed in 28 (51.8%) patients. Forty (74.1%) patients underwent anti-tumor necrosis factor (TNF) agents and 11 (20.4%) were treated with interleukin (IL)-1 inhibitors. The response to TNF inhibitors on recurrent fever episodes appeared more effective than that observed with anti-IL-1 agents; colchicine and other cDMARDs were more useful when combined with biotechnological agents. ConclusionSigns and symptoms referring to axial SpA should be inquired in patients with apparently unexplained recurrent fever episodes. The specific treatment for axial SpA may lead to a remarkable improvement in the severity and/or frequency of fever episodes in patients with unexplained fevers and concomitant axial SpA.
  • article 1 Citação(ões) na Scopus
    Efficacy of canakinumab in patients with Still's disease across different lines of biologic therapy: real-life data from the International AIDA Network Registry for Still's Disease
    (2023) VITALE, Antonio; CAGGIANO, Valeria; SFIKAKIS, Petros P.; DAGNA, Lorenzo; LOPALCO, Giuseppe; RAGAB, Gaafar; TORRE, Francesco La; ALMAGHLOUTH, Ibrahim A.; MAGGIO, Maria Cristina; SOTA, Jurgen; TUFAN, Abdurrahman; HINOJOSA-AZAOLA, Andrea; IANNONE, Florenzo; LOCONTE, Roberta; LASKARI, Katerina; DIRESKENELI, Haner; RUSCITTI, Piero; MORRONE, Maria; GIARDINI, Henrique A. Mayrink; PANAGIOTOPOULOS, Alexandros; COLA, Ilenia Di; MARTIN-NARES, Eduardo; MONTI, Sara; STEFANO, Ludovico De; KARDAS, Riza Can; DURAN, Rahime; CAMPOCHIARO, Corrado; TOMELLERI, Alessandro; ALABDULKAREEM, Abdulaziz Mohammed; GAGGIANO, Carla; TARSIA, Maria; BARTOLONI, Elena; ROMEO, Mery; HUSSEIN, Mohamed A.; LAYMOUNA, Ahmed Hatem; ANTONELLI, Isabele Parente de Brito; DAGOSTIN, Marilia Ambiel; FOTIS, Lampros; BINDOLI, Sara; NAVARINI, Luca; ALIBAZ-ONER, Fatma; SEVIK, Gizem; FRASSI, Micol; CICCIA, Francesco; IACONO, Daniela; CRISAFULLI, Francesca; PORTINCASA, Piero; JABER, Nour; KAWAKAMI-CAMPOS, Perla Ayumi; WIESIK-SZEWCZYK, Ewa; IAGNOCCO, Annamaria; SIMONINI, Gabriele; SFRISO, Paolo; BALISTRERI, Alberto; GIACOMELLI, Roberto; CONTI, Giovanni; FREDIANI, Bruno; FABIANI, Claudia; CANTARINI, Luca
    Introduction: The effectiveness of canakinumab may change according to the different times it is used after Still's disease onset. This study aimed to investigate whether canakinumab (CAN) shows differences in short- and long-term therapeutic outcomes, according to its use as different lines of biologic treatment.Methods: Patients included in this study were retrospectively enrolled from the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Still's disease. Seventy-seven (51 females and 26 males) patients with Still's disease were included in the present study. In total, 39 (50.6%) patients underwent CAN as a first-line biologic agent, and the remaining 38 (49.4%) patients were treated with CAN as a second-line biologic agent or subsequent biologic agent.Results: No statistically significant differences were found between patients treated with CAN as a first-line biologic agent and those previously treated with other biologic agents in terms of the frequency of complete response (p =0.62), partial response (p =0.61), treatment failure (p >0.99), and frequency of patients discontinuing CAN due to lack or loss of efficacy (p =0.2). Of all the patients, 18 (23.4%) patients experienced disease relapse during canakinumab treatment, 9 patients were treated with canakinumab as a first-line biologic agent, and nine patients were treated with a second-line or subsequent biologic agent. No differences were found in the frequency of glucocorticoid use (p =0.34), daily glucocorticoid dosage (p =0.47), or concomitant methotrexate dosage (p =0.43) at the last assessment during CAN treatment.Conclusion: Canakinumab has proved to be effective in patients with Still's disease, regardless of its line of biologic treatment.