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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  • article 2 Citação(ões) na Scopus
    Development and implementation of the AIDA International Registry for patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis syndrome
    (2022) CASA, Francesca Della; VITALE, Antonio; CATTALINI, Marco; TORRE, Francesco La; CAPOZIO, Giovanna; GIUDICE, Emanuela Del; MAGGIO, Maria Cristina; CONTI, Giovanni; ALESSIO, Maria; OGUNJIMI, Benson; RAGAB, Gaafar; EMMI, Giacomo; ARAGONA, Emma; GIANI, Teresa; LOPALCO, Giuseppe; PARRONCHI, Paola; SHAHRAM, Farhad; VERRECCHIA, Elena; RICCI, Francesca; CARDINALE, Fabio; NOI, Silvia Di; NUZZOLESE, Rossana; LUBRANO, Riccardo; PATRONITI, Serena; NADDEI, Roberta; SABATO, Vito; HUSSEIN, Mohamed A.; DOTTA, Laura; MASTRORILLI, Violetta; GENTILESCHI, Stefano; TUFAN, Abdurrahman; CAGGIANO, Valeria; HEGAZY, Mohamed Tharwat; SOTA, Jurgen; ALMAGHLOUTH, Ibrahim A.; IBRAHIM, Amr; WIESIK-SZEWCZYK, Ewa; OZKIZILTAS, Burcugul; GROSSO, Salvatore; FRASSI, Micol; TARSIA, Maria; PEREIRA, Rosa Maria R.; TAYMOUR, Maged; GAGGIANO, Carla; COLELLA, Sergio; FABIANI, Claudia; MORRONE, Maria; RUSCITTI, Piero; FREDIANI, Bruno; SPEDICATO, Veronica; GIARDINI, Henrique A. Mayrink; BALISTRERI, Alberto; RIGANTE, Donato; CANTARINI, Luca
    Objective: Aim of this paper is to illustrate the methodology, design, and development of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to patients with the Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Methods: This is a physician-driven, non-population- and electronic-based registry proposed to gather real-world demographics, clinical, laboratory, instrumental and socioeconomic data from PFAPA patients. Data recruitment is realized through the on-line Research Electronic Data Capture (REDCap) tool. This registry is thought to collect standardized information for clinical research leading to solid real-life evidence. The international scope and the flexibility of the registry will facilitate the realization of cutting-edge study projects through the constant updating of variables and the possible merging and transfer of data between current and future PFAPA registries. Results: A total of 112 centers have already been involved from 23 countries and 4 continents starting from August 24th, 2021, to April 6th, 2022. In total 56/112 have already obtained the formal approval from their local Ethics Committees. The platform counts 321 users (113 principal investigators, 203 site investigators, two lead investigators, and three data managers). The registry collects retrospective and prospective data using 3,856 fields organized into 25 instruments, including PFAPA patient's demographics, medical histories, symptoms, triggers/risk factors, therapies, and impact on the healthcare systems. Conclusions: The development of the AIDA International Registry for PFAPA patients will enable the on-line collection of standardized data prompting real-life studies through the connection of worldwide groups of physicians and researchers. This project can be found on NCT 05200715.
  • article 0 Citação(ões) na Scopus
    Lyme disease and Whipple's disease: a comprehensive review for the rheumatologist
    (2024) GIARDINI, Henrique Ayres Mayrink; NEVES, Fabricio Souza; PEREIRA, Ivanio Alves; CORDEIRO, Rafael Alves
    Despite their rarity, Lyme disease and Whipple's disease are of significant importance in rheumatology, as both can manifest as chronic arthritis, presenting challenges in the differential diagnosis of inflammatory arthropathies. In Lyme disease, arthritis typically emerges as a late manifestation, usually occurring six months after the onset of erythema migrans. The predominant presentation involves mono- or oligoarthritis of large joints, with a chronic or remitting-recurrent course. Even with appropriate antimicrobial treatment, arthritis may persist due to inadequate immunological control triggered by the disease. In contrast, Whipple's disease may present with a migratory and intermittent seronegative poly- or oligoarthritis of large joints, preceding classic gastrointestinal symptoms by several years. Both disorders, particularly Whipple's disease, can be misdiagnosed as more common autoimmune rheumatic conditions such as rheumatoid arthritis and spondyloarthritis. Epidemiology is crucial in suspecting and diagnosing Lyme disease, as the condition is transmitted by ticks prevalent in specific areas of the United States, Europe, and Asia. On the contrary, the causative agent of Whipple's disease is widespread in the environment, yet invasive disease is rare and likely dependent on host genetic factors. In addition to erythema migrans in Lyme disease and gastrointestinal manifestations in Whipple's disease, neurological and cardiac involvement can further complicate the course of both. This article offers a comprehensive review of the epidemiological, pathophysiological, clinical, and therapeutic aspects of both diseases.
  • article 0 Citação(ões) na Scopus
    What should rheumatologists know about Gaucher disease and Fabry disease? Connecting the dots for an overview
    (2024) CORDEIRO, Rafael Alves; ROSA NETO, Nilton Salles; GIARDINI, Henrique Ayres Mayrink
    Gaucher and Fabry diseases are lysosomal storage disorders in which deficient enzyme activity leads to pathological accumulation of sphingolipids. These diseases have a broad phenotypic presentation. Musculoskeletal symptoms and pain complaints are frequently reported by patients. Thus, rheumatologists can be contacted by these patients, contributing to the correct diagnosis, earlier indication of appropriate treatment and improvement of their prognosis. This review describes important concepts about Gaucher and Fabry diseases that rheumatologists should understand to improve patients' quality of life and change the natural history of these diseases.
  • article 3 Citação(ões) na Scopus
    The administration of methotrexate in patients with Still's disease, ""real-life"" findings from AIDA Network Still Disease Registry
    (2023) RUSCITTI, Piero; SOTA, Jurgen; VITALE, Antonio; LOPALCO, Giuseppe; IANNONE, Florenzo; MORRONE, Maria; GIARDINI, Henrique Ayres Mayrink; D'AGOSTIN, Marilia Ambuel; ANTONELLI, Isabelle Parente de Brito; ALMAGHLOUTH, Ibrahim; ASFINA, Kazi Nur; KHALIL, Najma; SFIKAKIS, Petros P.; LASKARI, Katerina; TEKTONIDOU, Maria; CICCIA, Francesco; IACONO, Daniela; RICCIO, Flavia; RAGAB, Gaafar; HUSSEIN, Mohamed A.; GOVONI, Marcello; RUFFILLI, Francesca; DIRESKENELI, Haner; ALIBAZ-ONER, Fatma; GIACOMELLI, Roberto; NAVARINI, Luca; BARTOLONI, Elena; RICCUCCI, Ilenia; MARTIN-NARES, Eduardo; TORRES-RUIZ, Jiram; CIPRIANI, Paola; COLA, Ilenia Di; HERNANDEZ-RODRIGUEZ, Jose; GOMEZ-CAVERZASCHI, Veronica; DAGNA, Lorenzo; TOMELLERI, Alessandro; MAKOWSKA, Joanna; BRZEZINSKA, Olga; IAGNOCCO, Annamaria; BELLIS, Elisa; CAGGIANO, Valeria; GAGGIANO, Carla; TARSIA, Maria; MORMILE, Ilaria; EMMI, Giacomo; SFRISO, Paolo; MONTI, Sara; ERTEN, Suekran; GIUDICE, Emanuela Del; LUBRANO, Riccardo; CONTI, Giovanni; OLIVIERI, Alma Nunzia; GULLO, Alberto Lo; THARWAT, Samar; KARAMANAKOS, Anastasios; GIDARO, Antonio; MAGGIO, Maria Cristina; TORRE, Francesco La; CARDINALE, Fabio; OGUNJIMI, Benson; MAIER, Armin; SEBASTIANI, Gian Domenico; OPRIS-BELINSKI, Daniela; FRASSI, Micol; VIAPIANA, Ombretta; BIZZI, Emanuele; CARUBBI, Francesco; FOTIS, Lampros; TUFAN, Abdurrahman; KARDAS, Riza Can; WIESIK-SZEWCZYK, Ewa; JAHNZ-ROZYK, Karina; FABIANI, Claudia; FREDIANI, Bruno; BALISTRERI, Alberto; RIGANTE, Donato; CANTARINI, Luca
    Objectives: To describe clinical characteristics of patients with Still's disease treated with methotrexate (MTX) and to assess drug effectiveness evaluating change in disease activity, reduction of inflammatory markers, and glucocorticoid (GC)-sparing effect. Methods: Patients with Still's disease treated with MTX were assessed among those included in AIDA Network Still Disease Registry.Results: In this registry, 171 patients with Still's disease were treated with MTX (males 43.3%, age 37.1 & PLUSMN; 16.0 years). They were mainly characterised by joint features and fever without a prominent multiorgan involvement. MTX was administered with GCs in 68.4% of patients, with other conventional synthetic DMARDs in 6.4%, and with biologic DMARDs in 25.1%. A significant reduction of the modified systemic score was observed, and 38.6% patients were codified as being in clinical remission at the end of follow-up. The concomitant administration of a biologic DMARD resulted a predictor of the clinical remission. Furthermore, a reduction of inflammatory markers and ferritin levels was observed following the administration of MTX. Additionally, a marked reduction of the dosage of concomitant GCs was identified, while 36.7% discontinued such drugs. Male gender appeared as a predictor of GC discontinuation. MTX was discontinued in 12.3% of patients because of adverse effects, and in 12.3% for lack of efficacy.Conclusions: Clinical characteristics of patients with Still's disease treated with MTX were described, mainly joint features and fever without a prominent multiorgan involvement. The clinical usefulness of MTX was reported in reducing the disease activity, decreasing the inflammatory markers, and as GC-sparing agent.
  • conferenceObject
    Anti Pentraxin 3 Autoantibodies in Brazilian Patients with Granulomatosis with Polyangiitis
    (2020) TOLEDO, A.; ASSUNCAO, G. L.; OLIVEIRA, E. P.; GIARDINI, H.; PEREIRA, R.; BARBAS, C. S.
  • 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.
  • conferenceObject
    ASSOCIATION BETWEEN OSTEOPROTEGERIN AND RANKLSINGLE NUCLEOTIDE POLYMORPHISMS AND DESTRUCTIVE RHINOSINUSITIS IN PATIENTS WITH GRANULOMATOSIS WITH POLYANGIIITIS
    (2023) FURQUIM, Marilia; HOUNPKE, Bidossessi; CAPARBO, Valeria; GIARDINI, Henrique; BARBAS, Carmen; DOMICIANO, Diogo; SHINJO, Samuel; PEREIRA, Rosa
  • conferenceObject
    CLOSE TO THE BONE: A CASE OF CONCURRENTMULTIFOCAL OSTEONECROSIS AND PERIPHERAL SPONDYLOARTHROPATHY
    (2022) MACEDO, M. B.; GIARDINI, H. A. M.; GUEDES, L. K. N.; PEREIRA, R. M. R.
  • article 2 Citação(ões) na Scopus
    Development and implementation of the AIDA international registry for patients with Schnitzler's syndrome
    (2022) SOTA, Jurgen; VITALE, Antonio; WIESIK-SZEWCZYK, Ewa; FRASSI, Micol; LOPALCO, Giuseppe; EMMI, Giacomo; GOVONI, Marcello; PAULIS, Amato de; MARINO, Achille; GIDARO, Antonio; MONTI, Sara; OPRIS-BELINSKI, Daniela; PEREIRA, Rosa Maria R.; JAHNZ-ROZYK, Karina; GAGGIANO, Carla; CRISAFULLI, Francesca; IANNONE, Florenzo; MATTIOLI, Irene; RUFFILLI, Francesca; MORMILE, Ilaria; RYBAK, Katarzyna; CAGGIANO, Valeria; AIRO, Paolo; TUFAN, Abdurrahman; GENTILESCHI, Stefano; RAGAB, Gaafar; ALMAGHLOUTH, Ibrahim A.; KHALIL, Adham Aboul-Fotouh; CATTALINI, Marco; TORRE, Francesco La; TARSIA, Maria; GIARDINI, Henrique A. Mayrink; SAAD, Moustafa Ali; BOCCHIA, Monica; CARONI, Federico; GIANI, Teresa; CINOTTI, Elisa; RUSCITTI, Piero; RUBEGNI, Pietro; DAGOSTIN, Marilia A.; FREDIANI, Bruno; GULER, Aslihan Avanoglu; CASA, Francesca Della; MAGGIO, Maria Cristina; RECKE, Andreas; BUBNOFF, Dagmar von; KRAUSE, Karoline; BALISTRERI, Alberto; FABIANI, Claudia; RIGANTE, Donato; CANTARINI, Luca
    Objective: The present paper describes the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry specifically dedicated to patients with Schnitzler's syndrome. Methods: This is a clinical physician-driven, population- and electronic-based registry implemented for the retrospective and prospective collection of real-life data from patients with Schnitzler's syndrome; the registry is based on the Research Electronic Data Capture (REDCap) tool, which is designed to collect standardized information for clinical research, and has been realized to change over time according to future scientific acquisitions and potentially communicate with other existing or future similar registries. Results: Since its launch, 113 centers from 23 countries in 4 continents have been involved. Fifty-seven have already obtained the approval from their local Ethics Committees. The platform counts 324 users (114 Principal Investigators, 205 Site Investigators, 2 Lead Investigators, and 3 data managers) at current (April 28th, 2022). The registry collects baseline and follow-up data using 3,924 fields organized into 25 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, laboratory, instrumental exams, therapies, socioeconomic information, and healthcare access. Conclusions: This International Registry for patients with Schnitzler's syndrome facilitates standardized data collection, enabling international collaborative projects through data sharing and dissemination of knowledge; in turn, it will shed light into many blind spots characterizing this complex autoinflammatory disorder.
  • article 4 Citação(ões) na Scopus
    Immunogenicity decay and case incidence six months post Sinovac-CoronaVac vaccine in autoimmune rheumatic diseases patients
    (2022) SILVA, Clovis A.; MEDEIROS-RIBEIRO, Ana C.; KUPA, Leonard V. K.; YUKI, Emily F. N.; PASOTO, Sandra G.; SAAD, Carla G. S.; FUSCO, Solange R. G.; PEREIRA, Rosa M. R.; SHINJO, Samuel K.; HALPERN, Ari S. R.; BORBA, Eduardo F.; SOUZA, Fernando H. C.; GUEDES, Lissiane K. N.; MIOSSI, Renata; BONFIGLIOLI, Karina R.; DOMICIANO, Diogo S.; SHIMABUCO, Andrea Y.; ANDRADE, Danieli C. O.; SEGURO, Luciana P. C.; FULLER, Ricardo; SAMPAIO-BARROS, Percival D.; ASSAD, Ana P. L.; MORAES, Julio C. B.; GOLDENSTEIN-SCHAINBERG, Claudia; GIARDINI, Henrique A. M.; SILVA, Henrique C.; MARTINS, Victor A. O.; VILLAMARIN, Lorena E. B.; NOVELLINO, Renata S.; SALES, Lucas P.; ARAUJO, Carlo S. R.; SILVA, Matheus S. R.; FILHO, Dilson M. N.; LOPES, Marta H.; DUARTE, Alberto J. S.; KALLAS, Esper G.; AIKAWA, Nadia E.; BONFA, Eloisa
    Characterising the response to SARS-CoV-2 post vaccination is critical in the appraisement of the induced immune response, performance and protective potential. Here the authors present data from a phase 4 clinical trial in autoimmune rheumatic disease patients 6 months post second dose of Sinovac-CoronaVac inactivated vaccine that show a marked reduction in antibody particularly in males or those under treatment with immune targeting therapies but saw no rise in COVID-19 disease. The determination of durability and vaccine-associated protection is essential for booster doses strategies, however data on the stability of SARS-CoV-2 immunity are scarce. Here we assess anti-SARS-CoV-2 immunogenicity decay and incident cases six months after the 2(nd) dose of Sinovac-CoronaVac inactivated vaccine (D210) in 828 autoimmune rheumatic diseases patients compared with 207 age/sex-balanced control individuals. The primary outcome is the presence of anti-S1/S2 SARS-CoV-2 IgG at 6 months compared to 6 weeks after 2nd vaccine dose for decay evaluation. Secondary outcomes are presence of neutralizing antibodies, percent inhibition by neutralizing, geometric mean titers and cumulative incident cases at 6 months after 2nd dose. Anti-S1/S2 IgG positivity and titers reduce to 23.8% and 38% in patients (p < 0.001) during the six-month follow up and 20% and 51% in controls (p < 0.001), respectively. Neutralizing antibodies positivity and percent inhibition declines 41% and 54% in patients (p < 0.001) and 39.7% and 47% in controls (p < 0.001). Multivariate logistic regression analysis show males (OR = 0.56;95% CI0.40-0.79), prednisone (OR = 0.56; 95% CI0.41-0.76), anti-TNF (OR = 0.66;95% CI0.45-0.96), abatacept (OR = 0.29; 95% CI0.15-0.56) and rituximab (OR = 0.32;95% CI0.11-0.90) associate with a substantial reduction in IgG response at day 210 in patients. Although cellular immunity was not assessed, a decrease of COVID-19 cases (from 27.5 to 8.1/100 person-years; p < 0.001) is observed despite the concomitant emergence and spread of the Delta variant. Altogether we show a reduction in immunity 6-months of Sinovac-CoronaVac 2nd dose, particularly in males and those under immunosuppressives therapies, without a concomitant rise in COVID-19 cases. (CoronavRheum clinicaltrials.gov:NCT04754698).