HENRIQUE AYRES MAYRINK GIARDINI
Índice h a partir de 2011
4
Projetos de Pesquisa
Unidades Organizacionais
P ICHC, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/17 - Laboratório de Investigação em Reumatologia, Hospital das Clínicas, Faculdade de Medicina
LIM/17 - Laboratório de Investigação em Reumatologia, Hospital das Clínicas, Faculdade de Medicina
10 resultados
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- 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, LucaObjectives: 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.
- 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, LucaIntroductionThis 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- 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, LucaTo 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.
- 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 NetworkObjectiveTo 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.
- 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, LucaBeckgroundDespite 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.
- Adult-onset Still's disease with ankylosis of the distal interphalangeal joints: beyond psoriatic arthritis(2023) CORDEIRO, R. A.; ANTONELLI, I. P. B.; GIARDINI, H. A. M.
- 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, LucaIntroduction: 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.
- Still's disease continuum from childhood to elderly: data from the international AIDA Network Still's disease registry(2023) VITALE, Antonio; CAGGIANO, Valeria; LOPALCO, Giuseppe; GIARDINI, Henrique A. Mayrink; CICCIA, Francesco; ALMAGHLOUTH, Ibrahim A.; RUSCITTI, Piero; SFIKAKIS, Petros P.; TUFAN, Abdurrahman; DAGNA, Lorenzo; GIACOMELLI, Roberto; HINOJOSA-AZAOLA, Andrea; RAGAB, Gafaar; DIRESKENELI, Haner; FOTIS, Lampros; SOTA, Jurgen; IANNONE, Florenzo; MORRONE, Maria; ANTONELLI, Isabele Parente de Brito; DAGOSTIN, Marilia Ambiel; IACONO, Daniela; PATRONE, Martina; ASFINA, Kazi; ALANAZI, Fehaid; COLA, Ilenia Di; GAGGIANO, Carla; TEKTONIDOU, Maria G.; KARDAS, Riza Can; KUCUK, Hamit; CAMPOCHIARO, Corrado; TOMELLERI, Alessandro; NAVARINI, Luca; BERARDICURTI, Onorina; MARTIN-NARES, Eduardo; TORRES-RUIZ, Jiram; MAHMOUD, Ayman Abdel-Monem Ahmed; ALIBAZ-ONER, Fatma; KOURTESI, Katerina; TARSIA, Maria; SFRISO, Paolo; MAKOWSKA, Joanna; GOVONI, Marcello; TORRE, Francesco La; MAGGIO, Maria Cristina; MONTI, Sara; GIUDICE, Emanuela Del; EMMI, Giacomo; BARTOLONI, Elena; HERNANDEZ-RODRIGUEZ, Jose; GOMEZ-CAVERZASCHI, Veronica; MAIER, Armin; SIMONINI, Gabriele; IAGNOCCO, Annamaria; CONTI, Giovanni; OLIVIERI, Alma Nunzia; PAULIS, Amato De; GULLO, Alberto Lo; VIAPIANA, Ombretta; WIESIK-SZEWCZYK, Ewa; ERTEN, Sukran; OGUNJIMI, Benson; CARUBBI, Francesco; THARWAT, Samar; LASKARI, Katerina; COSTI, Stefania; TRIGGIANESE, Paola; KARAMANAKOS, Anastasios; CONFORTI, Alessandro; FRASSI, Micol; SEBASTIANI, Gian Domenico; GIDARO, Antonio; MAURO, Angela; BALISTRERI, Alberto; FABIANI, Claudia; FREDIANI, Bruno; CANTARINI, LucaObjective Still's disease is more frequently observed in the paediatric context, but a delayed onset is not exceptional both in the adulthood and in the elderly. However, whether paediatric-onset, adult-onset and elderly-onset Still's disease represent expressions of the same disease continuum or different clinical entities is still a matter of controversy. The aim of this study is to search for any differences in demographic, clinical features and response to treatment between pediatric-onset, adult-onset and elderly-onset Still's disease. Methods Subjects included in this study were drawn from the International AutoInflammatory Disease Alliance Network registry for patients with Still's disease. Results A total of 411 patients suffering from Still's disease were enrolled; the disease occurred in the childhood in 65 (15.8%) patients, in the adult 314 (76.4%) patients and in the elderly in 32 (7.8%) patients. No statistically significant differences at post-hoc analysis were observed in demographic features of the disease between pediatric-onset, adult-onset and elderly-onset Still's disease. The salmon-coloured skin rash (p=0.004), arthritis (p=0.009) and abdominal pain (p=0.007) resulted significantly more frequent among paediatric patients than in adult cases, while pleuritis (p=0.015) and arthralgia (p<0.0001) were significantly more frequent among elderly-onset patients compared with paediatric-onset subjects. Regarding laboratory data, thrombocytosis was significantly more frequent among paediatric patients onset compared with adult-onset subjects (p<0.0001), while thrombocytopenia was more frequent among elderly-onset patients although statistical significance was only bordered. No substantial differences were observed in the response to treatments. Conclusions Despite some minor difference between groups, overall, demographic, clinical, laboratory and treatments aspects of Still's disease were similarly observed in patients at all ages. This supports that pediatric-onset, adult-onset and elderly-onset Still's disease is the same clinical condition arising in different ages.
- 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, LucaThis 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).