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Comparison of Early vs. Delayed Anakinra Treatment in Patients With Adult Onset Still's Disease and Effect on Clinical and Laboratory Outcomes

Identifieur interne : 000181 ( Main/Exploration ); précédent : 000180; suivant : 000182

Comparison of Early vs. Delayed Anakinra Treatment in Patients With Adult Onset Still's Disease and Effect on Clinical and Laboratory Outcomes

Auteurs : Antonio Vitale [Italie] ; Giulio Cavalli [Italie] ; Piero Ruscitti [Italie] ; Jurgen Sota [Italie] ; Serena Colafrancesco [Italie] ; Roberta Priori [Italie] ; Guido Valesini [Italie] ; Lorenza Maria Argolini [Italie] ; Elena Baldissera [Italie] ; Elena Bartoloni [Italie] ; Daniele Cammelli [Italie] ; Giovanni Canestrari [Italie] ; Elena Cavallaro [Italie] ; Maria Grazia Massaro [Italie] ; Paola Cipriani [Italie] ; Ginevra De Marchi [Italie] ; Salvatore De Vita [Italie] ; Giacomo Emmi [Italie] ; Micol Frassi [Italie] ; Roberto Gerli [Italie] ; Elisa Gremese [Italie] ; Florenzo Iannone [Italie] ; Marco Fornaro [Italie] ; Anna Paladini [Italie] ; Giuseppe Lopalco [Italie] ; Raffaele Manna [Italie] ; Alessandro Mathieu [Italie] ; Carlomaurizio Montecucco [Italie] ; Marta Mosca [Italie] ; Ilaria Piazza [Italie] ; Matteo Piga [Italie] ; Irene Pontikaki [Italie] ; Micol Romano [Italie] ; Silvia Rossi [Italie] ; Maurizio Rossini [Italie] ; Elena Silvestri [Italie] ; Chiara Stagnaro [Italie] ; Rosaria Talarico [Italie] ; Bruno Frediani [Italie] ; Angela Tincani [Italie] ; Ombretta Viapiana [Italie] ; Gianfranco Vitiello [Italie] ; Paola Galozzi [Italie] ; Paolo Sfriso [Italie] ; Carla Gaggiano [Italie] ; Salvatore Grosso [Italie] ; Donato Rigante [Italie] ; Lorenzo Dagna [Italie] ; Roberto Giacomelli [Italie] ; Luca Cantarini [Italie]

Source :

RBID : PMC:7047849

Abstract

Background: Aim of this study was to search for any difference in the outcome of patients with adult onset Still's disease (AOSD) treated with anakinra (ANK) in relation with the interval between disease onset and the start of anti-interleukin(IL)-1 treatment and according with the different lines of ANK treatment.

Patients and Methods: One hundred and forty-one AOSD patients treated with ANK have been retrospectively assessed. Statistically significant differences (p < 0.05) were analyzed in the frequency of ANK effectiveness, primary or secondary inefficacy to ANK and rate of resolution of clinical and laboratory AOSD manifestations after 3, 6, and 12 months since ANK treatment according with different lines of treatment and different times between AOSD onset and start of ANK.

Results: No significant differences were identified in the ANK effectiveness and frequency of primary or secondary inefficacy for patients starting ANK within 6 months (p = 0.19, p = 0.14, and p = 0.81, respectively) or 12 months (p = 0.37, p = 0.23, and p = 0.81, respectively) since AOSD onset compared with patients starting ANK thereafter; no significant differences were identified in ANK effectiveness and primary or secondary inefficacy according with different lines of ANK treatment (p = 0.06, p = 0.19, and p = 0.13, respectively). Patients starting ANK within 6 and 12 months since AOSD onset showed a significantly quicker decrease of erythrocyte sedimentation rate and C-reactive protein than observed among patients undergoing ANK treatment after 6 and 12 months. The number of swollen joints at the 3 month follow-up visit was significantly lower among patients undergoing ANK within 6 months since AOSD onset (p = 0.01), while no significance was identified at the 6 and 12 month assessments (p = 0.23 and p = 0.45, respectively). At the 3 and 6 month visits, the number of swollen joints was significantly higher among patients previously treated with conventional and biological disease modifying anti-rheumatic drugs (DMARDs) compared with those formerly treated only with conventional DMARDs (p < 0.017).

Conclusions: Clinical and therapeutic outcomes are substantially independent of how early ANK treatment is started in AOSD patients. However, a faster ANK effectiveness in controlling systemic inflammation and resolving articular manifestations may be observed in patients benefiting from IL-1 inhibition as soon as after disease onset.


Url:
DOI: 10.3389/fmed.2020.00042
PubMed: 32154255
PubMed Central: 7047849


Affiliations:


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Le document en format XML

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<name sortKey="Colafrancesco, Serena" sort="Colafrancesco, Serena" uniqKey="Colafrancesco S" first="Serena" last="Colafrancesco">Serena Colafrancesco</name>
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<name sortKey="Priori, Roberta" sort="Priori, Roberta" uniqKey="Priori R" first="Roberta" last="Priori">Roberta Priori</name>
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<name sortKey="Valesini, Guido" sort="Valesini, Guido" uniqKey="Valesini G" first="Guido" last="Valesini">Guido Valesini</name>
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<addr-line>Rome</addr-line>
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<name sortKey="Argolini, Lorenza Maria" sort="Argolini, Lorenza Maria" uniqKey="Argolini L" first="Lorenza Maria" last="Argolini">Lorenza Maria Argolini</name>
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<name sortKey="Baldissera, Elena" sort="Baldissera, Elena" uniqKey="Baldissera E" first="Elena" last="Baldissera">Elena Baldissera</name>
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<institution>Department of Experimental and Clinical Medicine, University of Florence</institution>
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<addr-line>Florence</addr-line>
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<country xml:lang="fr">Italie</country>
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<name sortKey="Cavallaro, Elena" sort="Cavallaro, Elena" uniqKey="Cavallaro E" first="Elena" last="Cavallaro">Elena Cavallaro</name>
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<institution>Department of Medical and Biological Sciences, Rheumatology Clinic, University of Udine</institution>
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<addr-line>Udine</addr-line>
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<name sortKey="Massaro, Maria Grazia" sort="Massaro, Maria Grazia" uniqKey="Massaro M" first="Maria Grazia" last="Massaro">Maria Grazia Massaro</name>
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<name sortKey="Cipriani, Paola" sort="Cipriani, Paola" uniqKey="Cipriani P" first="Paola" last="Cipriani">Paola Cipriani</name>
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<name sortKey="De Marchi, Ginevra" sort="De Marchi, Ginevra" uniqKey="De Marchi G" first="Ginevra" last="De Marchi">Ginevra De Marchi</name>
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<addr-line>Udine</addr-line>
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<addr-line>Florence</addr-line>
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<country xml:lang="fr">Italie</country>
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<addr-line>Brescia</addr-line>
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<name sortKey="Gerli, Roberto" sort="Gerli, Roberto" uniqKey="Gerli R" first="Roberto" last="Gerli">Roberto Gerli</name>
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<institution>Rheumatology Unit, Department of Medicine, University of Perugia</institution>
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<addr-line>Perugia</addr-line>
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<name sortKey="Gremese, Elisa" sort="Gremese, Elisa" uniqKey="Gremese E" first="Elisa" last="Gremese">Elisa Gremese</name>
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<addr-line>Rome</addr-line>
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<name sortKey="Fornaro, Marco" sort="Fornaro, Marco" uniqKey="Fornaro M" first="Marco" last="Fornaro">Marco Fornaro</name>
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<name sortKey="Paladini, Anna" sort="Paladini, Anna" uniqKey="Paladini A" first="Anna" last="Paladini">Anna Paladini</name>
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<name sortKey="Manna, Raffaele" sort="Manna, Raffaele" uniqKey="Manna R" first="Raffaele" last="Manna">Raffaele Manna</name>
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<name sortKey="Mathieu, Alessandro" sort="Mathieu, Alessandro" uniqKey="Mathieu A" first="Alessandro" last="Mathieu">Alessandro Mathieu</name>
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<institution>Department of Rheumatology, IRCCS Policlinico San Matteo Foundation, University of Pavia</institution>
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<addr-line>Pavia</addr-line>
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<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
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<name sortKey="Mosca, Marta" sort="Mosca, Marta" uniqKey="Mosca M" first="Marta" last="Mosca">Marta Mosca</name>
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<institution>Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa</institution>
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<addr-line>Pisa</addr-line>
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<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
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<name sortKey="Piazza, Ilaria" sort="Piazza, Ilaria" uniqKey="Piazza I" first="Ilaria" last="Piazza">Ilaria Piazza</name>
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<institution>Rheumatology Unit, Department of Medicine, University of Verona</institution>
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<addr-line>Verona</addr-line>
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<country>Italy</country>
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<country xml:lang="fr">Italie</country>
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<name sortKey="Piga, Matteo" sort="Piga, Matteo" uniqKey="Piga M" first="Matteo" last="Piga">Matteo Piga</name>
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<institution>Rheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari</institution>
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<addr-line>Cagliari</addr-line>
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<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
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<author>
<name sortKey="Pontikaki, Irene" sort="Pontikaki, Irene" uniqKey="Pontikaki I" first="Irene" last="Pontikaki">Irene Pontikaki</name>
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<institution>Division of Rheumatology, ASST Gaetano Pini</institution>
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<addr-line>Milan</addr-line>
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<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
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<name sortKey="Romano, Micol" sort="Romano, Micol" uniqKey="Romano M" first="Micol" last="Romano">Micol Romano</name>
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<institution>Division of Rheumatology, ASST Gaetano Pini</institution>
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<addr-line>Milan</addr-line>
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<country>Italy</country>
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<country xml:lang="fr">Italie</country>
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</author>
<author>
<name sortKey="Rossi, Silvia" sort="Rossi, Silvia" uniqKey="Rossi S" first="Silvia" last="Rossi">Silvia Rossi</name>
<affiliation wicri:level="1">
<nlm:aff id="aff15">
<institution>Department of Rheumatology, IRCCS Policlinico San Matteo Foundation, University of Pavia</institution>
,
<addr-line>Pavia</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Rossini, Maurizio" sort="Rossini, Maurizio" uniqKey="Rossini M" first="Maurizio" last="Rossini">Maurizio Rossini</name>
<affiliation wicri:level="1">
<nlm:aff id="aff17">
<institution>Rheumatology Unit, Department of Medicine, University of Verona</institution>
,
<addr-line>Verona</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Silvestri, Elena" sort="Silvestri, Elena" uniqKey="Silvestri E" first="Elena" last="Silvestri">Elena Silvestri</name>
<affiliation wicri:level="1">
<nlm:aff id="aff8">
<institution>Department of Experimental and Clinical Medicine, University of Florence</institution>
,
<addr-line>Florence</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Stagnaro, Chiara" sort="Stagnaro, Chiara" uniqKey="Stagnaro C" first="Chiara" last="Stagnaro">Chiara Stagnaro</name>
<affiliation wicri:level="1">
<nlm:aff id="aff16">
<institution>Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa</institution>
,
<addr-line>Pisa</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Talarico, Rosaria" sort="Talarico, Rosaria" uniqKey="Talarico R" first="Rosaria" last="Talarico">Rosaria Talarico</name>
<affiliation wicri:level="1">
<nlm:aff id="aff16">
<institution>Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa</institution>
,
<addr-line>Pisa</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Frediani, Bruno" sort="Frediani, Bruno" uniqKey="Frediani B" first="Bruno" last="Frediani">Bruno Frediani</name>
<affiliation wicri:level="1">
<nlm:aff id="aff1">
<institution>Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena</institution>
,
<addr-line>Siena</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Tincani, Angela" sort="Tincani, Angela" uniqKey="Tincani A" first="Angela" last="Tincani">Angela Tincani</name>
<affiliation wicri:level="1">
<nlm:aff id="aff12">
<institution>Rheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of Brescia</institution>
,
<addr-line>Brescia</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Viapiana, Ombretta" sort="Viapiana, Ombretta" uniqKey="Viapiana O" first="Ombretta" last="Viapiana">Ombretta Viapiana</name>
<affiliation wicri:level="1">
<nlm:aff id="aff17">
<institution>Rheumatology Unit, Department of Medicine, University of Verona</institution>
,
<addr-line>Verona</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Vitiello, Gianfranco" sort="Vitiello, Gianfranco" uniqKey="Vitiello G" first="Gianfranco" last="Vitiello">Gianfranco Vitiello</name>
<affiliation wicri:level="1">
<nlm:aff id="aff8">
<institution>Department of Experimental and Clinical Medicine, University of Florence</institution>
,
<addr-line>Florence</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Galozzi, Paola" sort="Galozzi, Paola" uniqKey="Galozzi P" first="Paola" last="Galozzi">Paola Galozzi</name>
<affiliation wicri:level="1">
<nlm:aff id="aff18">
<institution>Department of Medicine DIMED, Rheumatology Unit, University of Padua</institution>
,
<addr-line>Padua</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Sfriso, Paolo" sort="Sfriso, Paolo" uniqKey="Sfriso P" first="Paolo" last="Sfriso">Paolo Sfriso</name>
<affiliation wicri:level="1">
<nlm:aff id="aff18">
<institution>Department of Medicine DIMED, Rheumatology Unit, University of Padua</institution>
,
<addr-line>Padua</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Gaggiano, Carla" sort="Gaggiano, Carla" uniqKey="Gaggiano C" first="Carla" last="Gaggiano">Carla Gaggiano</name>
<affiliation wicri:level="1">
<nlm:aff id="aff19">
<institution>Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena</institution>
,
<addr-line>Siena</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Grosso, Salvatore" sort="Grosso, Salvatore" uniqKey="Grosso S" first="Salvatore" last="Grosso">Salvatore Grosso</name>
<affiliation wicri:level="1">
<nlm:aff id="aff19">
<institution>Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena</institution>
,
<addr-line>Siena</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Rigante, Donato" sort="Rigante, Donato" uniqKey="Rigante D" first="Donato" last="Rigante">Donato Rigante</name>
<affiliation wicri:level="1">
<nlm:aff id="aff20">
<institution>Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS</institution>
,
<addr-line>Rome</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Dagna, Lorenzo" sort="Dagna, Lorenzo" uniqKey="Dagna L" first="Lorenzo" last="Dagna">Lorenzo Dagna</name>
<affiliation wicri:level="1">
<nlm:aff id="aff2">
<institution>Department of General and Specialized Medicine, Vita-Salute San Raffaele University</institution>
,
<addr-line>Milan</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
<affiliation wicri:level="1">
<nlm:aff id="aff3">
<institution>Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute</institution>
,
<addr-line>Milan</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Giacomelli, Roberto" sort="Giacomelli, Roberto" uniqKey="Giacomelli R" first="Roberto" last="Giacomelli">Roberto Giacomelli</name>
<affiliation wicri:level="1">
<nlm:aff id="aff4">
<institution>Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, University of L'Aquila</institution>
,
<addr-line>L'Aquila</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Cantarini, Luca" sort="Cantarini, Luca" uniqKey="Cantarini L" first="Luca" last="Cantarini">Luca Cantarini</name>
<affiliation wicri:level="1">
<nlm:aff id="aff1">
<institution>Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena</institution>
,
<addr-line>Siena</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
</titleStmt>
<publicationStmt>
<idno type="wicri:source">PMC</idno>
<idno type="pmid">32154255</idno>
<idno type="pmc">7047849</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7047849</idno>
<idno type="RBID">PMC:7047849</idno>
<idno type="doi">10.3389/fmed.2020.00042</idno>
<date when="2020">2020</date>
<idno type="wicri:Area/Pmc/Corpus">000551</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000551</idno>
<idno type="wicri:Area/Pmc/Curation">000551</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">000551</idno>
<idno type="wicri:Area/Pmc/Checkpoint">000169</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">000169</idno>
<idno type="wicri:Area/Ncbi/Merge">001006</idno>
<idno type="wicri:Area/Ncbi/Curation">001006</idno>
<idno type="wicri:Area/Ncbi/Checkpoint">001006</idno>
<idno type="wicri:Area/Main/Merge">000181</idno>
<idno type="wicri:Area/Main/Curation">000181</idno>
<idno type="wicri:Area/Main/Exploration">000181</idno>
</publicationStmt>
<sourceDesc>
<biblStruct>
<analytic>
<title xml:lang="en" level="a" type="main">Comparison of Early vs. Delayed Anakinra Treatment in Patients With Adult Onset Still's Disease and Effect on Clinical and Laboratory Outcomes</title>
<author>
<name sortKey="Vitale, Antonio" sort="Vitale, Antonio" uniqKey="Vitale A" first="Antonio" last="Vitale">Antonio Vitale</name>
<affiliation wicri:level="1">
<nlm:aff id="aff1">
<institution>Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena</institution>
,
<addr-line>Siena</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Cavalli, Giulio" sort="Cavalli, Giulio" uniqKey="Cavalli G" first="Giulio" last="Cavalli">Giulio Cavalli</name>
<affiliation wicri:level="1">
<nlm:aff id="aff2">
<institution>Department of General and Specialized Medicine, Vita-Salute San Raffaele University</institution>
,
<addr-line>Milan</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
<affiliation wicri:level="1">
<nlm:aff id="aff3">
<institution>Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute</institution>
,
<addr-line>Milan</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Ruscitti, Piero" sort="Ruscitti, Piero" uniqKey="Ruscitti P" first="Piero" last="Ruscitti">Piero Ruscitti</name>
<affiliation wicri:level="1">
<nlm:aff id="aff4">
<institution>Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, University of L'Aquila</institution>
,
<addr-line>L'Aquila</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Sota, Jurgen" sort="Sota, Jurgen" uniqKey="Sota J" first="Jurgen" last="Sota">Jurgen Sota</name>
<affiliation wicri:level="1">
<nlm:aff id="aff1">
<institution>Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena</institution>
,
<addr-line>Siena</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Colafrancesco, Serena" sort="Colafrancesco, Serena" uniqKey="Colafrancesco S" first="Serena" last="Colafrancesco">Serena Colafrancesco</name>
<affiliation wicri:level="1">
<nlm:aff id="aff5">
<institution>Rheumatology Unit, Department of Internal Medicine and Medical Specialties, Sapienza University of Rome</institution>
,
<addr-line>Rome</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Priori, Roberta" sort="Priori, Roberta" uniqKey="Priori R" first="Roberta" last="Priori">Roberta Priori</name>
<affiliation wicri:level="1">
<nlm:aff id="aff5">
<institution>Rheumatology Unit, Department of Internal Medicine and Medical Specialties, Sapienza University of Rome</institution>
,
<addr-line>Rome</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Valesini, Guido" sort="Valesini, Guido" uniqKey="Valesini G" first="Guido" last="Valesini">Guido Valesini</name>
<affiliation wicri:level="1">
<nlm:aff id="aff5">
<institution>Rheumatology Unit, Department of Internal Medicine and Medical Specialties, Sapienza University of Rome</institution>
,
<addr-line>Rome</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Argolini, Lorenza Maria" sort="Argolini, Lorenza Maria" uniqKey="Argolini L" first="Lorenza Maria" last="Argolini">Lorenza Maria Argolini</name>
<affiliation wicri:level="1">
<nlm:aff id="aff6">
<institution>Division of Rheumatology, ASST Gaetano Pini</institution>
,
<addr-line>Milan</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Baldissera, Elena" sort="Baldissera, Elena" uniqKey="Baldissera E" first="Elena" last="Baldissera">Elena Baldissera</name>
<affiliation wicri:level="1">
<nlm:aff id="aff3">
<institution>Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute</institution>
,
<addr-line>Milan</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Bartoloni, Elena" sort="Bartoloni, Elena" uniqKey="Bartoloni E" first="Elena" last="Bartoloni">Elena Bartoloni</name>
<affiliation wicri:level="1">
<nlm:aff id="aff7">
<institution>Rheumatology Unit, Department of Medicine, University of Perugia</institution>
,
<addr-line>Perugia</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Cammelli, Daniele" sort="Cammelli, Daniele" uniqKey="Cammelli D" first="Daniele" last="Cammelli">Daniele Cammelli</name>
<affiliation wicri:level="1">
<nlm:aff id="aff8">
<institution>Department of Experimental and Clinical Medicine, University of Florence</institution>
,
<addr-line>Florence</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Canestrari, Giovanni" sort="Canestrari, Giovanni" uniqKey="Canestrari G" first="Giovanni" last="Canestrari">Giovanni Canestrari</name>
<affiliation wicri:level="1">
<nlm:aff id="aff9">
<institution>Institute of Rheumatology and Affine Sciences, Division of Rheumatology, Catholic University of the Sacred Heart</institution>
,
<addr-line>Rome</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Cavallaro, Elena" sort="Cavallaro, Elena" uniqKey="Cavallaro E" first="Elena" last="Cavallaro">Elena Cavallaro</name>
<affiliation wicri:level="1">
<nlm:aff id="aff10">
<institution>Department of Medical and Biological Sciences, Rheumatology Clinic, University of Udine</institution>
,
<addr-line>Udine</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Massaro, Maria Grazia" sort="Massaro, Maria Grazia" uniqKey="Massaro M" first="Maria Grazia" last="Massaro">Maria Grazia Massaro</name>
<affiliation wicri:level="1">
<nlm:aff id="aff11">
<institution>Periodic Fever Research Center, Institute of Internal Medicine, Catholic University of the Sacred Heart, Fondazione Policlinico A. Gemelli</institution>
,
<addr-line>Rome</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Cipriani, Paola" sort="Cipriani, Paola" uniqKey="Cipriani P" first="Paola" last="Cipriani">Paola Cipriani</name>
<affiliation wicri:level="1">
<nlm:aff id="aff4">
<institution>Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, University of L'Aquila</institution>
,
<addr-line>L'Aquila</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="De Marchi, Ginevra" sort="De Marchi, Ginevra" uniqKey="De Marchi G" first="Ginevra" last="De Marchi">Ginevra De Marchi</name>
<affiliation wicri:level="1">
<nlm:aff id="aff10">
<institution>Department of Medical and Biological Sciences, Rheumatology Clinic, University of Udine</institution>
,
<addr-line>Udine</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="De Vita, Salvatore" sort="De Vita, Salvatore" uniqKey="De Vita S" first="Salvatore" last="De Vita">Salvatore De Vita</name>
<affiliation wicri:level="1">
<nlm:aff id="aff10">
<institution>Department of Medical and Biological Sciences, Rheumatology Clinic, University of Udine</institution>
,
<addr-line>Udine</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Emmi, Giacomo" sort="Emmi, Giacomo" uniqKey="Emmi G" first="Giacomo" last="Emmi">Giacomo Emmi</name>
<affiliation wicri:level="1">
<nlm:aff id="aff8">
<institution>Department of Experimental and Clinical Medicine, University of Florence</institution>
,
<addr-line>Florence</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Frassi, Micol" sort="Frassi, Micol" uniqKey="Frassi M" first="Micol" last="Frassi">Micol Frassi</name>
<affiliation wicri:level="1">
<nlm:aff id="aff12">
<institution>Rheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of Brescia</institution>
,
<addr-line>Brescia</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Gerli, Roberto" sort="Gerli, Roberto" uniqKey="Gerli R" first="Roberto" last="Gerli">Roberto Gerli</name>
<affiliation wicri:level="1">
<nlm:aff id="aff7">
<institution>Rheumatology Unit, Department of Medicine, University of Perugia</institution>
,
<addr-line>Perugia</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Gremese, Elisa" sort="Gremese, Elisa" uniqKey="Gremese E" first="Elisa" last="Gremese">Elisa Gremese</name>
<affiliation wicri:level="1">
<nlm:aff id="aff9">
<institution>Institute of Rheumatology and Affine Sciences, Division of Rheumatology, Catholic University of the Sacred Heart</institution>
,
<addr-line>Rome</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Iannone, Florenzo" sort="Iannone, Florenzo" uniqKey="Iannone F" first="Florenzo" last="Iannone">Florenzo Iannone</name>
<affiliation wicri:level="1">
<nlm:aff id="aff13">
<institution>Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari</institution>
,
<addr-line>Bari</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Fornaro, Marco" sort="Fornaro, Marco" uniqKey="Fornaro M" first="Marco" last="Fornaro">Marco Fornaro</name>
<affiliation wicri:level="1">
<nlm:aff id="aff13">
<institution>Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari</institution>
,
<addr-line>Bari</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Paladini, Anna" sort="Paladini, Anna" uniqKey="Paladini A" first="Anna" last="Paladini">Anna Paladini</name>
<affiliation wicri:level="1">
<nlm:aff id="aff1">
<institution>Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena</institution>
,
<addr-line>Siena</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Lopalco, Giuseppe" sort="Lopalco, Giuseppe" uniqKey="Lopalco G" first="Giuseppe" last="Lopalco">Giuseppe Lopalco</name>
<affiliation wicri:level="1">
<nlm:aff id="aff13">
<institution>Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari</institution>
,
<addr-line>Bari</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Manna, Raffaele" sort="Manna, Raffaele" uniqKey="Manna R" first="Raffaele" last="Manna">Raffaele Manna</name>
<affiliation wicri:level="1">
<nlm:aff id="aff11">
<institution>Periodic Fever Research Center, Institute of Internal Medicine, Catholic University of the Sacred Heart, Fondazione Policlinico A. Gemelli</institution>
,
<addr-line>Rome</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Mathieu, Alessandro" sort="Mathieu, Alessandro" uniqKey="Mathieu A" first="Alessandro" last="Mathieu">Alessandro Mathieu</name>
<affiliation wicri:level="1">
<nlm:aff id="aff14">
<institution>Rheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari</institution>
,
<addr-line>Cagliari</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Montecucco, Carlomaurizio" sort="Montecucco, Carlomaurizio" uniqKey="Montecucco C" first="Carlomaurizio" last="Montecucco">Carlomaurizio Montecucco</name>
<affiliation wicri:level="1">
<nlm:aff id="aff15">
<institution>Department of Rheumatology, IRCCS Policlinico San Matteo Foundation, University of Pavia</institution>
,
<addr-line>Pavia</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Mosca, Marta" sort="Mosca, Marta" uniqKey="Mosca M" first="Marta" last="Mosca">Marta Mosca</name>
<affiliation wicri:level="1">
<nlm:aff id="aff16">
<institution>Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa</institution>
,
<addr-line>Pisa</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Piazza, Ilaria" sort="Piazza, Ilaria" uniqKey="Piazza I" first="Ilaria" last="Piazza">Ilaria Piazza</name>
<affiliation wicri:level="1">
<nlm:aff id="aff17">
<institution>Rheumatology Unit, Department of Medicine, University of Verona</institution>
,
<addr-line>Verona</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Piga, Matteo" sort="Piga, Matteo" uniqKey="Piga M" first="Matteo" last="Piga">Matteo Piga</name>
<affiliation wicri:level="1">
<nlm:aff id="aff14">
<institution>Rheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari</institution>
,
<addr-line>Cagliari</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Pontikaki, Irene" sort="Pontikaki, Irene" uniqKey="Pontikaki I" first="Irene" last="Pontikaki">Irene Pontikaki</name>
<affiliation wicri:level="1">
<nlm:aff id="aff6">
<institution>Division of Rheumatology, ASST Gaetano Pini</institution>
,
<addr-line>Milan</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Romano, Micol" sort="Romano, Micol" uniqKey="Romano M" first="Micol" last="Romano">Micol Romano</name>
<affiliation wicri:level="1">
<nlm:aff id="aff6">
<institution>Division of Rheumatology, ASST Gaetano Pini</institution>
,
<addr-line>Milan</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Rossi, Silvia" sort="Rossi, Silvia" uniqKey="Rossi S" first="Silvia" last="Rossi">Silvia Rossi</name>
<affiliation wicri:level="1">
<nlm:aff id="aff15">
<institution>Department of Rheumatology, IRCCS Policlinico San Matteo Foundation, University of Pavia</institution>
,
<addr-line>Pavia</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Rossini, Maurizio" sort="Rossini, Maurizio" uniqKey="Rossini M" first="Maurizio" last="Rossini">Maurizio Rossini</name>
<affiliation wicri:level="1">
<nlm:aff id="aff17">
<institution>Rheumatology Unit, Department of Medicine, University of Verona</institution>
,
<addr-line>Verona</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Silvestri, Elena" sort="Silvestri, Elena" uniqKey="Silvestri E" first="Elena" last="Silvestri">Elena Silvestri</name>
<affiliation wicri:level="1">
<nlm:aff id="aff8">
<institution>Department of Experimental and Clinical Medicine, University of Florence</institution>
,
<addr-line>Florence</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Stagnaro, Chiara" sort="Stagnaro, Chiara" uniqKey="Stagnaro C" first="Chiara" last="Stagnaro">Chiara Stagnaro</name>
<affiliation wicri:level="1">
<nlm:aff id="aff16">
<institution>Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa</institution>
,
<addr-line>Pisa</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Talarico, Rosaria" sort="Talarico, Rosaria" uniqKey="Talarico R" first="Rosaria" last="Talarico">Rosaria Talarico</name>
<affiliation wicri:level="1">
<nlm:aff id="aff16">
<institution>Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa</institution>
,
<addr-line>Pisa</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Frediani, Bruno" sort="Frediani, Bruno" uniqKey="Frediani B" first="Bruno" last="Frediani">Bruno Frediani</name>
<affiliation wicri:level="1">
<nlm:aff id="aff1">
<institution>Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena</institution>
,
<addr-line>Siena</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Tincani, Angela" sort="Tincani, Angela" uniqKey="Tincani A" first="Angela" last="Tincani">Angela Tincani</name>
<affiliation wicri:level="1">
<nlm:aff id="aff12">
<institution>Rheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of Brescia</institution>
,
<addr-line>Brescia</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Viapiana, Ombretta" sort="Viapiana, Ombretta" uniqKey="Viapiana O" first="Ombretta" last="Viapiana">Ombretta Viapiana</name>
<affiliation wicri:level="1">
<nlm:aff id="aff17">
<institution>Rheumatology Unit, Department of Medicine, University of Verona</institution>
,
<addr-line>Verona</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Vitiello, Gianfranco" sort="Vitiello, Gianfranco" uniqKey="Vitiello G" first="Gianfranco" last="Vitiello">Gianfranco Vitiello</name>
<affiliation wicri:level="1">
<nlm:aff id="aff8">
<institution>Department of Experimental and Clinical Medicine, University of Florence</institution>
,
<addr-line>Florence</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Galozzi, Paola" sort="Galozzi, Paola" uniqKey="Galozzi P" first="Paola" last="Galozzi">Paola Galozzi</name>
<affiliation wicri:level="1">
<nlm:aff id="aff18">
<institution>Department of Medicine DIMED, Rheumatology Unit, University of Padua</institution>
,
<addr-line>Padua</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Sfriso, Paolo" sort="Sfriso, Paolo" uniqKey="Sfriso P" first="Paolo" last="Sfriso">Paolo Sfriso</name>
<affiliation wicri:level="1">
<nlm:aff id="aff18">
<institution>Department of Medicine DIMED, Rheumatology Unit, University of Padua</institution>
,
<addr-line>Padua</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Gaggiano, Carla" sort="Gaggiano, Carla" uniqKey="Gaggiano C" first="Carla" last="Gaggiano">Carla Gaggiano</name>
<affiliation wicri:level="1">
<nlm:aff id="aff19">
<institution>Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena</institution>
,
<addr-line>Siena</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Grosso, Salvatore" sort="Grosso, Salvatore" uniqKey="Grosso S" first="Salvatore" last="Grosso">Salvatore Grosso</name>
<affiliation wicri:level="1">
<nlm:aff id="aff19">
<institution>Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena</institution>
,
<addr-line>Siena</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Rigante, Donato" sort="Rigante, Donato" uniqKey="Rigante D" first="Donato" last="Rigante">Donato Rigante</name>
<affiliation wicri:level="1">
<nlm:aff id="aff20">
<institution>Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS</institution>
,
<addr-line>Rome</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Dagna, Lorenzo" sort="Dagna, Lorenzo" uniqKey="Dagna L" first="Lorenzo" last="Dagna">Lorenzo Dagna</name>
<affiliation wicri:level="1">
<nlm:aff id="aff2">
<institution>Department of General and Specialized Medicine, Vita-Salute San Raffaele University</institution>
,
<addr-line>Milan</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
<affiliation wicri:level="1">
<nlm:aff id="aff3">
<institution>Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute</institution>
,
<addr-line>Milan</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Giacomelli, Roberto" sort="Giacomelli, Roberto" uniqKey="Giacomelli R" first="Roberto" last="Giacomelli">Roberto Giacomelli</name>
<affiliation wicri:level="1">
<nlm:aff id="aff4">
<institution>Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, University of L'Aquila</institution>
,
<addr-line>L'Aquila</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
<author>
<name sortKey="Cantarini, Luca" sort="Cantarini, Luca" uniqKey="Cantarini L" first="Luca" last="Cantarini">Luca Cantarini</name>
<affiliation wicri:level="1">
<nlm:aff id="aff1">
<institution>Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena</institution>
,
<addr-line>Siena</addr-line>
,
<country>Italy</country>
</nlm:aff>
<country xml:lang="fr">Italie</country>
<wicri:regionArea># see nlm:aff country strict</wicri:regionArea>
</affiliation>
</author>
</analytic>
<series>
<title level="j">Frontiers in Medicine</title>
<idno type="eISSN">2296-858X</idno>
<imprint>
<date when="2020">2020</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc>
<textClass></textClass>
</profileDesc>
</teiHeader>
<front>
<div type="abstract" xml:lang="en">
<p>
<bold>Background:</bold>
Aim of this study was to search for any difference in the outcome of patients with adult onset Still's disease (AOSD) treated with anakinra (ANK) in relation with the interval between disease onset and the start of anti-interleukin(IL)-1 treatment and according with the different lines of ANK treatment.</p>
<p>
<bold>Patients and Methods:</bold>
One hundred and forty-one AOSD patients treated with ANK have been retrospectively assessed. Statistically significant differences (
<italic>p</italic>
< 0.05) were analyzed in the frequency of ANK effectiveness, primary or secondary inefficacy to ANK and rate of resolution of clinical and laboratory AOSD manifestations after 3, 6, and 12 months since ANK treatment according with different lines of treatment and different times between AOSD onset and start of ANK.</p>
<p>
<bold>Results:</bold>
No significant differences were identified in the ANK effectiveness and frequency of primary or secondary inefficacy for patients starting ANK within 6 months (
<italic>p</italic>
= 0.19,
<italic>p</italic>
= 0.14, and
<italic>p</italic>
= 0.81, respectively) or 12 months (
<italic>p</italic>
= 0.37,
<italic>p</italic>
= 0.23, and
<italic>p</italic>
= 0.81, respectively) since AOSD onset compared with patients starting ANK thereafter; no significant differences were identified in ANK effectiveness and primary or secondary inefficacy according with different lines of ANK treatment (
<italic>p</italic>
= 0.06,
<italic>p</italic>
= 0.19, and
<italic>p</italic>
= 0.13, respectively). Patients starting ANK within 6 and 12 months since AOSD onset showed a significantly quicker decrease of erythrocyte sedimentation rate and C-reactive protein than observed among patients undergoing ANK treatment after 6 and 12 months. The number of swollen joints at the 3 month follow-up visit was significantly lower among patients undergoing ANK within 6 months since AOSD onset (
<italic>p</italic>
= 0.01), while no significance was identified at the 6 and 12 month assessments (
<italic>p</italic>
= 0.23 and
<italic>p</italic>
= 0.45, respectively). At the 3 and 6 month visits, the number of swollen joints was significantly higher among patients previously treated with conventional and biological disease modifying anti-rheumatic drugs (DMARDs) compared with those formerly treated only with conventional DMARDs (
<italic>p</italic>
< 0.017).</p>
<p>
<bold>Conclusions:</bold>
Clinical and therapeutic outcomes are substantially independent of how early ANK treatment is started in AOSD patients. However, a faster ANK effectiveness in controlling systemic inflammation and resolving articular manifestations may be observed in patients benefiting from IL-1 inhibition as soon as after disease onset.</p>
</div>
</front>
<back>
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<name sortKey="Iannone, Florenzo" sort="Iannone, Florenzo" uniqKey="Iannone F" first="Florenzo" last="Iannone">Florenzo Iannone</name>
<name sortKey="Lopalco, Giuseppe" sort="Lopalco, Giuseppe" uniqKey="Lopalco G" first="Giuseppe" last="Lopalco">Giuseppe Lopalco</name>
<name sortKey="Manna, Raffaele" sort="Manna, Raffaele" uniqKey="Manna R" first="Raffaele" last="Manna">Raffaele Manna</name>
<name sortKey="Massaro, Maria Grazia" sort="Massaro, Maria Grazia" uniqKey="Massaro M" first="Maria Grazia" last="Massaro">Maria Grazia Massaro</name>
<name sortKey="Mathieu, Alessandro" sort="Mathieu, Alessandro" uniqKey="Mathieu A" first="Alessandro" last="Mathieu">Alessandro Mathieu</name>
<name sortKey="Montecucco, Carlomaurizio" sort="Montecucco, Carlomaurizio" uniqKey="Montecucco C" first="Carlomaurizio" last="Montecucco">Carlomaurizio Montecucco</name>
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<name sortKey="Paladini, Anna" sort="Paladini, Anna" uniqKey="Paladini A" first="Anna" last="Paladini">Anna Paladini</name>
<name sortKey="Piazza, Ilaria" sort="Piazza, Ilaria" uniqKey="Piazza I" first="Ilaria" last="Piazza">Ilaria Piazza</name>
<name sortKey="Piga, Matteo" sort="Piga, Matteo" uniqKey="Piga M" first="Matteo" last="Piga">Matteo Piga</name>
<name sortKey="Pontikaki, Irene" sort="Pontikaki, Irene" uniqKey="Pontikaki I" first="Irene" last="Pontikaki">Irene Pontikaki</name>
<name sortKey="Priori, Roberta" sort="Priori, Roberta" uniqKey="Priori R" first="Roberta" last="Priori">Roberta Priori</name>
<name sortKey="Rigante, Donato" sort="Rigante, Donato" uniqKey="Rigante D" first="Donato" last="Rigante">Donato Rigante</name>
<name sortKey="Romano, Micol" sort="Romano, Micol" uniqKey="Romano M" first="Micol" last="Romano">Micol Romano</name>
<name sortKey="Rossi, Silvia" sort="Rossi, Silvia" uniqKey="Rossi S" first="Silvia" last="Rossi">Silvia Rossi</name>
<name sortKey="Rossini, Maurizio" sort="Rossini, Maurizio" uniqKey="Rossini M" first="Maurizio" last="Rossini">Maurizio Rossini</name>
<name sortKey="Ruscitti, Piero" sort="Ruscitti, Piero" uniqKey="Ruscitti P" first="Piero" last="Ruscitti">Piero Ruscitti</name>
<name sortKey="Sfriso, Paolo" sort="Sfriso, Paolo" uniqKey="Sfriso P" first="Paolo" last="Sfriso">Paolo Sfriso</name>
<name sortKey="Silvestri, Elena" sort="Silvestri, Elena" uniqKey="Silvestri E" first="Elena" last="Silvestri">Elena Silvestri</name>
<name sortKey="Sota, Jurgen" sort="Sota, Jurgen" uniqKey="Sota J" first="Jurgen" last="Sota">Jurgen Sota</name>
<name sortKey="Stagnaro, Chiara" sort="Stagnaro, Chiara" uniqKey="Stagnaro C" first="Chiara" last="Stagnaro">Chiara Stagnaro</name>
<name sortKey="Talarico, Rosaria" sort="Talarico, Rosaria" uniqKey="Talarico R" first="Rosaria" last="Talarico">Rosaria Talarico</name>
<name sortKey="Tincani, Angela" sort="Tincani, Angela" uniqKey="Tincani A" first="Angela" last="Tincani">Angela Tincani</name>
<name sortKey="Valesini, Guido" sort="Valesini, Guido" uniqKey="Valesini G" first="Guido" last="Valesini">Guido Valesini</name>
<name sortKey="Viapiana, Ombretta" sort="Viapiana, Ombretta" uniqKey="Viapiana O" first="Ombretta" last="Viapiana">Ombretta Viapiana</name>
<name sortKey="Vitiello, Gianfranco" sort="Vitiello, Gianfranco" uniqKey="Vitiello G" first="Gianfranco" last="Vitiello">Gianfranco Vitiello</name>
</country>
</tree>
</affiliations>
</record>

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