title: Adult-onset Still's disease: an Italian multicentre retrospective observational study of manifestations and treatments in 245 patients.
abstract: Adult-onset Still's disease (AOSD) is a systemic inflammatory condition of unknown aetiology characterized by typical episodes of spiking fever, evanescent rash, arthralgia, leukocytosis and hyperferritinemia. Given the lack of data in Italian series, we promote a multicentric data collection to characterize the clinical phenotype of Italian patients with AOSD. Data from 245 subjects diagnosed with AOSD were collected by 15 centres between March and May 2013. The diagnosis was made following Yamaguchi's criteria. Data regarding clinical manifestations, laboratory features, disease course and treatments were reported and compared with those presented in other published series of different ethnicity. The most frequent features were the following: arthritis (93 %), pyrexia (92.6 %), leukocytosis (89 %), negative ANA (90.4 %) and neutrophilia (82 %). As compared to other North American, North European, Middle Eastern and Far Eastern cohorts, Italian data show differences in clinical and laboratory findings. Regarding the treatments, in 21.9 % of cases, corticosteroids and traditional DMARDs have not been able to control the disease while biologics have been shown to be effective in 48 to 58 patients. This retrospective work summarizes the largest Italian multicentre series of AOSD patients and presents clinical and laboratory features that appear to be influenced by the ethnicity of the affected subjects.
authors.affiliation: Rheumatology Unit, Department of Medicine - DIMED, University of Padova, via Giustiniani, 2, 35128, Padova, Italy. paolo.sfriso@unipd.it.
authors.forename: Paolo
authors.initials: P
authors.lastname: Sfriso
authors.affiliation: Department of Internal Medicine and Medical Specialities - Rheumatology Unit, Sapienza University of Roma, Rome, Italy.
authors.forename: Roberta
authors.initials: R
authors.lastname: Priori
authors.affiliation: Department of Internal Medicine and Medical Specialities - Rheumatology Unit, Sapienza University of Roma, Rome, Italy.
authors.forename: Guido
authors.initials: G
authors.lastname: Valesini
authors.affiliation: Department of Rheumatology, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Rossi
authors.affiliation: Department of Rheumatology, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy.
authors.forename: Carlo Maurizio
authors.initials: CM
authors.lastname: Montecucco
authors.affiliation: Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: D'Ascanio
authors.affiliation: Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
authors.forename: Linda
authors.initials: L
authors.lastname: Carli
authors.affiliation: Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Bombardieri
authors.affiliation: Rheumatology Unit, Interdisciplinary Department of Medicine, University of Bari, Bari, Italy.
authors.forename: Gaetana
authors.initials: G
authors.lastname: LaSelva
authors.affiliation: Rheumatology Unit, Interdisciplinary Department of Medicine, University of Bari, Bari, Italy.
authors.forename: Florenzo
authors.initials: F
authors.lastname: Iannone
authors.affiliation: Rheumatology Unit, Interdisciplinary Department of Medicine, University of Bari, Bari, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Lapadula
authors.affiliation: Division of Rheumatology, Catholic University of the Sacred Heart, University of Roma, Rome, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Alivernini
authors.affiliation: Division of Rheumatology, Catholic University of the Sacred Heart, University of Roma, Rome, Italy.
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Ferraccioli
authors.affiliation: Rheumatology Unit, Department of Medical and Surgical Science for Adults and Children, University of Modena e Reggio Emilia, Modena, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: Colaci
authors.affiliation: Rheumatology Unit, Department of Medical and Surgical Science for Adults and Children, University of Modena e Reggio Emilia, Modena, Italy.
authors.forename: Clodoveo
authors.initials: C
authors.lastname: Ferri
authors.affiliation: Rheumatology Section, Department of Clinical and Experimental Internal Medicine, Second University of Napoli, Naples, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Iacono
authors.affiliation: Rheumatology Section, Department of Clinical and Experimental Internal Medicine, Second University of Napoli, Naples, Italy.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valentini
authors.affiliation: Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, Naples, Italy.
authors.forename: Luisa
authors.initials: L
authors.lastname: Costa
authors.affiliation: Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, Naples, Italy.
authors.forename: Raffaele
authors.initials: R
authors.lastname: Scarpa
authors.affiliation: Rheumatology Unit, Department of Clinical and Experimental Medicine, Sant'Anna Hospital, University of Ferrara, Ferrara, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: LoMonaco
authors.affiliation: Rheumatology Unit, Department of Clinical and Experimental Medicine, Sant'Anna Hospital, University of Ferrara, Ferrara, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Bagnari
authors.affiliation: Rheumatology Unit, Department of Clinical and Experimental Medicine, Sant'Anna Hospital, University of Ferrara, Ferrara, Italy.
authors.forename: Marcello
authors.initials: M
authors.lastname: Govoni
authors.affiliation: Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Piazza
authors.affiliation: Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy.
authors.forename: Silvano
authors.initials: S
authors.lastname: Adami
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, University of Palermo, Palermo, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Ciccia
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, University of Palermo, Palermo, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Triolo
authors.affiliation: Research Laboratory and Division of Clinical Rheumatology, University of Genova, Genoa, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Alessandri
authors.affiliation: Research Laboratory and Division of Clinical Rheumatology, University of Genova, Genoa, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Cutolo
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
authors.forename: Mauro
authors.initials: M
authors.lastname: Galeazzi
authors.affiliation: Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, University of L'Aquila, L'Aquila, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Ruscitti
authors.affiliation: Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, University of L'Aquila, L'Aquila, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Giacomelli
authors.affiliation: Rheumatology Unit, Department of Medicine - DIMED, University of Padova, via Giustiniani, 2, 35128, Padova, Italy.|Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, Naples, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Caso
authors.affiliation: Rheumatology Unit, Department of Medicine - DIMED, University of Padova, via Giustiniani, 2, 35128, Padova, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Galozzi
authors.affiliation: Rheumatology Unit, Department of Medicine - DIMED, University of Padova, via Giustiniani, 2, 35128, Padova, Italy.
authors.forename: Leonardo
authors.initials: L
authors.lastname: Punzi
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D015897:Comorbidity; D005260:Female; D005334:Fever / Q000453:epidemiology*; D006801:Humans; D007558:Italy; D007964:Leukocytosis / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016706:Still's Disease, Adult-Onset / Q000188:drug therapy*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D018501:Antirheumatic Agents
keywords: Adult-onset Still’s disease; Biologic drugs; Clinical presentation; Laboratory findings; Retrospective study