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Adult-onset Still’s disease: an Italian multicentre retrospective observational study of manifestations and treatments in 245 patients

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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.

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Authors and Affiliations

  1. Rheumatology Unit, Department of Medicine - DIMED, University of Padova, via Giustiniani, 2, 35128, Padova, Italy

    Paolo Sfriso, Francesco Caso, Paola Galozzi & Leonardo Punzi

  2. Department of Internal Medicine and Medical Specialities - Rheumatology Unit, Sapienza University of Roma, Rome, Italy

    Roberta Priori & Guido Valesini

  3. Department of Rheumatology, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy

    Silvia Rossi & Carlo Maurizio Montecucco

  4. Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy

    Anna D’Ascanio, Linda Carli & Stefano Bombardieri

  5. Rheumatology Unit, Interdisciplinary Department of Medicine, University of Bari, Bari, Italy

    Gaetana LaSelva, Florenzo Iannone & Giovanni Lapadula

  6. Division of Rheumatology, Catholic University of the Sacred Heart, University of Roma, Rome, Italy

    Stefano Alivernini & Gianfranco Ferraccioli

  7. Rheumatology Unit, Department of Medical and Surgical Science for Adults and Children, University of Modena e Reggio Emilia, Modena, Italy

    Michele Colaci & Clodoveo Ferri

  8. Rheumatology Section, Department of Clinical and Experimental Internal Medicine, Second University of Napoli, Naples, Italy

    Daniela Iacono & Gabriele Valentini

  9. Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, Naples, Italy

    Luisa Costa, Raffaele Scarpa & Francesco Caso

  10. Rheumatology Unit, Department of Clinical and Experimental Medicine, Sant’Anna Hospital, University of Ferrara, Ferrara, Italy

    Andrea LoMonaco, Valentina Bagnari & Marcello Govoni

  11. Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy

    Ilaria Piazza & Silvano Adami

  12. Division of Rheumatology, Department of Internal Medicine, University of Palermo, Palermo, Italy

    Francesco Ciccia & Giovanni Triolo

  13. Research Laboratory and Division of Clinical Rheumatology, University of Genova, Genoa, Italy

    Elisa Alessandri & Maurizio Cutolo

  14. Research Center of Systemic Autoinflammatory Diseases and Behçet’s Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy

    Luca Cantarini & Mauro Galeazzi

  15. Division of Rheumatology, Department of Biotechnological and Applied Clinical Science, University of L’Aquila, L’Aquila, Italy

    Piero Ruscitti & Roberto Giacomelli

Authors
  1. Paolo Sfriso
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  2. Roberta Priori
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  3. Guido Valesini
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  4. Silvia Rossi
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  5. Carlo Maurizio Montecucco
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  6. Anna D’Ascanio
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  7. Linda Carli
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  8. Stefano Bombardieri
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  9. Gaetana LaSelva
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  10. Florenzo Iannone
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  11. Giovanni Lapadula
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  12. Stefano Alivernini
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  13. Gianfranco Ferraccioli
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  14. Michele Colaci
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  15. Clodoveo Ferri
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  16. Daniela Iacono
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  17. Gabriele Valentini
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  18. Luisa Costa
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  19. Raffaele Scarpa
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  20. Andrea LoMonaco
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  21. Valentina Bagnari
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  22. Marcello Govoni
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  23. Ilaria Piazza
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  24. Silvano Adami
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  25. Francesco Ciccia
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  26. Giovanni Triolo
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  27. Elisa Alessandri
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  28. Maurizio Cutolo
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  29. Luca Cantarini
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  30. Mauro Galeazzi
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  31. Piero Ruscitti
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  32. Roberto Giacomelli
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  33. Francesco Caso
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  34. Paola Galozzi
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  35. Leonardo Punzi
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Corresponding author

Correspondence to Paolo Sfriso.

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This retrospective study was approved by Padua Hospital Ethics Committee.

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Sfriso, P., Priori, R., Valesini, G. et al. Adult-onset Still’s disease: an Italian multicentre retrospective observational study of manifestations and treatments in 245 patients. Clin Rheumatol 35, 1683–1689 (2016). https://doi.org/10.1007/s10067-016-3308-8

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  • DOI: https://doi.org/10.1007/s10067-016-3308-8

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