Article Text

PDF
THU0395 Adult Onset Still's Disease: A Multicenter Retrospective Cohort Study of 233 Italian Patients
  1. P. Sfriso1,
  2. R. Priori2,
  3. S. Colafrancesco2,
  4. G. Valesini2,
  5. S. Rossi3,
  6. C. Montecucco3,
  7. A. D'Ascanio4,
  8. L. Carli4,
  9. S. Bombardieri4,
  10. G. La Selva5,
  11. F. Iannone5,
  12. G. Lapadula5,
  13. S. Alivernini6,
  14. G. Ferraccioli6,
  15. F. Caso7,
  16. M. Colaci8,
  17. C. Ferri8,
  18. D. Iacono9,
  19. G. La Montagna9,
  20. L. Costa10,
  21. R. Scarpa10,
  22. A. Lo Monaco11,
  23. V. Bagnari11,
  24. M. Govoni11,
  25. I. Piazza12,
  26. S. Adami12,
  27. C. Schinocca13,
  28. F. Ciccia13,
  29. G. Triolo13,
  30. E. Alessandri14,
  31. M. Cutolo14,
  32. L. Cantarini15,
  33. M. Galeazzi15,
  34. L. Punzi7
  35. on behalf of Italian Adult Onset Still's Disease Study Group
  1. 1Department of Medicine, University of Padova, Padova
  2. 2La Sapienza University of Rome, Rome
  3. 3University of Pavia, Pavia
  4. 4University of Pisa, Pisa
  5. 5University of Bari, Bari
  6. 6Catholic University of Rome, Rome
  7. 7University of Padova, Padova
  8. 8University of Modena and Reggio Emilia, Modena
  9. 9Second University of Napoli
  10. 10University Federico II of Napoli, Napoli
  11. 11University of Ferrara, Ferrara
  12. 12University of Verona, Verona
  13. 13University of Palermo, Palermo
  14. 14University of Genova, Genova
  15. 15University of Siena, Siena, Italy

Abstract

Background Adult onset Still's disease (AOSD) is a rare rheumatic disease with an estimated prevalence of less than 1 case per 100,000 population. The diagnosis is difficult and is often delayed due to the lack of specific diagnostic tests and the need to rule out other pathological entities.

Objectives To describe the clinical characteristics of a multicenter Italian case series of patients with AOSD.

Methods 14 Italian University Hospital centers participated in the study. A standardized medical record containing clinical data, laboratory investigations, disease patterns and the different therapies has been sent to all participating centers. Each center collected data retrospectively.

Results In the first wave of data collection, from March to May 2013, 233 patients were included with AOSD according to Yamaguchi criteria (125 males, 53.6%, mean age at onset: 40.2±16.2 years, time between onset and diagnosis: 242±650 days). The most frequent manifestations at onset were: arthralgia (93.0%), fever (92.6%), leukocytosis (89.0%), typical rash (67.7%), sore throat (61.8%), lymphadenopathy/splenomegaly (60.4%), increased liver enzymes (53.5%). In 56.4% of patients ferritin was increased more than 5 times the normal threshold. Fever intensity was recorded up to 41 ° C (mean 39.1±0.7), with a single daily peak in 27.3% of cases and 2 peaks in 51.9% of cases. The average duration of febrile episodes was 10.3 days with variable interval between episodes (average 3.2 days). Based on clinical evolution the disease was classified polycyclic systemic in 40.8%, chronic articular polycyclicin 30.7%, systemic monocyclic in 23.9% and chronic articular monocyclic in 4.6%. In 21.9% of cases, corticosteroids and traditional DMARDs have not been able to control the disease while biological drugs have been shown to be effective. 56 cycles of biological drugs were used in 51 patients: anakinra in 22 cases, etanercept in 15 cases, infliximab in 8 cases, adalimumab in 7 cases, rituximab in 3 cases, tocilizumab in 2 cases, abatacept and golimumab in 1 case each (anti- TNFalpha 52.5%, 37.3% anakinra, other 10.2%).

Conclusions This study presents the largest Italian multicentre series of AOSD patients. The main clinical and laboratory characteristics are in line with those reported in the literature. In more than 20% of patients biologics are the only drugs that allow to control the disease.

Disclosure of Interest : None declared

DOI 10.1136/annrheumdis-2014-eular.5300

Statistics from Altmetric.com

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.