Abstract
Objective: Describe the frequency of juvenile onset rheumatologic inflammatory diseases (JIR ) in level III and IV complexity level hospitals in a quinquennium. Methods: Multicentric, descriptive and retrospective study, using CI E-10 coding of hospitals’ files in Asunción and Central Department. Results: Four hospitalary records were included. 382 patients followed inclusion criteria. Sex ratio (M:F) of the population was 1:1,6, with an average age at captation of 11.6 years (SD±4.5). Kawsaki’s disease group has an average age of 5.4 years (SD±3.7) and patients with systemic lupus erythematosus (SLE) were presented with 13.1 years on average (SD±3.9). The 68,6% of patients were found through follow up specialized consults. Idiopathic juvenile arthritis was the most frequently diagnosed disease (n=167 cases, 43,7% of the total), followed by SLE (n=130, 34,0%). Most of the cases (n=290, 75,9%) were patients from (place of birth) Asuncion or Central Department. Conclusion: The spectrum of JIR diseases is broad with variable distribution in each included hospital. This study orients to know the burden of disease from JIR diseases in Paraguay.References
(1) McGonagle D, McDermott MF. A Proposed Classification of the I mmunological Diseases. PLoS Med [Internet]. 2006 Aug [cited 2013 Sep 29];3(8). Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1564298/
(2) Hedrich CM. Shaping the spectrum - From autoinflammation to autoimmunity. Clin Immunol Orlando Fla. 2016 Apr;165:21–8.
(3) R avelli A, Martini A. Juvenile idiopathic arthritis. Lancet Lond Engl. 2007 Mar3;369(9563):767–78.
(4) Petty RE, Southwood TR, Manners P, Baum J, Glass DN, Goldenberg J, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004 Feb;31(2):390–2.
(5) Federici S, Sormani MP, Ozen S, Lachmann HJ, Amaryan G, Woo P, et al. Evidencebased provisional clinical classification criteria for autoinflammatory periodic fevers. Ann Rheum Dis. 2015 May;74(5):799–805.
(6) Sarrabay G, Touitou I. Autoinflammation. Management of hereditary recurrent fevers- SHARE experience. Nat Rev Rheumatol. 2015 Oct;11(10):567–9.
(7) Brunner HI, Gladman DD, Ibañez D, Urowitz MD, Silverman ED. Difference in disease features between childhood-onset and adultonset systemic lupus erythematosus. Arthritis Rheum. 2008 février; 58(2):556–62.
(8) Klein-Gitelman M, Reiff A, Silverman ED. Systemic lupus erythematosus in childhood. Rheum Dis Clin North Am. 2002 Aug;28(3):561–577, vi–vii.
(9) Masters SL, Simon A, Aksentijevich I, Kastner DL. Horror autoinflammaticus: the molecular pathophysiology of autoinflammatory disease (*). Annu Rev Immunol. 2009;27:621–68.
(10) Hinze C, Gohar F, Foell D. Management of juvenile idiopathic arthritis: hitting the target. Nat Rev Rheumatol. 2015 May;11(5):290–300.
(11) Murray CJL, Lopez AD. Measuring the Global Burden of Disease. N Engl J Med. 2013 Aug;369(5):448–57.
(12) Alum JNM, Bejarano MSC de. Sistema de salud de Paraguay. Rev Salud Pública Parag. 2013 Nov 30;1(1):13–25.
(13) N ew CIOMS International Ethical Guidelines now available [Internet]. [cited 2017 Mar 22]. Available from: http://www.cioms.ch/index.php/12-newsflash/403-new-ciomsinternational-ethical-guidelines-now-available
(14) Murray CJL, Lopez AD. Measuring global health: motivation and evolution of the Global Burden of Disease Study. The Lancet. 2017 Sep;390(10100):1460–4.
(15) Guillén MC. Paraguay. Sistemas de salud en Sudamérica: desafíos hacia la integralidad y equidad [Internet]. 2011. Available from: http://www.mspbs.gov.py/planificacion/wp-content/uploads/2012/07/SSSPARAGU AY-2011.pdf
(16). Abreu, P. Biobadaguay: controlando la seguridad del tratamiento coagentes biológicos. rpr. 2016 Diciembre;2(2):62:63.
(17). M. Franco et al. Artropatias inflamatorias. rpr. 2017 Jul;3 (Suplemento 2):1:2.
(18) Alonso Ruiz A, Vidal Fuentes J, Tornero Molina J, Carbonell Abelló J, Lázaro P, Mercado de, et al. Estándares de calidad asistencial en reumatología. Reumatol Clínica. 2007 Sep;3(5):218–25.