SAT0506 KAWASAKI DISEASE AND CARDIOVASCULAR OUTCOMES IN THE LAST 25 YEARS. STUDY OF 55 PATIENTS FROM A REFERAL HOSPITAL IN THE NORTH OF SPAIN
Authors: José Luis Martín-Varillas, D. Prieto-Peña, Lara Sánchez Bilbao, Eva Peña Sainz-Pardo, Belén Atienza-Mateo, Monica Calderón-Goercke, Iñigo González-Mazón, Natalia Palmou-Fontana, Maria Teresa Viadero Ubierna, María Jesús Cabero, Miguel A. González-Gay, Ricardo Blanco
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Kawasaki disease (KD) or "mucocutaneous lymph node syndrome" is a systemic vasculitis in children that involve medium-sized vessels with predilection for coronary arteries. Due to the high probability of cardiovascular complications, an early diagnosis and treatment is required. <h3>Objectives:</h3> A) To describe demographic, clinical and analytical features in a cohort of patients with KD diagnosis from northern Spain. B) To assess the rate of long-term cardiovascular outcomes. <h3>Methods:</h3> We set up an observational study of patients with KD in a University Hospital between Jan-94 and Dec-18. Classic classification criteria were used for diagnosis<sup>1</sup>. Diagnosis of aneurysms was made by serial echocardiography and/or coronary-CT. Results are expressed as mean±SD or as median and interquartile range (IQR) as appropriate. <h3>Results:</h3> 55 patients (28 women/27 men), with a mean age at diagnosis of 3.6±2.8 years. 60% presented a previous infection prior to diagnosis, most of them viral infection (<b>table 1</b>). At diagnosis, 43 patients were classified as complete KD (78.2%), presenting fever &gt;5 days (98.2%), maculopapular rash (98.2%), oral mucosal involvement (89.1%), hands and feet edema (74.5%), conjunctival hyperemia (85.5%) and cervical adenopathy (50.9%). Analytically, there was an increase in acute phase reactants, presence of leucocytosis and thrombocytosis. All patients presented a monophasic disease course with a mean duration of symptoms of 12.6±4.9 days (<b>table 2</b>). In 8 patients (14.5%) coronary aneurysms were demonstrated and pericarditis/myocarditis was observed in 2. However, after a follow-up of 13.9±7.0 months, only 1 of them maintained coronary involvement. 98.2% received intravenous immunoglobulin (IGIV) according to international recommendations (2 g/kg). All patients received anti-inflammatory doses of acetylsalicylic acid at diagnosis and subsequently antiplatelet doses. 2 required endovenous corticosteroid, and in 1, an anti-TNF drug (infliximab) was needed (<b>table 2</b>). The incidence rate of KD in our cohort was 0.37 cases/100,000/year, 8-10 times lower than other national series<sup>2</sup>. However, mean age at onset, presence of previous viral infections and aneurysms development, were similar to countries with higher prevalence<sup>3</sup>. <h3>Conclusion:</h3> Although the incidence of KD in our population is lower than other territories, it is still the most frequent cause of acquired heart disease in childhood. Early recognition and treatment with IVIG improve prognosis leading to a decrease in the rate of long-term cardiovascular outcomes. <h3>References</h3> [1] Wood LE. Expert Rev Cardiovasc Ther.2007;5:553-61. [2] Sánchez-Manubens J. Clin Exp Rheumatol 2016;34 (Suppl. S139-S144). [3] Rowley AH, et al. Front Pediatr. 2018Dec11;6:374. <h3>Disclosure of Interests:</h3> José Luis Martín-Varillas: None declared, D. Prieto-Peña: None declared, Lara Sánchez Bilbao: None declared, Eva Peña Sainz-Pardo: None declared, Belén Atienza-Mateo: None declared, Monica Calderón-Goercke: None declared, Iñigo González-Mazón: None declared, Natalia Palmou-Fontana: None declared, Maria Teresa Viadero Ubierna: None declared, María Jesús Cabero: None declared, Miguel A González-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker's bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen