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SAT0454 The Retrospective Analysis of Patients with Kawasaki Disease from Three Pediatric Rheumatology Centers in Poland
  1. D. Gorczyca1,
  2. J. Postępski2,
  3. M. Lubieniecka1,
  4. E. Olesińska2,
  5. I. Lachór-Motyka3,
  6. V. Opoka-Winiarska2,
  7. A. Emeryk2,
  8. A. Gruenpeter3
  1. 13rd Department and Clinic of Pediatrics, Immunology and Rheumatology of Developmental Age, Medical University, Wrocław
  2. 2Department of Pediatric Pulmonology and Rheumatology, Medical University, Lublin
  3. 3Department of Pediatric Rheumatology, John Paul II Pediatric Center, Sosnowiec, Poland

Abstract

Background Kawasaki Disease (KD) is one of the most common vasculitides of childhood. The diagnosis of Complete KD (C-KD) requires the presence of fever lasting at least 5 days without any other explanation combined with at least 4 of 5 following criteria: conjunctival injection, oral mucous membrane changes, peripheral extremity changes, rash, cervical lymphadenopathy. Incomplete Kawasaki (IC-KD) is diagnosed in patients who do not fulfill the classic criteria and present three or fewer of the five typical findings.

Objectives To determine incidence of KD and to evaluate its presenting symptoms, clinical course, particularly cardiovascular involvement, laboratory tests and treatment in patients with C-KD and IC-KD.

Methods The retrospective analysis of patients discharged with a diagnosis of KD at three pediatric rheumatology centers in Poland. The medical records of all inpatients diagnosed KD from 1.01.2011 to 31.12.2012 were reviewed.

Results A total of 27 Caucasians children with median age 3y (range 4 months – 12 years) (M/F:12/15) were included in this study. The incidence of C-KD vs. IC-KD was 17/27(63%) vs. 10/27 (37%) respectively. 2/10 (20 %) patients with IC-KD presented only two classical signs, 8/10 (80%) patients presented three classical KD signs. There were no differences between patients with C-KD and IC-KD in age, clinical symptoms, coronary artery abnormalities, and laboratory investigation. Patients with IC-KD significantly rarely presented cervical lymphadenopathy (20.0 % vs 88.2 %; p=0.00075), changes in extremities (30.0 % vs. 76.5 %; p=0.04), bilateral nonpurulent conjunctivitis (60.0 % vs. 100.0 %; p=0.01). Other clinical signs frequently noted in C-KD vs. IC-KD patients: gastrointestinal symptoms (58,8 % vs. 80%), hepatic dysfunction (64,7% vs. 40%), arthalgia and/or arthritis (47% vs. 40%), sterile pyuria (35,3% vs. 20%%) respectively. Cardiac assessments show that the majority of patients with KD have not coronary artery aneurysms (CAA). CAA were noted in C-KD vs. IC-KD patients: small - 23,5% vs. 30%, large - 5,9% vs. 10% respectively. All patients were treated with IVIG and ASA in typical regime doses. The median time from symptoms onset to IVIG infusion was 7 days for C-KD and 11 days for IC-KD. IVIG delay in the IC-KD had no effect on the incidence of CAA.

Conclusions There were no differences in demographic features, age of onset, and laboratory tests of patients with C-KD and IC-KD. Patients with IC-KD significantly rarely presented cervical lymphadenopathy, changes in extremities, bilateral nonpurulent conjunctivitis. Despite the fact that incomplete forms of presentation often delays diagnosis, in most patients treatment with IVIG can avoid complication of CAA.

Disclosure of Interest None Declared

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