Judith Sánchez-Manubens

Diagnosis and classification of Kawasaki disease.

Kawasaki disease is an acute systemic vasculitis of unknown etiology. Diagnosis is based on clinical criteria that include fever, exanthema, conjunctivitis, changes in the extremities, erythema of oral mucosa and lips and cervical lymphadenopathy. However, these criteria have low sensitivity and specificity and therefore, other clinical and laboratory features may be helpful in establishing the diagnosis, especially for cases of atypical or incomplete Kawasaki disease. Prognosis depends on the extent of cardiac involvement; coronary aneurysms develop in 20-25% of untreated patients and these may lead to myocardial infarction and sudden death. Treatment with high-dose intravenous immunoglobulin is effective in reducing the risk of coronary aneurysms in most cases and is the treatment of choice for initial Kawasaki disease.

Incidence and clinical features of kawasaki disease in Catalonia (Spain)

between onset of the disease and diagnostic was 7.2±5.3 days. Ethnic distribution was: Caucasian 279 patients(69.9%), North African 26 (6.5%), Amerindian 21 (5.2%), Asian 14 (3.5%) and Sub-Saharan 4 (1%). Ethnicity was not available in 55 (13.8%) patients. Distribution of classical manifestations for KD was: fever in 100% of patients, changes in extremities 40.3% (desquamation in 31% of them), exanthema 84.2%, conjunctival injection 79.7%, changes in lips and oral cavity 55.6% and lymphadenopathy 28.8%. Other clinical findings reported were: sterile pyuria in 80(20%) patients, nausea and vomiting in 96(24%), abdominal pain in 85(21.3%), gallbladder distention in 14 (3.5%), transaminase elevation in 120(30%), jaundice in 21(5.1%), irritability in 118(29.5%), aseptic meningitis in 16(4%), sensorineural hearing loss in 2 patients, uveitis in 11(2.7%) and arthritis or arthralgia in 55(13.8%). Cardiologic findings were: perivascular brightness of the coronary wall in 42(10.5%) patients, pericarditis in 9(2.3%), myocarditis in 4(1%), mitral regurgitation in 28 (7%) and CA in 53 patients(13.3%), 26(49%) of them disappearing before the 2 nd month after the onset of KD. 4 patient had giant CA. Intravenous immunoglobulin (IVIG) was administered in 389(97.5%) patients with response to the 1 st dose in 332(83.2%). Day of IVIG administration was 7.5±3.1. Other treatment plans were: 2 nd (69% response) and 3 rd IVIG doses, oral or iv corticosteroids and abciximab (administered in 3 of the patients with giant CA). 97.7% of patients received anti-platelet dose aspirin in the convalescent phase. Conclusion

Kawasaki disease in infants 3 months of age and younger: a multicentre Spanish study

Kawasaki disease (KD) is a multisystem vasculitis of small and medium vessels typical of childhood. Timely treatment with intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery abnormalities (CAAs) from 25% to approximately 4%.1 Asian studies have focused on infants under 3 months of age, but there are no published data about these patients from Western countries.2 3 We reviewed 621 patients under 16 years old with a diagnosis of KD between 2011 and 2016 from a multicentre study in Spain (KAWA-RACE study); 84 hospitals participated throughout the country. We found seven children under 90 days (1.13%), with a male predominance (6 of 7). Five presented irritability, but only two fulfilled the criteria for complete KD (table 1).1 The following were the main laboratory findings (median, IQR): highest C reactive protein (CRP) 24 mg/L (8.48–31.4), highest erythrocyte sedimentation rate 79 mm (70–105.5), maximum and minimum platelet count 900 ×10^9/L (682–1 117) and 506 ×10^9/L (449–612), minimum haemoglobin 10 g/dL (9–10.8), maximum leucocytes 21 ×10^9/L (16.45–23.37), minimum sodium 135.5 mEq/L (133–137.5), and minimum albumin 2.9 mg/dL (2.6–3.4). View this table: Table 1 Diagnosis, symptoms, cardiological and microbiological findings In three cases, a viral infection was diagnosed and four patients presented with CAA, but no other echocardiographic findings were detected (table 1). The median time interval since fever onset to IVIG administration was 8 days. All patients responded well to the first dose of IVIG, and only one received concomitant intravenous steroids because he was considered to be at high risk for IVIG resistance. All CAAs were transient and resolved during follow-up (table 1). Epidemiology is different in Western countries when compared with Asian …

Consenso nacional sobre diagnóstico, tratamiento y seguimiento cardiológico de la enfermedad de Kawasaki

Kawasaki disease is a self-limiting acute vasculitis that affects small and medium-sized vessels, and is the most common cause of acquired heart disease in children in our environment. Up to 25% of untreated patients develop coronary aneurysms. It is suspected that an infectious agent may be the trigger of the disease, but the causative agent is still unknown. Based on the previous evidence, recommendations are proposed for the diagnosis, treatment of acute disease, and the long-term management of these patients, in order to unify criteria. The diagnosis must be quick, based on easy-to-use algorithms and with the support of complementary tests. This document includes the indication of available imaging techniques, as well as the planning of cardiological examinations based on the initial involvement. Intravenous immunoglobulin is the basis of the initial treatment. The role of corticosteroids is still controversial, but there are studies that support its use as adjuvant treatment. A multidisciplinary working group has developed a scheme with different treatment guidelines depending on the risk factors at diagnosis, the patients clinical situation, and response to previous treatment, including indications for thromboprophylaxis in patients with coronary involvement. The stratification of risk for long-term treatment is essential, as well as the recommendations on the procedures based on the initial cardiological involvement and its progression. Patients with coronary aneurysms require continuous and uninterrupted cardiological monitoring for life.

Infecciones previas o coincidentes con la sospecha de enfermedad de Kawasaki ¿debemos cambiar nuestra actitud?

INTRODUCTION Kawasaki disease (KD) is a multisystem vasculitis associated with coronary artery abnormalities. Infections could be a trigger of the inflammation. The main aim of this study was to describe the presence of infections in children with KD, and to analyse the clinical characteristics and the presence of coronary abnormalities in these cases. PATIENTS AND METHODS A retrospective study was performed within the Kawasaki Diseases Network (KAWA-RACE (2011-2016). An analysis was performed that included patients with positive microbiological findings (PMF) during the acute phase, as well as those with a previous recent infection (PRI) during the 4 weeks preceding KD diagnosis. RESULTS The study included total of 621 children with KD, with PMF being found in 101 (16.3%) patients, and a PRI in 107 (17.2%). Significantly less echocardiographic abnormalities were found in the in the group with a PRI, when compared to those without a PRI (23 vs. 35%, P=.01) and also a lower proportion of overall coronary artery lesions (16 vs. 25%, P=.054). No significant differences were found in the proportion of aneurysms in either of these groups (PRI or PMF) when compared to those without infection. CONCLUSIONS In the present study, no differences were found in the incidence of coronary aneurysms in either of the groups, with or without PRI or PMF. Therefore, if KD is suspected, appropriate treatment should be started despite having a confirmed infection.

Efficacy and safety of TNF-alpha antagonists in children with juvenile idiopathic arthritis who started treatment under 4 years of age

The experience in the use of tumour necrosis factor (TNF) antagonists in children below 4 years is limited, although there are some trials in the literature which support safety and efficacy under this age.

Prospective analysis of Kawasaki disease cases in Catalonia (Spain) from March 2013 to March 2014.

Kawasaki disease (KD) is an acute self-limited systemic vasculitis relatively common in childhood. In Japan, last published survey shows an incidence up to 239.6/105 children <5 years old (yo). In Madrid (Spain) a retrospective study with no well defined reference area showed an incidence of 15.1/105 children <5yo.

PReS-FINAL-1014: Role of MHC class I overexpression on muscle biopsy of patients with juvenile dermatomyositis

Juvenile Dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in childhood, a systemic vasculopathy that usually affects skin and skeletal muscle but also can affect gastrointestinal tract and other organs. Diagnosis is based on Bohan and Peters criteria and the goals of treatment include control of skin and muscle symptoms and prevention of disease complications. Stepwise aggressive treatment decreases JDM activity and improves long-term outcome. Muscle involvement in JDM can be assessed by electromyography (EMG), magnetic resonance imaging (MRI) and/or muscle biopsy. Muscle biopsy assesses the presence of lymphocytic inflammatory infiltrate, perifascicular atrophy, muscle fibers necrosis and it allows studying the overexpression of major histocompatibility complex (MHC) class I in the sarcolemma and sarcoplasm of the muscle cell. In healthy muscles, there is no MHC class I expression but in inflammatory myopathies there is a distinctive and generalized overexpression, not limited to the affected areas, it appears before the inflammatory infiltrate occurs and it is not modified by immunosuppressive treatment.