scholarly journals High risk of coronary artery aneurysm in Kawasaki disease

Rheumatology ◽  
2020 ◽  
Author(s):  
Maria Mossberg ◽  
Aladdin J Mohammad ◽  
Fredrik Kahn ◽  
Mårten Segelmark ◽  
Robin Kahn
Keyword(s):  
Coronary Artery ◽  
High Risk ◽  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Demographic Data ◽  
Artery Aneurysm ◽  
Heart Association ◽  
Coronary Aneurysms ◽  
Immunoglobulin Treatment ◽  
Timely Treatment

Abstract Objective Kawasaki disease (KD) is a vasculitis of unknown aetiology with a high risk of coronary aneurysms if untreated. Timely treatment with intravenous immunoglobulin decreases the risk for coronary artery aneurysms (CAA). In this study, we set out to elucidate the factors associated with the risk of developing CAA. Methods Records of all KD-diagnosed children in Skåne between 2004 and 2014 were collected and clinical and demographic data were compiled. KD is defined according to the revised American Heart Association diagnostic criteria and classified as either complete KD (cKD) or incomplete KD (iKD). Results KD was diagnosed in 77 children and CAA was found in 31% (n = 24). Children with CAA were younger compared with children without (median; 20 vs 34 months) and intravenous immunoglobulin treatment within 10 days was less likely to be received (75% vs 91%). In children presenting with iKD, 47% developed CAA compared with 21% in cKD patients. Using multivariate analysis, an association between the risk of CAA with low age in children with iKD was observed. Conclusion The risk of CAA development is disturbingly high in young children with iKD. This highlights the importance of rapid intense treatment and vigilance in infants, who are the most difficult to diagnose, in order to reduce the frequency of CAA.

scholarly journals Corticosteroids Added to Initial Intravenous Immunoglobulin Treatment for the Prevention of Coronary Artery Abnormalities in High‐Risk Patients With Kawasaki Disease

2020 ◽  
Vol 9 (17) ◽  
Author(s):  
Ryusuke Ae ◽  
Joseph Y. Abrams ◽  
Ryan A. Maddox ◽  
Lawrence B. Schonberger ◽  
Yosikazu Nakamura ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Treatment Group ◽  
High Risk ◽  
Kawasaki Disease ◽  
Treatment Failure ◽  
Intravenous Immunoglobulin ◽  
Combination Treatment ◽  
Coronary Artery Abnormalities ◽  
Estimated Risk ◽  
Immunoglobulin Treatment

BACKGROUND Randomized controlled trials previously provided different conclusions about the superiority of adding corticosteroids to initial intravenous immunoglobulin treatment for the prevention of coronary artery abnormalities in patients with Kawasaki disease (KD). To further assess this issue, we analyzed large‐scale data from nationwide KD surveys in Japan, where combination treatment (corticosteroids added to initial standard intravenous immunoglobulin treatment) has become commonly used for patients at high risk for KD. METHODS AND RESULTS Standard intravenous immunoglobulin treatment and combination treatment were compared using data from time periods with and without combination treatment. Outcome measures were coronary artery abnormalities and initial intravenous immunoglobulin treatment failure. Hospitals where ≥20% of patients received combination treatment were identified, and treatment and control groups were selected via matching by age, sex, illness day at initial treatment, and KD recurrence. Matched group selection and subsequent analyses were conducted 1000 times to minimize sampling bias and potential confounders (bootstrapping). From 115 hospitals, 1593 patients with KD in the treatment group and 1593 controls were selected for each of the 1000 sample iterations. The median proportion of patients who developed coronary artery abnormalities among the treatment group and controls were 4.6% (95% CI, 3.8%–5.8%) and 8.8% (95% CI, 7.5%–10.0%), respectively: an estimated risk ratio of 0.53 (0.41–0.67). A median of 14.1% (95% CI, 12.4%–15.9%) of the patients in the treatment group and 21.7% (95% CI, 19.8%–23.4%) in the controls had treatment failure: an estimated risk ratio of 0.65 (0.56―0.75). CONCLUSIONS Combination treatment reduced coronary artery abnormality risk by an estimated 47% and treatment failure by 35%. Multiple‐dose corticosteroids may provide benefit in selected patients at high risk for KD.

Stratified therapy for Kawasaki disease using a new scoring system to predict the response to a lower dose of intravenous immunoglobulin therapy

2021 ◽  
pp. 1-5
Author(s):  
Misa Matsuura ◽  
Daisuke Sugawara ◽  
Eishi Makita ◽  
Yuka Hirakubo ◽  
Kazuhito Nonaka ◽  
...  
Keyword(s):  
Coronary Artery ◽  
High Risk ◽  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Risk Score ◽  
Risk Group ◽  
Immunoglobulin Therapy ◽  
Low Risk ◽  
Immunoglobulin Treatment ◽  
New Scoring

Abstract Background: Several studies have reported treatment options for patients with Kawasaki disease refractory to standard immunoglobulin therapy; however, no studies have reported low-dose immunoglobulin therapy for patients with a low risk of Kawasaki disease. Methods: A total of 277 patients with Kawasaki disease were included in this study. We used Kobayashi score and our Less high-risk score to divide the patients into three groups. Patients in the high-risk group (Kobayashi score ≥ 5 points) received 2 g/kg intravenous immunoglobulin and prednisolone. Patients in the moderate-risk group (Kobayashi score < 5 points and Less high-risk score ≥ 2 points) received 2 g/kg intravenous immunoglobulin treatment. Patients in the low-risk group (Kobayashi score < 5 points and Less high-risk score < 2 points) received 1 g/kg intravenous immunoglobulin treatment. The response rate and the incidence of coronary artery lesions at 4 weeks after treatment were evaluated in each group. Results: The treatment response rates in the high-risk (n = 110), moderate-risk (n = 80), and low-risk (n = 87) groups were 74.5, 72.5, and 77.0%, respectively. Coronary artery lesions occurred in 7.3, 3.8, and 2.3% of patients in the high-, moderate-, and low-risk groups, respectively. There were no significant differences between the groups regarding treatment response or coronary artery lesion rate. Conclusion: The therapeutic response rate and the therapeutic effect of low-dose intravenous immunoglobulin in the low-risk group identified with our new scoring were satisfactory. Stratified therapies for patients with Kawasaki disease based on the scoring system may be useful.

Usefulness and safety of anakinra in refractory Kawasaki disease complicated by coronary artery aneurysm

2018 ◽  
Vol 28 (5) ◽  
pp. 739-742 ◽  
Author(s):  
Marie-Paule Guillaume ◽  
Héloïse Reumaux ◽  
François Dubos
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Coronary Arteries ◽  
Coronary Artery Aneurysm ◽  
Artery Aneurysm ◽  
Intravenous Immunoglobulins ◽  
Unknown Aetiology ◽  
Partial Regression ◽  
Coronary Aneurysms

AbstractKawasaki disease is an acute self-limited vasculitis of unknown aetiology. The prognosis depends mainly on coronary damage. There is no consensus regarding optimal adjunctive therapeutics for refractory forms to treatment by intravenous immunoglobulins and corticosteroids. We report the case of an 18-month-old infant with refractory Kawasaki disease complicated by diffuse aneurysms of coronary arteries and successfully treated by anakinra with partial regression of coronary aneurysms

Abstract 137: Efficacy Of Raise Protocol For Children With Incomplete Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Kazuyuki Naoi ◽  
Yuji Asami ◽  
Shinichi Takatsuki ◽  
Mitsuru Seki ◽  
Satoshi Ikehara ◽  
...  
Keyword(s):  
Coronary Artery ◽  
High Risk ◽  
Kawasaki Disease ◽  
Initial Treatment ◽  
Risk Group ◽  
Artery Aneurysm ◽  
Pulse Therapy ◽  
High Risk Group ◽  
Low Risk ◽  
Steroid Pulse

Background: Although RAISE study demonstrated that intravenous immunoglobulins (IVIG) plus prednisolone (PSL) therapy for refractory Kawasaki disease (KD) reduced coronary artery aneurysm, the study population included a few incomplete KD. The aim of study was to evaluate the efficacy of RAISE protocol for incomplete KD. Methods: Children with incomplete KD which have 4 or less major symptoms of KD were enrolled. Using Kobayashi score, children were divided into low and high risk for IVIG resistance. Children with low risk group received IVIG monotherapy, whereas IVIG plus PSL therapy administrated for high risk group. Retrospectively, we assessed initial treatment response and coronary artery abnormalities (CAAs). Results: Overall, 63 incomplete KD (age; median and range 17 months (1-115 months), sex ratio; boys:girls 37:26) were enrolled. Median day of illness at diagnosis was 5 day of illness (2-10 days). Low risk group included 52 cases (83%) and the remaining 11 cases were high risk group. In low risk group, 87% of children (45 cases) were initial treatment responders. All 7 non-responders were responded to additional methylprednisolone steroid pulse therapy. All 11 children with high risk group were responders to the initial treatment. Five cases have equal to greater than 2.5 of Z-Score, which were all low risk group. All CAAs regressed to normal coronary diameter within 1 month. Conclusions: RAISE protocol was useful for the treatment of incomplete KD without any CAAs.

Abstract 103: Incomplete Kawasaki Disease - Patients With ≦ 5 Days Of Fever Successfully Treated By Antibiotics But Left With Coronary Aneurysms -

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Kenji Suda ◽  
Hiroshi Nishino ◽  
Yoshiyuki Kudo ◽  
Hironaga Yoshimoto ◽  
Shintaro Kishimoto ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Case Series ◽  
Artery Aneurysm ◽  
Ivig Treatment ◽  
Incomplete Kawasaki Disease ◽  
Antithrombotic Treatment ◽  
Coronary Aneurysms ◽  
Appropriate Treatment ◽  
Echocardiographic Examination

Objective: We report four patients with incomplete Kawasaki disease (KD) successfully treated by antibiotics without intravenous immunoglobulin (IVIG) treatment with ≦ 5 days of fever but left with coronary artery lesion. Result: The patients were 2 babies and 2 young children age ranged from 2 months to 2 years old and 9 months. They showed fever and other signs of KD, but did not fulfill diagnostic criteria. The numbers of symptoms compatible with KD were 2 or 3 and included conjunctival injection, oral or lip injection, and eruption. Within 5 days, they became afebrile by antibiotics without IVIG treatment. However, they were noted to have coronary artery aneurysm (CAA) on day from 7 to 33 of illness. All patients had been placed on antithrombotic treatment including aspirin or aspirin plus warfarin and, fortunately, showed regression of CAA within 2 years. Conclusions: This case series indicates that there are patients with incomplete KD successfully treated by antibiotics without IVIG but left with CAA. These cases must be underdiagnosed if intentional echocardiographic examination are scheduled and might be left without appropriate treatment, antithrombotic treatment. Further collection of data of these patients is indispensable.

scholarly journals 3,4-Methylenedioxymethamphetamine (MDMA, Ecstasy) Intoxication in a Young Adult with Giant Coronary Aneurysms from Kawasaki Disease

2018 ◽  
Vol 8 (3) ◽  
pp. 752-762 ◽  
Author(s):  
Renee Eigsti ◽  
Dennis J. Firchau ◽  
Marcus B. Nashelsky
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Left Ventricle ◽  
Coronary Arteries ◽  
Coronary Artery Spasm ◽  
Artery Aneurysm ◽  
Unknown Etiology ◽  
Stress Tests ◽  
Coronary Aneurysms ◽  
Increased Risk

Kawasaki disease is an acute vasculitis of unknown etiology that usually occurs in children less than five years old. Coronary artery aneurysm due to arteritis is the most common cardiac complication. Chronic aneurysms are associated with an increased risk of developing luminal thrombosis and ischemic myocardial injury. We present a case of an 18-year-old male with a history of Kawasaki disease complicated by chronic giant aneurysms of the right and left coronary arteries. Serial echocardiographic studies and treadmill electrocardiogram stress tests as recent as 1.5 years before death revealed excellent cardiac function by clinical criteria. The decedent sustained a witnessed collapse after ingesting 3,4-methylenedioxymethamphetamine (MDMA) and ethanol. He was pronounced dead in the emergency department after unsuccessful resuscitative efforts. Autopsy revealed large aneurysms of the three main epicardial coronary arteries with extensive foci of severe stenosis by intimal fibrosis and organizing thrombus. Microscopic examination revealed multifocal severe myocyte hypertrophy. There were remote microinfarcts in the anterior and posterior aspects of the left ventricle and a recent, healing microinfarct in the posterior aspect of the left ventricle. Toxicology examination of postmortem femoral blood revealed MDMA, ethanol, and amiodarone. This case illustrates the residual, lasting effects of cardiac disease due to Kawasaki disease and a potential complication in the setting of use of an illicit stimulant, MDMA, an amphetamine derivative that produces sympathetic activation and cardiovascular effects including tachycardia, vasoconstriction, dysrhythmias, and coronary artery spasm. Kawasaki disease-related abnormalities of the heart likely resulted in a lower threshold for developing a fatal cardiac dysrhythmia under the circumstance of stimulant use.

A meta-analysis of re-treatment for intravenous immunoglobulin-resistant Kawasaki disease

2015 ◽  
Vol 25 (6) ◽  
pp. 1182-1190 ◽  
Author(s):  
Xi Yang ◽  
Guiying Liu ◽  
Yaqian Huang ◽  
Stella Chen ◽  
Junbao Du ◽  
...  
Keyword(s):  
Drug Therapy ◽  
Coronary Artery ◽  
Body Temperature ◽  
Kawasaki Disease ◽  
Confidence Interval ◽  
Intravenous Immunoglobulin ◽  
Odds Ratio ◽  
Meta Analysis ◽  
Coronary Artery Lesions ◽  
Immunoglobulin Treatment

AbstractObjectiveTo determine the optimal drug therapy for intravenous immunoglobulin-resistant Kawasaki disease.MethodsStudies regarding drug therapy for intravenous immunoglobulin-resistant Kawasaki disease were selected from medical electronic databases including PubMed, Medline, Elsevier, and Springer Link. The effectiveness in terms of temperature recovery and coronary artery damage was compared between a second intravenous immunoglobulin treatment and glucocorticosteroid treatment for children with intravenous immunoglobulin-resistant Kawasaki disease using meta-analysis with Review Manager 5.3 software. Indices to evaluate the effects were body temperature, biomarker levels, and coronary artery lesions detected by echocardiography. Results are reported as relative risks or odds ratio with a 95% confidence interval and p<0.05.ResultsMeta-analysis included 52 patients in the second intravenous immunoglobulin treatment group and 75 patients in the glucocorticosteroid treatment control group from four studies that met our inclusion criteria. Temperatures of patients who received glucocorticosteroid treatment were effectively controlled compared with those who received a second intravenous immunoglobulin treatment (relative risk=0.73, 95% confidence interval: 0.58–0.92, p=0.007). There were no differences, however, in the incidence of coronary artery lesions between the two groups (odds ratio=1.55, 95% confidence interval: 0.57–4.20, p=0.39).ConclusionsGlucocorticosteroids are more effective in controlling body temperature compared with intravenous immunoglobulin re-treatment in intravenous immunoglobulin-resistant Kawasaki disease children; however, glucocorticosteroids and intravenous immunoglobulin re-treatment showed no difference in the prevention of coronary artery lesions.

scholarly journals The Occurrence of Coronary Artery Lesions in Kawasaki Disease Based on C-reactive Protein Levels: A Retrospective Cohort Study

2020 ◽  
Author(s):  
Hyo Soon An ◽  
Gi-Beom Kim ◽  
Mi Kyoung Song ◽  
Sang Yun Lee ◽  
Hye Won Kwon ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Demographic Data ◽  
Artery Aneurysm ◽  
Nationwide Survey ◽  
Coronary Artery Lesions ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
Significant Difference ◽  
Z Scores

Abstract BackgroundThis study aimed to assess the occurrence of coronary artery lesions (CAL) in patients with Kawasaki disease (KD) according to serum C-reactive protein (CRP) levels. MethodsThis retrospective analysis was based on the nationwide survey of KD conducted in the Republic of Korea between 2015 and 2017. We enrolled 9131 patients and defined low (<3 mg/dL) and high (≥3 mg/dL) CRP groups. Demographic data, clinical characteristics, z-scores, and scores based on the Japanese criteria for CAL were compared between the two groups. Logistic regression analysis was used to identify CAL risk factors.ResultsThe low CRP group accounted for 23% of patients. A significant difference was observed for the mean age at diagnosis (high vs. low CRP, 34.4 ± 24.9 vs. 31.7 ± 24.8 months, p<0.001) and fever duration (high vs. low CRP, 6.6 ± 2.2 vs. 6.3 ± 2.5 days, p<0.001). A non-response to intravenous immunoglobulin treatment was found in 1377 patients (20.1%) and 225 patients (11.7%) in the high and low CRP groups, respectively (p<0.001). CAL were found in 12.9% and 18.3% of the high and low CRP patients, respectively (p<0.001), based on z-scores; and in 9.9% and 12.5%, respectively (p = 0.001), based on the Japanese criteria in the acute phase. The giant coronary artery aneurysm occurrence ratio was similar between groups (p=1.0).ConclusionsCAL occurred in patients with both high and low CRP. Therefore, patients with KD should be carefully monitored regardless of their CRP levels.

Abstract O.44: Infliximab For Kawasaki Disease Patients Who Did Not Respond To The Initial Therapy : A Japanese Nationwide Surveillance

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Tohru Kobayashi ◽  
Shinichi Takatsuki ◽  
Tsutomu Saji ◽  
Tomoyoshi Sonobe ◽  
Shunichi Ogawa ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Initial Treatment ◽  
Coronary Artery Aneurysm ◽  
Demographic Data ◽  
Age At Onset ◽  
Artery Aneurysm ◽  
Initial Therapy ◽  
Additional Treatment ◽  
Nationwide Surveillance

Backgrounds: In Japan, infliximab (IFX) has been used in patients with Kawasaki disease (KD) who did not respond to initial treatment. However, nationwide surveillance of the IFX for KD patients has not been reported. The objective of the present study is to reveal epidemiological and clinical features of KD patients treated with IFX. Method: This retrospective observational study was conducted from 2007 to 2013. We sent a questionnaire to members of Japanese Society of Kawasaki disease annually and collected demographic data and clinical outcomes of KD patients treated with IFX. A total of 315 patients (208 boys and 107 girls, age at onset 37±25 months, illness days of initial treatment 4.3±1.2) from 46 hospitals were identified and analyzed in the present study. Results: No patients received IFX as initial treatment. Over 60% of the patients were treated with IFX as first or second line additional rescue treatment. After the IFX administration, 237 patients (75.7%) became afebrile within 24 hours and 82 patients (26.0%) treated additional rescue treatments subsequently. Before IFX administration, 55 patients already had coronary artery aneurysm exceeding 4mm and 69 patients were finally affected coronary artery aneurysm exceeding 4mm. Serum level of C reactive protein were significantly decreased after IFX administration (10.6±7.2 to 4.9±4.7, P<0.001). Patients who required additional treatment after IFX showed significantly higher CRP levels before IFX compared to patients without additional treatment after IFX (12.3±8.3 vs. 10.0±6.7, P=0.049). Adverse events were observed in 50 patients (15.9%) which recovered spontaneously or after interventions. Conclusions: IFX therapy for Kawasaki disease patients who did not respond to the initial treatment would be well tolerated and play a certain role to improve clinical course.

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