Sequelae of Kawasaki disease in adolescents and young adults

Kawasaki disease is an acute vasculitis of unknown etiology that predominantly affects children <5 years of age. Structural damage to the coronary arteries after the acute, self-limited illness is detected by echocardiography in ∼25% of untreated patients. The long-term effects of the acute coronary arteritis are unknown. To define the spectrum of clinical disease in young adults that can be attributed to Kawasaki disease in childhood, we performed a retrospective survey of cases reported in the English and Japanese published data of adult coronary artery disease attributed to antecedent Kawasaki disease. The mean age at presentation with cardiac sequelae was 24.7 ± 8.4 years (range 12 to 39) for the 74 patients identified with presumed late sequelae of Kawasaki disease. Symptoms at the time of presentation with cardiac sequelae included chest pain/myocardial infarction (60.8%), arrhythmia (10.8%) and sudden death (16.2%). These symptoms were precipitated by exercise in 82% of patients. One-third of the patients in whom a chest radiograph was taken had ring calcification. Angiographic findings included coronary artery aneurysm (93.2%) and coronary artery occlusion (66.1%). Extensive development of collateral vessels was reported in 44.1% of patients. Autopsy findings included coronary artery aneurysms (100%0 and coronary artery occlusion (72.2%). The acute vasculitis of Kawasaki disease can results in coronary artery damage and rheologic changes predisposing to thrombus formation or progressive atherosclerotic changes that may remain clinically silent for many years. Coronary artery aneurysms and calcification on chest radiography were unusual features in this group of patients. A history of antecedent Kawasaki disease should be sought in all young adults who present with acute myocardial infarction or sudden death.     

Kawasaki syndrome

Kawasaki syndrome is an acute self-limited illness of young children that may result in significant cardiovascular sequelae in 20% of patients. The etiology is unknown, but clinical and epidemiologic features strongly suggest an infectious etiology. The incidence of coronary artery abnormalities has been dramatically reduced by administration of intravenous gamma-globulin in the acute phase of illness. This paper reviews the clinical, laboratory, etiologic, epidemiologic, cardiovascular, pathologic, and therapeutic aspects of Kawasaki syndrome. Discovery of the etiology of this disorder will enable more specific diagnostic tests and therapies to be developed.     

Kawasaki disease: An update

Kawasaki disease (KD) was first reported in Japan more than 30 years ago, but the original diagnostic clinical criteria defined by Dr. Kawasaki are still authentic. The cause of KD remains unknown. Several epidemiologic and clinical observations suggest that it is caused by one or multiple infectious agents, each of which can result in the clinical manifestation of the disease. Advances have been made in the management of the disease with the introduction of aspirin and intravenous immunoglobulin (IVIG) that have had a significant impact on lowering the rate of coronary artery aneurysms and death from the disease. Questions remain regarding the management of those patients whose disease does not respond to IVIG. Some patients with severe KD who are resistant to IVIG may benefit from IV pulse steroids therapy. It remains to be seen whether steroid or other anti-inflammatory agents play a role in the management of patients with KD. Further research studies are needed to identify the possible cause, define the treatment, and predict the long-term outcome of this enigmatic disease.     

Kawasaki disease and juvenile systemic lupus erythematosus

Kawasaki disease (KD) is a common vasculitis in childhood. To the authors' knowledge, only one case of juvenile systemic lupus erythematosus (JSLE)-like onset mimicking KD and another case of KD and JSLE association have previously been described. However, the prevalence of this association of the two diseases was not reported. Therefore, over 27 consecutive years, 5419 patients were followed at the Pediatric Rheumatology Unit and 271 (5%) of them met the ACR classification criteria for JSLE. Two (0.7%) of them were female. These also had KD according to European League against Rheumatism / Paediatric Rheumatology European Society (EULAR/PReS) consensus criteria and are described in this report. One case was a 13-year-old who presented all six KD criteria. Echocardiogram showed pericardial effusion, dilatation and tortuosity of right and left coronary, and her symptoms promptly improved after treatment with intravenous immunoglobulin (IVIG). Lupus diagnosis was established a few days later. Another case was a 4-year-old who had also met all six KD criteria, with improvement after IVIG, and lupus diagnosis was made 1 year later. In conclusion, the frequency of the association between these two autoimmune diseases was rare. The occurrence of a second autoimmune systemic disease in a patient with a history of KD should also be considered. Furthermore, the initial presentation of lupus may mimic KD.     

Usefulness of new diagnostic criteria for chronic hypersensitivity pneumonitis established on the basis of a Delphi survey: A Japanese cohort study

BackgroundChronic hypersensitivity pneumonitis (CHP) is a fibrotic interstitial lung disease (ILD) caused by repeated exposure to a variety of organic particles. In November 2017, new criteria for CHP diagnosis were proposed by Morisset et al. based on a modified Delphi survey of ILD experts. However, it remains unclear whether these criteria are useful to accurately diagnose CHP. We aimed to evaluate the newly proposed CHP diagnostic criteria.MethodsWe retrospectively applied Morisset's CHP diagnostic criteria to consecutive Japanese patients who underwent surgical lung biopsy for diagnosis of ILD from 2008 to 2015. All patients underwent bronchoalveolar lavage and pulmonary function testing. Patients who had connective tissue disease complications or showed an acute or subacute disease onset were excluded.ResultsA total of 251 patients were included. The diagnoses based on multidisciplinary discussion (MDD) were CHP (n = 27), idiopathic pulmonary fibrosis (n = 117), unclassifiable interstitial pneumonia (IP) (n = 65), and other diagnoses (n = 42). Of the 27 MDD-CHP patients, 14 were classified as a CHP group with diagnostic confidence >50% and 13 were not categorized (sensitivity, 51.9%; specificity, 77.7%). Morisset's CHP diagnostic criteria could help avoid SLB for the diagnosis of CHP in seven patients. Of the 13 MDD-CHP patients who were not categorized in the CHP group with diagnostic confidence >50%, the reason for the exclusion was an inconsistent with UIP pattern without CHP features.ConclusionsHalf of the MDD-CHP patients were diagnosed with CHP using Morisset's CHP diagnostic criteria. Further investigation will be important for developing improved diagnostic criteria for CHP.     

Recurrent Kawasaki Disease in a Child With Retropharyngeal Involvement: A Case Report and Literature Review

Kawasaki disease (KD) is a multisystemic vasculitic disease. Recurrent KD is rare and generally presents in a similar clinical picture as the first episode, and early diagnosis with prompt treatment is the key point in preventing associated cardiovascular morbidities.A 9-year-old boy, who was diagnosed with KD when he was 1.5 years’ old, was referred to our hospital for surgical drainage of retropharyngeal abscess. He had a 7-day history of high fever, sore throat, left-sided neck swelling, and restricted neck movements. Subsequently, he was diagnosed with recurrent KD and retropharyngeal involvement. He was successfully treated with a single dose of intravenous immunoglobulin (IVIG) and acetyl salicylic acid.Recurrence is rare and occurs most commonly in children. Atypical presentation, incomplete disease, short duration of fever, and reduced response to IVIG treatment were found to be the risk factors for recurrence. KD can occasionally present with clinical and radiographic findings of deep neck bacterial infection. Unusual presentations in KD may cause delay in diagnosis and increase the risk of life-threatening complications.We describe a case of recurrent KD presenting with a clinical picture resembling retropharyngeal infection who fully recovered after 1 dose of IVIG instead of surgical drainage and antibiotic use.     

Kawasaki disease

Opinion statement Kawasaki disease is an acute vasculitis of childhood, complicated by coronary artery aneurysms in up to 25% of affected children. High-dose intravenous immunoglobulin (IVIG) administered in the acute phase of Kawasaki disease leads to a fivefold reduction in the prevalence of coronary aneurysms. When fever persists, a second IVIG infusion should be administered. Rescue therapies for IVIG-resistant Kawasaki disease include pulsed-dose methylprednisolone or other steroid regimens, as well as infliximab, a tumor necrosis factor-α antagonist. Aspirin is used initially in an anti-inflammatory dosage, then at a low dosage until approximately 6 weeks after illness onset. Patients with coronary aneurysms require chronic antithrombotic therapy. For small aneurysms, aspirin alone is sufficient. For larger aneurysms, agents added to aspirin include clopidogrel and, for giant aneurysms, warfarin or low molecular weight heparin. Long-term management is tailored to the degree of coronary artery involvement. Evidence-based data to guide indications for transcatheter and surgical intervention are limited.     

Coronary artery aneurysms and myocardial infarction: adult sequelae of Kawasaki disease?

Coronary artery aneurysms developed in a 43 year old man who had suffered an acute myocardial infarction at the age of 30. In childhood he had had an illness that was consistent with Kawasaki disease, and it is suggested that the proximal discrete aneurysms and myocardial infarction may be the adult sequelae of this.     

Osteoprotegerin serum levels in Kawasaki disease: an additional potential marker in predicting children with coronary artery involvement

OBJECTIVE: Emerging evidence from in vitro studies and mouse genetics attributes to osteoprotegerin (OPG), a member of the tumor necrosis factor receptor family, an important role in vascular biology. We evaluated serum levels of OPG in a group of children with Kawasaki disease (KD), before immunoglobulin (IVIG) infusion and at 3-month followup. METHODS: Fifty patients (38 boys, 20 girls, median age 3.6 yrs, range 4 mo-7.4 yrs) fulfilling criteria for the diagnosis of KD, 30 febrile controls with infectious diseases, 18 patients with juvenile systemic lupus erythematosus (JSLE), and 40 healthy controls were enrolled. All KD patients received IVIG treatment within the first 10 days of illness, and aspirin. Coronary artery abnormalities (CAA) were reported in 6 out of 58 patients; all were male and younger than 5 years. Serum OPG was measured by ELISA in patients with KD before IVIG and at 3-month followup (median time 3.2 mo, range 3-3.5). RESULTS: At baseline and at the 3-month followup, KD patients had significantly higher OPG serum levels than febrile controls (p < 0.001 and p < 0.004, respectively), JSLE patients (p < 0.0001), and healthy controls (p < 0.0001). At baseline, KD patients who developed CAA had higher OPG serum levels than those without CAA (p = 0.0001); this difference was not present at 3-month followup. The optimal OPG cutoff value of 123.2 pg/ml was a significant predictor for CAA, with a sensitivity of 100% (6/6), a specificity of 96% (50/52), and a positive predictive value of 75% (6/8). CONCLUSION: High OPG levels might be the result of compensatory production during acute and subacute phases of KD. OPG assay might be an additional clinically useful marker to monitor and differentiate patients who develop, from those who do not develop, such coronary artery abnormalities.     

Coronary artery aneurysms and myocardial infarction: adult sequelae of Kawasaki disease?

Coronary artery aneurysms developed in a 43 year old man who had suffered an acute myocardial infarction at the age of 30. In childhood he had had an illness that was consistent with Kawasaki disease, and it is suggested that the proximal discrete aneurysms and myocardial infarction may be the adult sequelae of this.     

Maladie de Kawasaki de l’adulte

Kawasaki disease (KD) is a multisystemic vasculitis affecting mainly the skin, mucosa, and lymph nodes. Coronary artery aneurysms occur in 25% of patients but their prevalence is reduced to 4% in those patients treated with intravenous immunoglobulin (IVIg) within 10 days of illness onset. Interesting data recently published relate to physiopathology and diagnosis of the disease. Investigations identified an antigen-driven IgA oligoclonal response directed against cytoplasmic inclusions in KD tissues. An algorithm using laboratory tests and echocardiography has been recently proposed to improve early detection of incomplete KD. Although KD predominantly affects children, it may be also of interest for adult physicians. First, patients may develop long-term cardiovascular event. Coronary artery aneurysms may lead to the development of coronary stenosis or thrombosis. Despite the absence of coronary lesions during the acute phase of the disease, patients may present morphological and functional sequelae of coronary and peripheral arteries at convalescent phase. These potential arterial sequelae require long-term follow-up and treatment of associated cardiovascular risk factors. Although the level of injury seems to be correlated with the severity of initial coronary lesions, long-term course of vascular injuries is poorly known. Second, KD may occur in adults with 91 cases reported in the literature. Twenty-one cases have been reported in HIV infected patients. Intravenous immunoglobulins appear to shorten the disease course. Recent studies highlight the existence of incomplete KD and symptomatic coronary aneurysms in adults. Overall, these data suggest that adult patients with biological or echocardiographic features suggestive of incomplete KD should receive prompt IVIg to prevent coronary artery sequelae.     

Morbidity prevalence rate of kawasaki disease assessed by single cross-sectional history-taking

The need for long-term follow-up in Kawasaki disease is poorly recognized although cardiac sudden death attacks asymptomatic young people with past illness after a long latent period. Therefore, in order to prevent cardiac disasters, high risk groups should be identified and the prevalence rate of the disease should be determined for crisis management. A total of 9,965 consecutive freshmen at the University of Tokyo were the subject of a questionnaire. Their parents/guardians who were briefed on the diagnostic criteria of the acute phase of Kawasaki disease actually completed the questionnaire. Students with a positive diagnosis underwent rest and exercise-stress electrocardiography and routine echocardiography. The overall prevalence rate was 0.57%. The rate in males (0.63%) was greater than that in females (0.32%) (P < 0.05). Electrocardiography and routine echocardiography identified no indices specific to a past illness of Kawasaki disease. The prevalence rate indicated that about 6 in 1000 students were high risk students who needed special care while at university. Since there are few symptoms and no signs indicating a past illness of Kawasaki disease, intensive history-taking from parents/guardians who are familiar with their acute symptoms during childhood is required in order to identify those at high risk of a coronary event.     

Initial intravenous gamma-globulin treatment failure in Iranian children with Kawasaki disease

The purpose of this study was to determine the initial rates of intravenous gamma-globulin treatment (IVIG) failure in Kawasaki disease (KD) and their predisposing factors. This study was a retrospective analysis of the initial response to IVIG (2 g/kg), assessed from the medical reports of all patients admitted to Namazee Hospital pediatric ward, from March 1998 to March 2002, and who fulfilled the criteria for KD. Data were available for 64 patients, 58 of whom (90.6%) became afebrile 48 hours after completion of the initial dose of IVIG (Group I) and six (9.4%) who remained febrile (Group II). Two patients had a prompt response to a second dose of IVIG. In Group I, five patients (8.6%) developed coronary artery disease, seen on echocardiography. In Group II, two patients (33.3%) developed coronary artery disease. No significant difference was found in the prevalence of coronary artery disease between the two groups (p = 0.12), or in age or gender. The rate of initial treatment failure was 9.4% in this cohort of patients, which is comparable with previous reports. No predictive factors such as coronary artery disease, age or gender were found for initial treatment failure in KD.     

Transient heart failure in an adult with Kawasaki disease

Kawasaki disease is a mucocutaneous lymph node syndrome with important cardiovascular complications that usually afflicts young children. We describe a 31-year-old woman who developed transient heart failure during the acute phase of Kawasaki disease. The diagnosis was supported by the presence of all six criteria of the disease: fever, conjunctivitis, strawberry tongue, cervical lymphadenopathies, truncal exanthem, and periungual membranous desquamation. Related clinical and laboratory findings included heart failure, arthralgias, transverse nail grooves, thrombocytosis, and elevated serum glutamic oxaloacetic transaminase (SGOT), serum glutamic pyruvic transaminase (SGPT), and bilirubin. Alternative diagnoses were excluded. During her acute febrile illness, the patient developed tachycardia, hypotension, pulmonary rales, S3 gallop, and hepatojugular reflux. The chest roentgenogram showed new Kerley A and B lines. A first-pass isotopic ventriculography showed diffuse hypokinesia and decreased ventricular ejection fractions; spontaneous recovery occurred after a few days. A coronarography performed two months later showed no aneurysmal dilatation. Kawasaki disease is a cause, albeit rare, of myocardial dysfunction in the adult human, and should be sought for actively in a patient with heart failure during the course of an acute febrile illness, associated with mucocutaneous changes.     

Predictive factors for renal sequelae in adults with Henoch-Schönlein purpura

OBJECTIVE: To examine the outcome and risk factors for renal sequelae in an unselected population of adults with Henoch-Schonlein Purpura (HSP). METHODS: Retrospective study of adult patients (> 20 years) with biopsy proved cutaneous vasculitis diagnosed as having HSP seen at a single center between 1984 and 1998. Patients were classified as having HSP according to proposed criteria. Only those patients with a followup of at least 1 year were included in this study of renal sequelae. RESULTS: Twenty-eight patients with a mean followup of 5.5 years fulfilled the inclusion criteria. When the study was concluded, 10 patients (36%) had renal sequelae and 2 (7%) had renal insufficiency. Men outnumbered women. However, neither a previous history of drugs, gender, nor age at disease onset was associated with a higher risk of permanent renal involvement. Patients with hematuria at disease onset or renal involvement during the course of the disease more commonly developed renal sequelae (p < 0.001). The presence of anemia (p = 0.05) at the time of diagnosis and the onset in summer (p < 0.05) were also more common in those with permanent renal involvement (renal sequelae). Patients with relapses had also a higher trend to develop renal sequelae (p = 0.07). All patients who fulfilled more than 2 of these 5 risk factors developed permanent renal involvement. With this model we were able to predict renal sequelae in 8 of the 10 patients who had this complication. The Goodman-Kruskal gamma test value was 0.92 (95% CI 0.78-1.00). CONCLUSION: In unselected adults with HSP, permanent renal involvement (renal sequelae) is not uncommon. Hematuria at disease onset and persistence of renal manifestations during the course of the disease are significant indicators of possible development of renal sequelae. These manifestations plus other features such as onset in summer, anemia at disease onset, or relapses of the disease may predict the development of renal sequelae in most patients.     

Ultrastructural characteristics of myocardial and coronary microvascular lesions in Kawasaki disease.

Kawasaki disease (KD) is an acute febrile mucocutaneous lymph node syndrome with multisystemic vasculitis affecting mainly infants and children. Although several studies on cardiovascular lesions in KD have been done at the light microscopic level, the ultrastructural characteristics and incidences of these lesions have not been well defined. In order to investigate the myocardial and coronary microvascular sequelae in KD, we performed an ultrastructural study on endomyocardial biopsy specimens obtained during follow-up from 54 patients who had typical clinical manifestations of KD, of whom 47 had associated coronary aneurysms as demonstrated by coronary arteriography (CAG) or two-dimensional echocardiography in the acute or healed stage. The average age of onset was 2.2 years old and the duration of illness was from 2 months to 23 years. Follow-up CAG showed that the coronary aneurysms persisted in 33 of the 47 patients (8 with associated stenosis) and resolved in the remaining 14 patients. Ultrastructurally, the myocardial changes revealed hypertrophy, various degrees of degeneration, proliferation and abnormality of mitochondria, infiltration of a small number of lymphocytes, and fibrosis. The coronary microvascular lesion was characterized by microvascular dilatation, endothelial cell injury, platelet aggregation with thrombosis, and stenotic lumen with thickened walls in the small arterioles. It persisted after convalescent stage even up to 23 years and closely correlated with the myocardial sequelae. Moreover, significantly increased incidences of myocardial and coronary microvascular lesions were found in patients with coronary artery lesion. These findings suggest the coronary microvascular lesion as a possible underlying factor of persistent sequelae in KD.     

Kawasaki disease is also a disease of adults: report of six cases

Kawasaki disease is an inflammatory arterial disease of unknown cause usually affecting young children, the principal complication of which is coronary artery aneurysm. Early treatment with immunoglobulins and aspirin prevents this complication. The diagnosis requires expert clinical criteria and, in atypical forms, a more recent decisional diagnostic tree has to be used. The authors report 6 cases of adult Kawasaki disease. As in the other sixty or so cases in the literature, hepatic forms were the commonest (5/6). Only three of the six cases met the classical clinical criteria and the diagnosis was made by the decisional tree or after coronary complications in the oldest subject. The five treated patients progressed favourably after a course of immunoglobulins. Echocardiography detected 100% of children with coronary disease but it was more difficult in adults in whom new non-invasive methods of coronary imaging (fast CT and MRI) and stress testing should complete the investigations. The association of prolonged pyrexia, clinical criteria and a biological inflammatory syndrome should, after exclusion of the differential diagnoses, suggest a diagnosis of Kawasaki disease in the adult as in the child. The possibility of coronary disease, even though extremely rare, should be recognised by the cardiologist and lead to diagnostic and therapeutic managements as aggressive as in children.     

Histopathological studies of endomyocardial biopsy on the children with Kawasaki disease

Since there has been many reports on the coronary artery lesions in the patients with Kawasaki disease, but only a few reports on histopathological investigation employing endomyocardial biopsy, histopathological study was performed in eighteen children of Kawasaki disease with coronary artery lesions and eighteen children of Kawasaki disease with intact coronary artery, aged from one year and ten months to fourteen years. There were twenty males and sixteen females. Comparing Kawasaki disease who had coronary artery lesions with those not, there was no striking difference in histologic findings. Kawasaki disease without coronary artery lesions revealed myocellular hypertrophy, degeneration of myocytes, disarray, interstitial fibrosis and endocardial changes in 44%, 61%, 11% and 44%, respectively. Among Kawasaki disease who had coronary artery lesions, children with coronary artery stenosis showed a higher incidence of histological abnormalities than those with coronary artery aneurysm. Some cases of Kawasaki disease were recognized to have significant myocardial abnormalities even in three to six years after onset, which seemed to persist whether or not they would have coronary artery lesions. Since Kawasaki disease who would have myocardial damages might have the potential for developing cardiomyopathy, careful attention should be payed to myocardial lesions as well as sequelae of coronary artery lesions.     

Adult Kawasaki disease

PURPOSE: Review of the literature on adult Kawasaki disease. CURRENT KNOWLEDGE AND KEY POINTS: Kawasaki disease is an acute multisystemic vasculitis affecting predominantly young children. Several studies have suggested that Kawasaki disease is mediated by bacterial superantigens. The diagnosis is established on clinical criteria since no specific laboratory test yet exists for this disorder. The severity of Kawasaki disease relates to the possible occurrence of coronary aneurysms in 20% of childhood cases. Treatment with intravenous immunoglobulins before day 10 is recommended to prevent aneurysm formation. The occurrence of Kawasaki disease is unusual in adults and 52 cases only have been reported in adult patients. Seventy-one per cent of cases occur between 18 and 30 years. The incidence of specific clinical features is quite similar between adults and children. However meningitis and thrombocytosis are more common in children than in adults, while conversely both arthralgias and liver function abnormalities are more common among adults. Coronary aneurysms are less common in the adults with Kawasaki disease. Other diseases with similar clinical presentation such as drug hypersensitivity reaction and the toxic shock syndrome must be ruled out. Kawasaki disease is often diagnosed after the acute phase at the step of desquamation, when it is too late to expect any beneficial effect from immunoglobulins. FUTURE PROSPECTS AND PROJECTS: Diagnostic criteria of Kawasaki disease have not been validated in an adult population. Criteria of exclusion are necessary to eliminate toxic shock syndrome and drug hypersensitivity syndrome. An international retrospective study to collect data on epidemiologic, clinical, laboratory, and cardiovascular features of adult Kawasaki disease is necessary to validate specific diagnostic criteria and to improve the knowledge on this disease.     

Follow-up studies of Hokkaido residents with Kawasaki disease

OBJECTIVES: Sixteen national surveys of Kawasaki disease in Japan from 1970 to 2000 have identified a total of 169,117 patients with Kawasaki disease. Based on that figure, 8,460 residents of Hokkaido probably have a history of Kawasaki disease. It is also estimated that almost 270 Hokkaido residents would have Kawasaki disease-related coronary artery disease. We underwent follow-up studies of Hokkaido residents > or = 15 years with Kawasaki disease. METHODS: We mailed a questionnaire to the departments of internal medicine, cardiology or cardiovascular surgery inquiring about the health status of patients with Kawasaki disease at 451 hospitals with 20 or more beds in Hokkaido. RESULTS: We obtained replies from 185 hospitals (41.0%). Only 11 hospitals(5.9%) reported experience of patients with Kawasaki disease(with or without coronary artery disease) at hospital follow-up. Detailed patient histories for 60 patients from 7 hospitals were obtained: Twenty patients had Kawasaki disease complicated with coronary artery disease, whereas 40 patients had a history of Kawasaki disease and no present coronary artery disease. Thirty-seven patients without coronary artery disease were followed up at one hospital. The 60 patients were aged from 15 to 36 years. Thirty-nine patients(65%) were in the 15 to 20 year age bracket. Coronary aneurysms were recognized in 25 patients(24 males and 1 female) with Kawasaki disease(41.7%) at the onset of the disease. Twenty patients had an aneurysm(s) in the left main trunk and/or the left anterior descending artery, and 13 patients in the right coronary artery. There was a history of myocardial infarction in four patients (6.7%) and nine patients(15.0%) still suffered from angina pectoris. Aortocoronary bypass surgery was performed in one patient, whereas two patients required percutaneous coronary intervention. CONCLUSIONS: Our study suggests that the majority of Hokkaido residents with Kawasaki disease(with or without coronary artery disease) are not being followed up at area hospitals. The characteristics of patients who were followed up included age < or = 20 years, male sex and the presence of severe coronary artery disease other than circumflex involvement.