Systemic and pulmonary artery aneurysms in incomplete Kawasaki disease

Briceno-Medina, Mario; Perez, Michael; Waller, B. Rush; Sathanandam, Shyam

doi:10.1016/j.jccase.2016.02.003

Cited by 19 publications

(22 citation statements)

References 9 publications

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“…[2][3][4] Since the advent of intravenous immunoglobulin (IVIg) therapy, almost all English-language literature on KD-related SAAs consist of case reports revealing that axillary, subclavian, brachial, iliac, and femoral arteries are common sites of involvement. [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] To date, there are only 2 reports from the same Japanese medical center in which authors reported the incidence of SAAs in patients with KD, but, in fact, less than half of their patients with KD were screened for SAAs; it was during their study that they found that only patients with giant CAAs developed SAAs. 3,4 Although it is a reasonable assumption that there is a concomitant decrease in SAA formation with early IVIg therapy, as well as a decrease in CAAs, there are no published data to support this.…”

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confidence: 99%

Systemic Artery Aneurysms and Kawasaki Disease

Zhao

Chu

et al. 2019

Pediatrics

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BACKGROUND: Coronary artery aneurysms (CAAs) are a well-known complication of Kawasaki disease (KD), but there are no data on incidence or outcomes of systemic artery aneurysms (SAAs) in the current era. METHODS: From April 1, 2016, to March 31, 2019, we screened for SAAs in 162 patients with KD at risk for SAAs with magnetic resonance angiography or peripheral angiography and analyzed incidence and early outcomes of SAAs. RESULTS: Twenty-three patients had SAAs, demonstrating an incidence of 14.2% (23 of 162) in patients who were screened at 1 month after onset. The proportion of patients with SAAs was estimated to be 2% (23 of 1148) of all patients with KD. The median age at onset of KD with SAAs was 5 months. All patients with SAAs had CAAs, with z scores .8. Of patients with giant CAAs, 38.6% (17 of 44) had SAAs. A total of 129 SAAs occurred in 17 different named arteries. The most common sites for SAAs were the axillary (18.6%), common iliac (12.4%), and brachial (11.6%) arteries. During a median follow-up time of 6 months, 92.9% (79 of 85) of SAAs had some degree of regression, with 80% (68 of 85) of SAAs returning to normal. The overall regression rate was higher for medium to large SAAs than for medium to giant CAAs. CONCLUSIONS: Although the incidence of SAAs may not be as dramatically reduced as we expected compared with previous data, SAAs have a high regression rate during short-term follow-up. WHAT'S KNOWN ON THIS SUBJECT: Studies from the 1980s revealed that systemic artery aneurysms (SAAs) may occur in 0.8% to 2% of patients with Kawasaki disease at 3 months after onset. It is speculated that SAAs are now much less common than before. WHAT THIS STUDY ADDS: SAAs occur in 14.2% of patients at risk for SAAs at 1 month after onset, giving an estimated proportion of patients with SAAs as 2% of all patients with Kawasaki disease. SAAs have a high regression rate during short-term follow-up.

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confidence: 99%

Systemic Artery Aneurysms and Kawasaki Disease

Zhao

Chu

et al. 2019

Pediatrics

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“…Both neonatal KD and KD-related SAAs are not well recognized due to their rarity, and thus there are only sporadic reports of a few cases in the English language literature concerning either of these issues [13][14][15][16]. To the best of our knowledge, this is the first report of KD in a newborn with both CAAs and SAAs.…”

Section: Discussionmentioning

confidence: 99%

Neonatal Kawasaki disease with multiple arterial aneurysms: a case report

Zhao

Liang

et al. 2020

Pediatr Rheumatol

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Background: Kawasaki disease (KD) is a medium vessel vasculitis that typically occurs in children aged between 6 months and 5 years. It is extraordinarily rare in the neonatal period. KD-related systemic artery aneurysms (SAAs) have never been reported in neonates. Case presentation: A male infant was transferred to our institution for persistent high-grade fever lasting 16 days. Symptoms started at day 14 of life, and he was admitted to a children's hospital on the second day of fever. Physical examination at the time found no signs suggestive of KD. The only laboratory parameters which were of significance were values suggestive of systemic inflammation. However, his fever persisted and inflammatory markers continued to rise despite 2 weeks of antibiotic therapy. KD as a noninfectious cause of fever was considered when he came to our institution, and echocardiographic findings of left and right medium coronary artery aneurysms (CAAs) confirmed our suspicions. Full-body magnetic resonance angiography also revealed bilateral axillary artery aneurysms. Administration of intravenous gamma globulin resulted in rapid improvement. His fever resolved on the next day and CAAs and SAAs regressed to normal at 6 months and 3 months after diagnosis, respectively. Conclusion: This unique case of incomplete KD highlights the importance of considering KD in neonates with unexplained prolonged fever and reinforces the need to remain vigilant for SAAs in KD.

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“…We compared clinical management and outcomes in our series to those of 7 previously reported cases in 3 published series. [10][11][12]14 CYC-treated KD cases varied signi cantly with respect to indications, dosing, duration and use of other medications (Table 3). In our cohort, we used CYC for worsening CAA, whereas prior case reports described use of CYC for refractory fever and/or persistent KD criteria.…”

Section: Discussionmentioning

confidence: 99%

“…Aside from self-resolving neutropenia in one patient as detailed above, there were no other reported short-term adverse events, consistent with reported experience. 10,11,12 Long-term toxicity of CYC in regard to secondary malignancies and infertility was not reported but would not be expected given the young age of the patients and the low cumulative dose of CYC.…”

Section: Discussionmentioning

confidence: 99%

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Cyclophosphamide Use in Treatment of Refractory Kawasaki Disease With Coronary Artery Aneurysms

Halyabar

Friedman

Sundel

et al. 2020

Preprint

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Background. Despite timely administration of IVIG, some patients with Kawasaki disease (KD) develop rapidly progressive or giant coronary artery aneurysms (CAA). Case presentation. We describe our experience using cyclophosphamide (CYC) for the treatment of such cases as well as a review of the literature on the use of CYC in KD. Through a retrospective chart review of our KD population, we identified ten children treated for KD with intravenous CYC (10 mg/kg/dose) for one or two doses. Seven patients were male, the median age was 2.0 years (range 4 months -5 years). All patients received initial IVIG between day 4-10 of illness. Other anti-inflammatory treatments administered before CYC included second IVIG (n=9), corticosteroids (n=10), infliximab (n=4), cyclosporine (n=2), and anakinra (n=1). Median illness day at administration of the first CYC dose was 22.5 days (range:10-36 days). The primary indication for treatment with CYC for all patients was large or giant CAA and/or rapid progression of CAA. Three patients received a second dose of CYC (10mg/kg) for progressively enlarging CAA. CAA did not progress after final CYC treatment. One patient with a history of neutropenia in infancy developed severe neutropenia 9 days after treatment with CYC, which recovered without intervention or complications. No patient developed infections or other serious toxicity from CYC. Conclusion. In KD patients with severe and progressive enlargement of CAA despite anti-inflammatory therapy, CYC seemed to arrest further dilation and was well-tolerated. Future multicenter studies are needed to confirm our findings in this subgroup of KD patients.

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Systemic and pulmonary artery aneurysms in incomplete Kawasaki disease

Cited by 19 publications

References 9 publications

Systemic Artery Aneurysms and Kawasaki Disease

Systemic Artery Aneurysms and Kawasaki Disease

Neonatal Kawasaki disease with multiple arterial aneurysms: a case report

Cyclophosphamide Use in Treatment of Refractory Kawasaki Disease With Coronary Artery Aneurysms

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