Adult Kawasaki disease: a rare and challenging diagnosis-a case report

Abstract

Background: Kawasaki disease (KD) is an acute systemic vasculitis which predominantly occurs in childhood but rarely in adulthood. Diagnosis relies on the presence of typical clinical features; however, patients may present atypically, increasing the challenge of timely diagnosis for physicians.

Case summary: We report a case of a 40-year-old male presenting with persistent fever, rash, and unilateral neck swelling. Initial investigations were suggestive of necrotizing lymphadenitis, with a presumed infective aetiology. However, extensive microbiology and immunological investigations remained negative. Cardiac injury was evident with elevated troponin T and NT-proBNP; however, left ventricular systolic function was normal. After 4 days, clinical features consistent with KD were noted and the results of a lymph node biopsy supported this diagnosis. Despite timely treatment with intravenous immunoglobulins (IVIG) and high-dose aspirin, follow-up computed tomography (CT) coronary angiography demonstrated two sequential aneurysms (max 6 mm) in the right coronary artery, plus one small subtle aneurysm in the proximal left anterior descending artery (4 mm).

Discussion: Diagnosis of adult KD remains challenging, as symptoms often present sequentially over time rather than simultaneously and many of the clinical features necessary for diagnosis share commonality with other infectious disease processes.

Keywords: Adult; Case report; Coronary artery aneurysm; Kawasaki disease.

Figure 1

Histopathology of lymph node core biopsy. Evidence of areas of necrosis (arrow) which are associated with abundant neutrophils and debris. Occasional fibrin thrombi are noted in small vessels. Features are of suppurative necrotizing lymphadenitis with a wide differential diagnosis including Kawasaki disease.

Figure 2

Coronary computed tomography angiography shows aneurysms (marked with asterisk) in the right coronary artery and left anterior descending artery 3 months post discharge (A–C). Interval improvement in size of aneurysms noted on follow-up imaging at 12 months post discharge (D–F) Ao, aorta; RCA, right coronary artery; LAD, left anterior descending artery.

Figure 3

Cardiac magnetic resonance imaging with post contrast (gadolinium) imaging showing a confluent stripe of mid-wall fibrosis in the inferolateral/lateral wall at the basal level (A) as well as some patchy inferolateral mid-wall fibrosis at the mid-ventricular level (B).