Kawasaki Disease

Abstract

Kawasaki disease (KD) is an acute-onset systemic vasculitis of medium-sized vessels that mostly affects infants and toddlers. Globally, it is the most common form of childhood primary vasculitis. Delayed diagnosis and treatment results in coronary artery aneurysms in up to 25% of all affected individuals. Thus, KD is the most common acquired heart disease in developed countries. Here, the current understanding of clinical presentations, pathophysiological concepts, disease-associated complications, and available pharmaceutical treatment is provided and discussed in the context of available literature.

Keywords: IvIg therapy; fever without infection; inflammatory disorders; kawasaki disease; vasculitis.

Figure 1

Coronary artery aneurysms. (A) Cardial ultrasound of a 3 year old male showing right coronary vessel with aneurysm, and (B) classical coronary angiography in a 2 year old male unveiled giant aneurysm of the left coronay vessel.

Figure 2

Clinical criteria in KD. (A) Bilateral non-purulent conjunctivitis (80–90%), (B) changes to oropharyngeal mucous membranes, including injected and/or fissured lips, strawberry tongue (80–90%), (C) Palmar and/or (D) plantar erythema (E) polymorphous exanthema, primarily truncal, not vesicular (>90%), and (F, G) cervical lymphadenopathy (>1.5 cm) (50%). (G) Ultrasound of enlarged cervical lymph nodes with increased perfusion. (H) Periungual desquamation (in covalescent phase) (80%), (I) Beau lines.

Figure 3

Phases of KD are characterised by variable clinical symptoms. Kawasaki disease reconstitutes a systemic inflammatory disorder with an acute, subacute, and convalescent/recovery phases. Clinical symptoms vary over the course of disease. Classical temporal characteristics are typical of the course of the disease [Figure modified from (23)].

Figure 4

Suggested diagnostic algorithm in suspected incomplete KD. While generally following the suggestions of McCrindle et al. (4), the authors feel that cardiac ultrasound should be performed in all children with fever without focus over ≥5 days and ≥2 clinical criteria for KD or infants with fever without focus ≥7 days independent of CRP levels at least initially.

Figure 5

Additional symptoms and possible organ involvement in KD.

Figure 6

Suggested therapeutic algorithm in KD patients. IVIG, intravenous immunoglobulins; ASA, acetylic acid; MPP, Methylprednisolone i.v. pulse [Modified after (4, 47)].