Kawasaki disease natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dildar Hussain, MBBS [2]

Overview

If left untreated, the symptoms will eventually relent, but coronary artery aneurysms will not improve, resulting in a significant risk of death or disability due to myocardial infarction. If treated in a timely fashion, this risk can be mostly avoided and the course of illness cut short. Patients with Kawasaki disease may progress to develop long term cardiovascular illness such as coronary artery disease, and pre-mature atherosclerosis. Common complications of Kawasaki disease include vasculitis and coronary artery aneurysms. Prognosis is generally excellent and the mortality rate of patients with Kawasaki disease is approximately 2%.

If left untreated, the symptoms will eventually relent, but coronary artery aneurysms will not improve, resulting in a significant risk of death or disability due to myocardial infarction.
If treated in a timely fashion, this risk can be mostly avoided and the course of illness cut short.
Patients with Kawasaki disease may progress to develop long term cardiovascular illness such as coronary artery disease, and pre-mature atherosclerosis.^[1]

The cardiac complications are, by far, the most important aspect of Kawasaki disease:^[2]^[3]
- Vasculitic changes in the coronary arteries
- Coronary artery aneurysms
  - These aneurysms can lead to myocardial infarction even in young children. Overall, about 10 – 18% of children with Kawasaki disease develop coronary artery aneurysms, with much higher prevalence among patients who are not treated early in the course of illness.
  - Kawasaki disease is the most common cause of acquired heart disease among children in the United States.

Organ and Tissue	Associated Conditions
Liver	Hepatitis
Lung	Interstitial pneumonitis
Gastrointestinal tract	Abdominal pain Diarrhea Vomiting Gallbladder hydrops
Meninges	Aseptic meningitis Irritability
Heart	Myocarditis Pericarditis Valvulitis
Urinary tract	Pyuria
Pancreas	Pancreatitis
Lymph nodes	Lymphadenopathy

With early treatment, rapid recovery from the acute symptoms can be expected and the risk of coronary artery aneurysms greatly reduced.
Untreated, the acute symptoms of Kawasaki disease are self-limited, but the risk of coronary artery involvement is much greater.
Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every 1 – 2 years to screen for the progression of cardiac involvement.
Overall, about 2% of the patients die from complications of coronary vasculitis.
It is also not uncommon that a relapse of symptoms may occur soon after initial treatment with IVIG.
- This usually requires re-hospitalization and retreatment. Treatment with IVIG can cause allergic and non-allergic acute reactions, aseptic meningitis, fluid overload and rarely other serious reactions.
- Aspirin may increase the risk of bleeding from other causes and may be associated with Reye’s syndrome.
- Overall, life-threatening complications resulting from therapy for Kawasaki disease are exceedingly rare, especially compared with the risk of non-treatment.

↑ Kato H (September 2014). “[Natural history of Kawasaki disease vasculitis]”. Nippon Rinsho (in Japanese). 72 (9): 1530–5. PMID 25518398.
↑ Belay E, Maddox R, Holman R, Curns A, Ballah K, Schonberger L (2006). “Kawasaki syndrome and risk factors for coronary artery abnormalities: United States, 1994-2003”. Pediatr Infect Dis J. 25 (3): 245–9. PMID 16511388.
↑ McCrindle, Brian W.; Rowley, Anne H.; Newburger, Jane W.; Burns, Jane C.; Bolger, Anne F.; Gewitz, Michael; Baker, Annette L.; Jackson, Mary Anne; Takahashi, Masato; Shah, Pinak B.; Kobayashi, Tohru; Wu, Mei-Hwan; Saji, Tsutomu T.; Pahl, Elfriede (2017). “Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association”. Circulation. 135 (17): e927–e999. doi:10.1161/CIR.0000000000000484. ISSN 0009-7322.
↑ Amano S, Hazama F, Hamashima Y (July 1979). “Pathology of Kawasaki disease: I. Pathology and morphogenesis of the vascular changes”. Jpn. Circ. J. 43 (7): 633–43. PMID 41111.