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Author Anonymous (Edited by Ayokunle Olubaniyi and Alison Leibowitz)
Exam Type USMLE Step 2 CK
Main Category Pediatrics
Sub Category Allergy/Immunology
Prompt A 13 year-old boy is brought to the clinic for a gradually worsening fever of 10 days duration accompanied by a diffuse rash. His mother states that the fever has been as high as 103.0°F (39.4°C) and she has kept him out of school because of this. Observed upon physical exam is a diffuse morbilliform rash over his trunk and extremities, which also becomes confluent in the groin. Both sclerae are infected and his tongue is red and bumpy on appearance. Notable lymphadenopathy is present in the anterior and posterior cervical chains.

Which of the following complications is the most common and life-threatening in this patient?

Answer A Myocarditis
Answer A Explanation [[AnswerAExp::This is also a common complication of Kawasaki disease which usually responds well to intravenous immunoglobulin (IVIG) therapy. Other cardiac involvement include: cardiomyopathy and left ventricular dysfunction.]]
Answer B Hearing loss
Answer B Explanation Sensorineural hearing loss is a rare and transient complication. It responds promptly to intravenous immunoglobulin (IVIG) therapy.
Answer C Interstitial nephritis
Answer C Explanation [[AnswerCExp::Acute interstitial nephritis is a rare complication. Other urinary abnormalities associated with Kawasaki disease include: mild proteinuria, acute renal failure, hemolytic-uremic syndrome, and immune complex-mediated glomerulonephritis.]]
Answer D Pneumonitis
Answer D Explanation This is rare; it responds promptly to intravenous immunoglobulin (IVIG) therapy.
Answer E Coronary artery aneurysm
Answer E Explanation This is the most life-threatening complication.
Right Answer E
Explanation [[Explanation::The patient in this vignette has a condition known as Kawasaki disease. Kawasaki disease is an acute febrile vasculitis affecting many systems, including the skin, nervous system, gastrointestinal tract, musculo-skeletal system, kidneys, lungs, eyes, and the hematologic system.

The most common and life-threatening complications of Kawasaki syndrome are aneurysms which are usually confined to the medium-sized vessels and classically those located in the heart. Coronary aneurysms may be small (<5 mm) or giant (>8 mm). The latter is associated with profound morbidity and mortality. High risk patients of coronary artery aneurysms include:

  • Children without intravenous immunoglobulin (IVIG) within 10 days from the onset of fever
  • Patients who have persistent fever despite IVIG treatment
  • Patients with laboratory findings suggestive of persistent inflammation (increased ESR, C-reactive protein (CRP) or both);
  • Young children (<6 months old) or older children (>8 years old)
  • Patients of the male sex

With identification of Kawasaki disease it is essential to schedule follow-up exams 6-8 weeks after resolution of the disease, coupled with a timely referral to a cardiologist.
Educational Objective: Cardiac involvement, especially, the involvement of the coronary artery, leading to the development of coronary aneurysms is the most feared complication of Kawasaki disease. The likelihood of this complication reduces with a prompt initiation of intravenous immunoglobulin (IVIG) therapy within the first 10 days of onset of symptoms.
References: Mori M, Imagawa T, Yasui K, et al. Predictors of coronary artery lesions after intravenous gamma-globulin treatment in Kawasaki disease. J Pediatr. 2000;137:177-180]]

Approved No
Keyword [[WBRKeyword::Kawasaki disease]], complications of kawasaki disease
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