Case Review

Adult Kawasaki Disease in a Young Aboriginal Male: A First Case Report

Ella Goodman, MPH, Nancy Woods, MD, Chris Loreto, MD, Clodagh Ryan, MD, Patrick Bergin, MD

About the Authors

Ella Goodman is a member of the charter class of the Northern Ontario School of Medicine. Nancy Woods is a family physician and Chris Loreto is an emergency physician in Northern Ontario. Clodagh Ryan is a respirologist at the Toronto General Hospital, and Pat Bergin is a community general internist.

Kawasaki disease is a systemic vasculitis of unknown etiology that occurs in children of all races.1 There are an estimated 60 documented cases of adult Kawasaki disease in the English literature.2 We review a case of a previously healthy young Aboriginal male diagnosed with Kawasaki disease after presenting with rash, jaundice, and fever of unknown origin.

Presentation

An 18-year-old male presented to the emergency department (ER) three times in five days with initial vague complaints of fever, malaise, sore throat, coryza, and subsequently a macular rash on his hands and feet. He had no recent history of travel, camping, tick bites, or home renovations. He had not consumed food or water from new or unknown sources, and reported no high-risk behaviours. His family history was significant for a brother with dormant tuberculosis. Results on an initial chest radiograph were normal, and urinalysis, throat swab, and blood cultures were negative. He was started empirically on oral penicillin on his second ER visit. He was admitted to hospital on his fourth visit with a presumptive diagnosis of adult Kawasaki disease. Internal medicine was consulted, and antibiotics were discontinued less than 24 hours later. The ER physician noted, “If he was 5 years old, he would have Kawasaki disease.”

On examination, he looked unwell. He was febrile (temperature range 38–39.3°C), but his pulse and blood pressure were stable. He had bilateral nonpurulent conjunctivitis, a strawberry tongue, fissured lips, mild tender hepatomegaly, and jaundice. He did not have exudative pharyngitis, cervical lymphadenopathy, splenomegaly, inflammatory synovitis, or signs of meningeal irritation. A lumbar puncture was not felt to be clinically indicated. His cardiorespiratory examination was normal. A confluent macular rash was present not only on the extensor surfaces of his elbows and knees, but also on the volar aspect of the hands and feet (Figure 1).

Figure 1. A confluent macular rash was present.

Laboratory investigations revealed an elevated WBC of 22.7 × 109/L with a left shift, platelets 327 × 109/L, hemoglobin 137g/L with a normal MCV. Electrolytes, BUN, and creatinine were normal. Liver transaminases were mildly elevated with an AST of 57 U/L and ALT of 62 U/L. Alkaline phosphatase (377 U/L), GGT (461 U/L), and bilirubin (136 μmol/L – mixed) were more significantly elevated. INR, LDH, and protein were normal, but albumin (28 g/L) was decreased. His inflammatory markers ESR (65 mm/h) and CRP (148 mg/L) were increased.

While in hospital extensive immunological and serological laboratory tests were completed, all of which were negative, including IgG subclasses, IgA, IgM, RF, ANA, anti-ENA antibody, and antimitochondrial antibody, blastomycosis, histoplasmosis, toxoplasmosis, CMV, and EBV serologies. Monospot, VDRL, HIV-1 and -2 antibodies, and hepatitis A, B, and C studies were normal. A urine drug screen was negative, and blood salicylates and acetaminophen levels were normal. Urine, blood, and upper respiratory tract cultures were all negative for bacteria and tuberculosis. Tuberculosis skin testing was negative. An ASOT was slightly increased at 270 IU/mL (normal up to 263 IU/mL).

Several diagnostic imaging tests were completed during his stay in hospital. A repeat chest radiograph on admission revealed right lower lobe infiltrate. An echocardiogram, abdominal ultrasound, and CT scan of the abdomen and pelvis were normal. A thoracic CT scan demonstrated multiple poorly defined nodular opacities measuring ≤7 mm in the mid-lung zones bilaterally and a consolidation in right lower lobe.

Discussion

Kawasaki disease is classically a disease of young children and was first described by Dr. Tomisaku Kawasaki and colleagues in 1967 in Japan,3 where the incidence is the highest in the world.4 The Canadian Institute for Health Information provides an incidence of 13.8 per 100,000 across Canada among children less than 5 years of age.3 Although rare, approximately 60 cases of adult Kawasaki disease have been reported in the English literature.2 

Pediatric Kawasaki disease is characterized by a fever of 5 or more days’ duration, plus four of the following five diagnostic criteria:

1.           Changes in the extremities such as peripheral edema, peripheral erythema, and desquamation

2.           Polymorphous exanthems

3.           Bilateral conjunctival injection

4.           Changes in the mucous membranes of the oral cavity, such as injected pharynx, injected lips, and strawberry tongue

5.           Acute non-suppurative swelling of the cervical lymph nodes5

Seve et al. completed a literature review of 57 reported cases of Kawasaki disease using a MEDLINE search from 1967 to 2003. They compared clinical features of adult and pediatric Kawasaki disease and found that adults tended to present more frequently with adenopathy (93% versus 15%), arthralgia (61% versus 24%), hepatitis (37% versus 10%), and elevated liver enzymes (65% versus 10%). Adults had much lower rates of aseptic meningitis (10% versus 34%), electrocardiographic abnormalities (34% versus 50%), coronary aneurysm (5% versus 20%), and thrombocytosis (55% versus 100%). Some features of Kawasaki disease were similar in both adults and children, including conjunctivitis, perioral signs and symptoms, edema, erythema, and desquamation of palms and soles.6

Perhaps the most devastating complication of Kawasaki disease is the possibility of coronary artery sequelae (usually aneurysms). It is estimated that up to 25% of children who go untreated develop coronary artery aneurysms.7 However, in those receiving the recommended treatment, the rate of coronary artery aneurysms drops to 4–8%.3 Adult cases of Kawasaki disease have a much lower rate of coronary artery aneurysms ranging from 5 to 7%.2,6,8 The goal of treatment is to prevent coronary artery sequelae.

Current guidelines for the treatment of pediatric cases include intravenous gamma globulin (IVIG) 2 g/kg as a single infusion over 12 hours within 10 days’ onset of fever (or 400 mg/kg/d for 4 days), and high-dose ASA 80–100 mg/kg/d until the patient is afebrile, then 3–5 mg/kg/d for 6–8 weeks.3 A recent randomized controlled trial found no benefit to the addition of pulsed corticosteroid therapy.9 Currently, there are no specific diagnostic criteria or treatment guidelines established for adults.

Case Treatment and Results

Approximately 9 days after symptom onset, our patient was treated for presumed Kawasaki disease with IVIG therapy and high-dose ASA. He defervesced within 24 hours and clinically improved. The ASA was then decreased to a lower maintenance dose (4 mg/kg/d). During the course of his hospital stay, the patient’s rash changed from macular to coalescing fine pustules (Figure 2) on an erythematous base, with mild residual hyperpigmentation. After a week in hospital, he developed desquamation of the skin of his hands.

Figure 2. The macular rash changed to coalescing fine pustules.

After receiving IVIG, his WBC decreased to 10.5 × 109/L; however, his LFTs (GGT 701 U/L, AST 186 U/L, ALT 186 U/L) and ESR (104 mm/h) continued to rise. With rising LFTs and inflammatory markers, our patient was transferred to the University Health Network in Toronto (Toronto General Hospital) for further evaluation. Adult and pediatric rheumatology, hepatology, and infectious disease consultations agreed with a clinical diagnosis of adult Kawasaki disease. Coronary CT angiography, bronchoscopy, and washings were normal. All fungal, bacterial, and TB cultures were negative. Respiratory viral panel was negative, but Mycoplasma titres were positive. A liver biopsy was not pursued on the advice of hepatology. An extensive repeat vasculitis and connective tissue workup was normal except for a mild elevation in pANCA level (171 AAU/mL, normal <149). He improved clinically and was discharged home with a diagnosis of Kawasaki disease, with plans for follow-up echocardiography and an outpatient referral to a vasculitis specialist. His LFTs normalized on follow-up blood work several weeks later.

The presence of pediatric Kawasaki disease and concurrent Mycoplasma pneumonia has been identified elsewhere in the literature, but to our knowledge not confirmed in adult cases.10–14 The etiology of Kawasaki disease is unclear, but it has been postulated that superantigens produced by M. pneumoniae, staphylococci, streptococci, or retroviruses may be associative or causative in Kawasaki disease.11 Mycoplasma pneumonia has been associated with small pulmonary nodules such as those seen in this case,15,16 and Kawasaki disease has also been associated with a positive pANCA, as reported in the literature.17–20 The full significance of the association of Mycoplasma pneumonia and/or positive pANCA with Kawasaki disease remains unknown.

Summary

In summary, Kawasaki disease is often a disease of young children, although adult cases have been infrequently reported. Despite its low prevalence but potential for development of coronary artery aneurysms, one should maintain an increased index of suspicion for Kawasaki disease in adults presenting with persistent (>5 days) fever of unknown origin. Such case reports of adult Kawasaki disease – we believe this to be the first confirmed in an Aboriginal adult – are important so that diagnostic criteria and treatment guidelines can be created specifically for adults.

References

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 Article Citation: Goodman E, Woods N, Loreto C, Ryan C, Bergin P. Adult Kawasaki Disease in a Young Aboriginal Male: A First Case Report. Can J Gen Intern Med 2008 3(2);84-86