Kawasaki Shock Syndrome Complicating a Recurrence of Kawasaki Disease

Abstract

We describe a case of recurrent Kawasaki disease (KD) in a non-Asian 6-year-old boy who had been diagnosed with typical KD without cardiac involvement at age 3 years. He was admitted to the PICU 3 years later for heart failure, hypotension, and deterioration of his general condition. Ultrasonography revealed left ventricular dysfunction with a 44% ejection fraction and grade I mitral valve failure without coronary artery involvement. Subsequent observation of hyperemic conjunctiva, bilateral cervical adenopathies with erythematous skin (normal neck ultrasound and computed axial tomography findings), peeling of the fingertips at day 8 of the illness, and occurrence of an inflammatory syndrome led to a diagnosis of incomplete recurrent KD with a clinical picture of Kawasaki shock syndrome (KSS). Clinical improvement was rapidly obtained after intravenous immunoglobulin and intravenous corticosteroid therapy (30 mg/kg per day for 3 subsequent days). Left ventricular function gradually improved, with ultrasound returning to normal after 3 months. Diagnosis was difficult to establish because of the recurrence of the disease and the incomplete clinical picture, with clinical features of KSS. Physicians need to be aware of these pitfalls in the management of patients with clinical signs of KD. Kawasaki disease (KD) is an acute, selflimited vasculitis. Its origin is still unknown despite intensive research. It affects children, mainly between the ages of 6 months and 5 years. Its diagnosis relies on the compilation of clinical and biological data, because no specific diagnostic test exists. Diagnosis must be made early to avoid coronary involvement, which is the most severe complication of KD. The disease can sometimes display atypical or incomplete clinical pictures that the physician needs to know. Also, the disease is liable to recur many months after a first episode. PATIENT PRESENTATIONAt age 3 years, our patient had developed typical KD (fever .5 days, erythema, cheilitis, conjunctivitis, and cervical adenopathies) with a biological inflammatory syndrome (C-reactive protein [CRP]: 18.3 mg/dL; erythrocyte sedimentation rate: 64 mm). Investigations for infection (throat swab polymerase chain reaction analysis for herpes, blood cultures, and serology for mycoplasmas and herpes virus) were negative. Echocardiography on day 6 was normal (telediastolic left ventricle diameter: 35 mm; shortening fraction: 34%; ejection fraction: 65%; no valvular involvement, no coronary abnormalities, and no pericardial effusion). He was treated with intravenous g-globulin (IVIG; Tegeline; LFB Biomédicaments, Les Ulis, Essonne, France) 2 g/kg on day 5 and acetylsalicylic acid (80 mg/kg per day for 2 weeks and then 3 mg/kg per day for 1 month), and his clinical condition improved rapidly. A diminution of blood pressure occurred during the injection, and was considered to be an anaphylactic reaction. Follow-up echocardiograms at 1 and 3 months were normal.Three years later, he was again admitted to the PICU for clinical signs of he...

Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease

“…The most frequent diagnosis at the time of admission was TSS or septic shock. Twenty patients were diagnosed with TSS, 16 of whom had coronary artery abnormalities . Other common diagnoses were suppurative lymphadenitis and myocarditis.…”

“…Twenty patients were diagnosed with TSS, 16 of whom had coronary artery abnormalities. 5,7,9,17,[19][20][21][22][23]26,33,34,39 Other common diagnoses were suppurative lymphadenitis and myocarditis. Seventy-two patients had complete KD (69.9%), and 31 had incomplete KD (31.1%).…”

The epidemiology of Kawasaki disease: a global update

“…The diagnosis is usually made on a constellation of characteristic clinical findings, which may appear sequentially 1. KD is a serious and potentially life-threatening condition 2. It results in coronary artery aneurysms in up to a quarter of untreated patients.…”

Kawasaki disease shock syndrome: A report of two cases and literature review

“…However, in most studies, including in our two patients, in acute KDSS, lower platelet counts were more common . Given these considerations, it is very important to make a correct diagnosis using echocardiography to detect coronary artery lesions and to give appropriate treatment . In our two patients, we also found that serum BNP or N‐terminal proBNP (NT‐proBNP) might play an important role in differentiating KDSS from TSS, each of these values increased during hypotension.…”

“…Lin, et al stated that, echocardiographic abnormities, especially coronary artery lesions, anemia, and thrombocytosis were highly suggestive of KDSS . However, in most studies, including in our two patients, in acute KDSS, lower platelet counts were more common . Given these considerations, it is very important to make a correct diagnosis using echocardiography to detect coronary artery lesions and to give appropriate treatment .…”