Summary about Disease
Kawasaki Disease Shock Syndrome (KDSS) Kawasaki Disease Shock Syndrome (KDSS) is a rare but serious complication of Kawasaki disease (KD). KD is an acute, self-limited vasculitis of unknown etiology that primarily affects young children. KDSS is characterized by the typical features of KD along with signs of circulatory shock, such as low blood pressure, poor organ perfusion, and potentially multi-organ dysfunction. The addition of shock significantly increases the risk of cardiac complications and mortality compared to KD alone. Early recognition and aggressive treatment are crucial.
Symptoms
KDSS encompasses the typical symptoms of Kawasaki disease, along with signs of shock:
Kawasaki Disease Symptoms:
Fever (typically high and persistent, >5 days)
Rash (often polymorphous, meaning it can take different forms)
Bilateral conjunctival injection (redness of the eyes without discharge)
Changes in the lips and oral cavity (e.g., strawberry tongue, cracked lips, redness of the mouth)
Cervical lymphadenopathy (swollen lymph nodes in the neck, usually at least one node >1.5 cm)
Changes in the extremities (redness and swelling of hands and feet, followed by peeling of skin in the convalescent phase)
Shock Symptoms:
Hypotension (low blood pressure)
Tachycardia (rapid heart rate)
Signs of poor perfusion (e.g., cool extremities, delayed capillary refill)
Lethargy or altered mental status
Oliguria (decreased urine output)
Evidence of organ dysfunction (e.g., elevated liver enzymes, acute kidney injury)
Causes
The exact cause of KD, and thus KDSS, is unknown. It is thought to involve a complex interaction between genetic predisposition, environmental triggers (possibly infectious agents), and an abnormal immune response. In KDSS, the inflammatory process is so severe that it leads to vasodilation, capillary leak, and myocardial dysfunction, resulting in circulatory shock.
Medicine Used
The primary treatment for KDSS involves addressing both the underlying Kawasaki disease and the shock state:
Intravenous Immunoglobulin (IVIG): This is the standard treatment for KD and is aimed at modulating the immune response and reducing the risk of coronary artery aneurysms.
Aspirin: High-dose aspirin is initially used for its anti-inflammatory properties. Once the fever subsides, the dose is reduced to a low-dose for its antiplatelet effects to prevent blood clots.
Vasopressors/Inotropes: Medications like norepinephrine, epinephrine, or dopamine may be needed to support blood pressure and improve cardiac output.
Fluid Resuscitation: Careful fluid administration is essential to improve circulatory volume, but excessive fluids can worsen pulmonary edema.
Corticosteroids: Some studies suggest a role for corticosteroids, particularly in patients with KDSS who do not respond to IVIG, but the evidence is still debated.
Other Immunomodulatory Therapies: In refractory cases (those not responding to IVIG), other agents like infliximab or anakinra may be considered.
Is Communicable
Kawasaki disease and, therefore, Kawasaki Disease Shock Syndrome, are not communicable. They are not caused by a contagious infection that can be spread from person to person.
Precautions
Since the cause of KD and KDSS is unknown, specific precautions to prevent them are not established. General measures include:
Prompt Medical Attention: Early recognition of symptoms is crucial. Parents should seek immediate medical evaluation if their child develops a persistent fever accompanied by other symptoms suggestive of KD.
Strict Adherence to Treatment: Following the prescribed treatment plan, including IVIG and aspirin, is essential for managing KD and reducing the risk of complications like KDSS.
Monitoring for Complications: Regular follow-up appointments with a cardiologist are needed to monitor for cardiac complications, such as coronary artery aneurysms.
How long does an outbreak last?
Kawasaki disease occurs in individual cases and sometimes clusters of cases, but it does not present as a sustained outbreak in the way that a communicable disease might. The acute phase of KD typically lasts 1-2 weeks if untreated. With treatment, the fever and other symptoms usually resolve within 24-48 hours of IVIG infusion. However, the entire course of the illness, including the convalescent phase and long-term monitoring for cardiac complications, can last several months.
How is it diagnosed?
KDSS is diagnosed based on the following criteria:
Diagnostic Criteria for Kawasaki Disease: The diagnosis of KD is based on clinical criteria. The classic criteria include fever for at least 5 days, along with at least four of the following five criteria:
Bilateral conjunctival injection
Changes in lips and oral cavity
Cervical lymphadenopathy
Polymorphous rash
Changes in the extremities
Presence of Shock: Signs of circulatory shock, such as hypotension, poor perfusion, and/or evidence of organ dysfunction, must be present.
Exclusion of Other Conditions: Other conditions that can mimic KD or cause shock, such as sepsis, toxic shock syndrome, or viral infections, should be ruled out.
Laboratory Tests: Laboratory tests can support the diagnosis and help assess the severity of the illness. These may include:
Complete blood count (CBC)
Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) (measures of inflammation)
Liver function tests (LFTs)
Renal function tests
Blood cultures (to rule out sepsis)
Echocardiogram (to evaluate for coronary artery abnormalities)
Timeline of Symptoms
The typical timeline of KDSS follows a similar progression to KD, with the addition of shock:
Phase 1 (Acute Phase): (1-2 weeks)
High fever (often the first symptom)
Other classic KD symptoms (rash, conjunctivitis, oral changes, lymphadenopathy, extremity changes) appear.
Signs of shock may develop during this phase, typically within the first week of illness.
Phase 2 (Subacute Phase): (2-4 weeks)
Fever subsides, but other symptoms may persist.
Skin peeling, especially on the fingers and toes, is common.
The risk of coronary artery aneurysms is highest during this phase.
Phase 3 (Convalescent Phase): (Several weeks to months)
Symptoms gradually resolve.
Laboratory values normalize.
Long-term monitoring for cardiac complications is essential.
Important Considerations
Early Recognition: Prompt recognition of KDSS is critical because it allows for timely intervention, which can improve outcomes.
Aggressive Management: KDSS requires aggressive management, including IVIG, aspirin, vasopressors/inotropes, and potentially other immunomodulatory therapies.
Cardiac Monitoring: All patients with KDSS should undergo close cardiac monitoring, including serial echocardiograms, to assess for coronary artery aneurysms.
Multidisciplinary Approach: Management of KDSS often requires a multidisciplinary approach involving pediatricians, cardiologists, intensivists, and other specialists.
IVIG Resistance: Some patients with KDSS may be resistant to IVIG therapy, requiring alternative treatments such as corticosteroids or other immunomodulatory agents.