Understanding Resistant Kawasaki Disease at Sheikh Shakhbout Medical City (SSMC)
Kawasaki disease (KD) is a significant pediatric condition characterized by systemic vasculitis, primarily affecting children under five years old. It is recognized as the leading cause of acquired heart disease in this age group in developed countries. At Sheikh Shakhbout Medical City (SSMC), the Division of Pediatric Rheumatology specializes in the diagnosis and management of resistant cases of Kawasaki disease.
What is Kawasaki Disease?
Kawasaki disease manifests as an acute febrile illness that can lead to serious cardiovascular complications, including coronary artery aneurysms. The etiology of KD remains largely unknown, though it is suspected to arise from an abnormal immune response to an infectious agent in genetically predisposed children. The condition is marked by several clinical features, including:
- Prolonged Fever: Lasting more than five days.
- Rash: Polymorphous rash that typically appears early in the illness.
- Conjunctivitis: Bilateral, non-exudative conjunctivitis.
- Oral Changes: Such as a "strawberry tongue" and cracked lips.
- Lymphadenopathy: Particularly unilateral cervical lymph nodes.
Challenges in Diagnosis and Treatment
The diagnosis of Kawasaki disease can be challenging due to the absence of specific laboratory tests. Instead, clinicians rely on clinical criteria and supportive laboratory findings. In some cases, patients may exhibit incomplete Kawasaki disease, where not all diagnostic criteria are met but significant clinical features are present.At SSMC, the pediatric rheumatology team emphasizes the importance of early recognition and treatment to prevent severe complications. First-line treatment typically includes intravenous immunoglobulin (IVIG), which has been shown to reduce the risk of coronary artery damage significantly. However, approximately 10% of patients may be resistant to initial IVIG therapy, necessitating additional interventions.
Management of Resistant Kawasaki Disease
For children who do not respond adequately to IVIG therapy, alternative management strategies are crucial. These may include:
- Repeat IVIG Infusion: Administering a second dose of IVIG.
- Corticosteroids: Utilizing corticosteroids either alone or in conjunction with IVIG for high-risk patients.
- Other Immunomodulatory Therapies: Exploring options such as anti-TNFα agents or other novel therapies under investigation.
Importance of Multidisciplinary Care
The management of resistant Kawasaki disease at SSMC involves a multidisciplinary approach. Collaboration with pediatric cardiology is essential for monitoring and managing potential coronary artery lesions. Regular follow-up with echocardiograms and other imaging modalities helps assess the progression or regression of coronary artery abnormalities.
Sheikh Shakhbout Medical City’s Division of Pediatric Rheumatology is dedicated to providing comprehensive care for children with Kawasaki disease, particularly those who exhibit resistance to standard treatments. Through early diagnosis, effective treatment protocols, and a collaborative approach, SSMC aims to improve outcomes for affected children and reduce the risk of long-term cardiovascular complications associated with this serious condition.
Why choose SSMC?
A Multidisciplinary Approach to Pediatric Rheumatology
Our Division of Pediatric Rheumatology is led by experienced consultants who specialize in diagnosing and treating a wide range of autoimmune and autoinflammatory diseases in children. We take a comprehensive, patient-centered approach, working closely with a multidisciplinary team of experts to provide the highest quality care.
State-of-the-Art Technology and Innovative Treatments
SSMC is equipped with advanced technology and offers unique treatment options for pediatric rheumatology patients:
Specialized Procedures
- Intra-articular Injections: We administer intra-articular triamcinolone hexacetonide injections, a long-acting steroid, to treat joint inflammation in children. These injections can be performed in our clinic or under general anesthesia, depending on the child's needs.
- Day Care Infusions: We provide day care infusion services for a variety of treatments, including intravenous steroids, biologics, chemotherapy, rituximab, IVIG, and bisphosphonates. These treatments are tailored to each child's specific condition and administered under the supervision of our experienced team.
Comprehensive Diagnostic Services
- Recurrent Fever Syndrome Evaluation: We offer thorough assessments to identify the underlying causes of recurrent fever syndrome in children. Our team utilizes advanced diagnostic tools and techniques to accurately diagnose and manage this complex condition.
By combining expert care, cutting-edge technology, and a patient-centered approach, SSMC is committed to providing the best possible outcomes for children with pediatric rheumatic diseases
