KAWASAKI DISEASE
A.L. Billson, A.V. Pearce, C. Tuffrey in Key Topics in Paediatrics, 1994
Kawasaki disease (mucocutaneous lymph node syndrome) is a systemic vasculitis of early childhood, with a prevalence of 3.4 per 100 000 children under 5 years in the British Isles. Oriental races are more at risk than Caucasian (80 000 reported cases in Japan), and although epidemiological studies are highly suggestive of an infective aetiology no definite pathogen has yet been established. Sudden death from coronary complications occurs in at least 2% of cases, and although specific therapy with aspirin and intravenous immunoglobulin may reduce coronary artery abnormalities, treatment must be given during the initial 10 days of the illness to be of benefit. Prompt diagnosis is therefore essential.
Intravenous Immunoglobulin in the Treatment of Vasculitis
Marinos C. Dalakas, Peter J. Späth in Intravenous Immunoglobulins in the Third Millennium, 2004
The primary systemic vasculitides have an annual incidence of 40/million and are classified according to their association with anti-neutrophil cytoplasm autoantibodies (ANCAs). Those predominantly involving microscopic vessels without immune deposits include Wegener’s granulomatosis, microscopic polyangiitis and Churg-Strauss angiitis, and are usually ANCA-positive and represent 50-75% of the total. The ANCAnegative group include those with immune deposits, such as Henoch-Schönlein purpura and cryoglobulinemia or those restricted to muscular arteries, including polyarteritis nodosa and giant cell and Takayasu’s arteritis1,2. The treatment of primary systemic vasculitis with high-dose corticosteroids and immune-suppressive drugs is generally effective in controlling clinical evidence of disease activity in new presentations3,4. This approach is complicated by frequent, severe toxicity and infections, a high relapse rate and chronic morbidity with increasing incapacity. Following demonstration of the efficacy of intravenous immunoglobulin (IVIG) in reducing the incidence of coronary artery aneurysms in the childhood vasculitis, Kawasaki disease, there has been interest in treating other vasculitides with IVIG.
Intravenous lmmunoglobulin (IVIg) in the Treatment of Autoimmune Diseases
Vibeke Strand in Novel Therapeutic Agents for the Treatment of Autoimmune Diseases, 1997
I. The decision to use intravenously administered immunoglobulin (TVlg) in a variety of autoimmune diseases, including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), dermatomyositis/polymyositis (DM/PM), vasculitis, neurological diseases such as Guillain-Barre syndrome (GBS), multiple sclerosis, amyotrophic lateral sclerosis, and myasthenia gravis, and hematological diseases such as autoimmune hemolytic anemia was largely based on its successful empirical use in idiopathic thrombocytopenic purpura (TTP). Randomized controlled trials (RCTs) have subsequently confirmed the efficacy ofIVIg therapy compared to aspirin in Kawasaki disease (KD) ( J ,2), to corticosteroids in ITP (3), to placebo in DM (4), and to plasmapheresis in OBS (5).
Autoimmune haemolytic anaemia caused by anti-M antibody in a patient with Kawasaki disease
Published in Modern Rheumatology Case Reports, 2020
Masaki Shimomura, Yuka Okura, Osamu Ohta, Yutaka Takahashi, Ichiro Kobayashi
Intravenous immunoglobulin (IVIG) is a standard therapy for Kawasaki disease (KD), because it prevents formation of coronary artery aneurysm, a major complication of KD. Herein, we report a 3-year-old boy with KD complicated by haemolytic anaemia (HA) which developed following two courses of IVIG. Although both direct and indirect antiglobulin tests and anti-M antibodies were positive in his blood obtained after the onset of HA, indirect antiglobulin tests and anti-M antibodies were negative either in the blood sample before the treatment or the same lot of IVIG products as those used for the therapy, suggesting autoimmune mechanism. This is, to our knowledge, the first report of autoimmune HA caused by anti-M autoantibodies after IVIG therapy in KD.
Elevated serum YKL-40 levels in patients with Kawasaki disease
Published in Biomarkers, 2017
K. Y. Kim, Y. Ahn, D. Y. Kim, Ho-Seong Kim, D. S. Kim
Context: YKL-40 is an inflammatory biomarker for endothelial dysfunction that may have a role in Kawasaki disease (KD). Objectives: We investigated the association of serum YKL-40 levels with KD and established laboratory parameters for YKL-40 levels and other inflammatory markers. Methods: YKL-40 levels and other inflammatory markers of 23 KD patients, 9 disease control patients and 11 age-matched healthy controls. Results: YKL-40 concentration in the serum of KD patients significantly increased during the acute disease phase compared with those of disease controls and healthy controls. Conclusions: Increased YKL-40 levels may provide a useful inflammatory marker for patients with KD.
Vaccines and Kawasaki disease
Published in Expert Review of Vaccines, 2016
Susanna Esposito, Sonia Bianchini, Rosa Maria Dellepiane, Nicola Principi
The distinctive immune system characteristics of children with Kawasaki disease (KD) could suggest that they respond in a particular way to all antigenic stimulations, including those due to vaccines. Moreover, treatment of KD is mainly based on immunomodulatory therapy. These factors suggest that vaccines and KD may interact in several ways. These interactions could be of clinical relevance because KD is a disease of younger children who receive most of the vaccines recommended for infectious disease prevention. This paper shows that available evidence does not support an association between KD development and vaccine administration. Moreover, it highlights that administration of routine vaccines is mandatory even in children with KD and all efforts must be made to ensure the highest degree of protection against vaccine-preventable diseases for these patients. However, studies are needed to clarify currently unsolved issues, especially issues related to immunologic interference induced by intravenous immunoglobulin and biological drugs.
Related Knowledge Centers
- Arteries
- Lymph
- Lymph Nodes
- Lymphatic Vessels
- Vasculitis
- Veins
- Vascular Skin Diseases