Diagnostic value of anti-neutrophil cytoplasmic and anti-endothelial cell antibodies in early Kawasaki disease

doi:10.1016/S0022-3476(05)83180-4

The Journal of Pediatrics

Volume 124, Issue 6, June 1994, Pages 917-920

https://doi.org/10.1016/S0022-3476(05)83180-4 Get rights and content

Objective:

To assess the diagnostic potential of anti-neutrophil cytoplasmic antibodies (ANCA) and anti-endothelial cell antibodies (AECA) to distinguish early Kawasaki disease (KD) from febrile diseases resembling KD.

Design: Case-control study.

Setting: Tertiary care facility.

Patients: Eighteen patients with KD tested within 2 weeks of disease onset and before immune globulin therapy; 20 control children with fever and at least one other KD criterion; 21 children with noninflammatory disorders (patients scheduled for elective surgery, or after trauma).

Methods: We detected ANCA by immunofluorescence and enzyme-linked immunosorbent assay (ELISA), and AECA by a cell-ELISA with fixed human umbilical vein endothelial cells.

Results: We found that 7 of 18 patients with KD and 6 of 20 febrile control patients had ANCA (by immunofluorescence or ELISA, p value not significant); 3 of 18 patients with KD and 8 of 20 febrile control patients had AECA (p value not significant). One of three patients with KD who had aneurysms had ANCA; none had AECA.

Conclusions: The ANCA and AECA tests used in this study did not differentiate early KD from other childhood diseases with which it may be confused.

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There are no specific laboratory tests and the diagnosis is essentially clinical. Anti-neutrophil cytoplasmic antibodies may be present but are not diagnostic as they may also be positive due to infection.13 Intravenous immunoglobulins (2 g/kg) are the treatment of choice and will prevent the development of coronary artery disease if given early.
Fever is a common symptom in children and may sometimes be prolonged or recurrent. There are many differential diagnoses, which may lead to significant diagnostic delay. Diagnosis is based on the clinical presentation as well as a widespread panel of investigations that are necessary in order to exclude the many potential causes of fever before reaching a definite diagnosis. In particular, the physician will look for infections and malignancies before considering the disease as inflammatory. This chapter reviews the differential diagnosis of prolonged or recurrent fever, and discusses most of the inflammatory syndromes presenting with fever.
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However, later studies provided conflicting evidence on the effective role of AECA in KD. Guzman et al. [52] observed AECA in just 17% of 22 patients with KS and AECA were not able to differentiate KD from other febrile disorders of childhood resembling KD. Similar findings were reported by Nash et al. [53] in 58 children with acute KD, which showed no increased AECA reactivity compared to 35 children with febrile infections.
Anti‐endothelial cell antibodies (AECA) represent a heterogeneous family of autoantibodies directed against structural endothelial proteins, as well as antigens adhering to endothelial cells. Although AECA immunoassays still show a high‐interlaboratory variability, several findings suggest a pathogenic role of these autoantibodies in diseases characterized by endothelial damage. In this chapter, we analyze the knowledge about AECA prevalence, clinical relevance, and their pathogenic role in autoimmune diseases focusing in particular on systemic lupus erythematosus, antiphospholipid syndrome, systemic sclerosis (SSc), and systemic vasculitis.
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Kawasaki disease (KD) is the most common cause of vasculitis affecting children. Although the inflammatory response is found in medium and small vessels throughout the body, the most common site of end organ damage is the coronary arteries. Kawasaki disease is now recognized as the leading cause of acquired heart disease in children in the developed world. Despite appropriate therapy with intravenous gammaglobulin, affected children continue to develop coronary artery lesions. Many gaps exist in our knowledge of the etiology and pathogenesis of Kawasaki disease making improvements in therapy difficult. Despite the widely held belief that Kawasaki disease is an infectious disease, its etiology remains elusive. Many different etiologic agents ranging from bacteria to viruses have been identified, but no one causative agent has been consistently found. The etiology debate has centered around the mechanism of immune activation: conventional antigen versus superantigen. Superantigens (SAGs) encompass a family of proteins that are able to cause dramatic immune activation that is T cell dependent. A variety of microbes have the ability to produce superantigens and many of these have been implicated in the pathogenesis of human disease including Kawasaki disease. In this article, I will review the evidence pointing to superantigens as culprits in the immunopathogenesis of Kawasaki disease and provide a unifying hypothesis to account for the ostensibly opposing data used by proponents of both the conventional antigen and superantigen theories.

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¹: Supported in part by a grant from British Columbia's Children's Hospital, Vancouver, Canada.

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Original articleDiagnostic value of anti-neutrophil cytoplasmic and anti-endothelial cell antibodies in early Kawasaki disease1

Objective:

J Pediatr

Lancet

J Immunol Methods

Kawasaki disease

Curr Opin Rheumatol

A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome

N Engl J Med

Anti-neutrophil cytoplasmic antibodies in Wegener's granulomatosis recognize an elastinolytic enzyme

J Exp Med

Wegener's granulomatosis autoantibodies identify a novel diisopropylfluorophosphate-binding protein in the lysosomes of normal human neutrophils

J Clin Invest

Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis

N Engl J Med

Value of tests for antineutrophil cytoplasmic autoantibodies in the diagnosis and treatment of vasculitis

Curr Opin Rheumatol

Anti-endothelial cell antibodies in patients with Wegener's granulomatosis and micropolyarteritis

Clin Exp Immunol

Original article
Diagnostic value of anti-neutrophil cytoplasmic and anti-endothelial cell antibodies in early Kawasaki disease1