Intravenous Immunoglobulin in Kawasaki Disease-Evolution and Pathogenic Mechanisms.
Pallavi L NadigVibhu JoshiRakesh Kumar PilaniaRajni KumrahJayakanthan KabeerdossSaniya SharmaDeepti SuriDeepti SuriSurjit SinghPublished in: Diagnostics (Basel, Switzerland) (2023)
Kawasaki disease (KD) is an acute vasculitis of childhood that affects the medium vessels with a special predilection to the involvement of coronary arteries. The major morbidity of this disease is due to coronary artery aneurysm, which occurs in about 25-30% of untreated cases. For decades now, intravenous immunoglobulin (IVIg) has consistently been shown to reduce the risk of CAAs to less than 5%. However, the mechanism of immunomodulation remains unclear. Several studies on the role of IVIg in the modulation of toll-like receptor pathways, autophagy, and apoptosis of the mononuclear phagocytic system, neutrophil extracellular trap, and dendritic cell modulation suggest a modulatory effect on the innate immune system. Similarly, certain studies have shown its effect on T-cell differentiation, cytokine release, and regulatory T-cell function. In this review, we discuss the potential mechanisms underlying the immunomodulatory actions of IVIg in patients with Kawasaki disease. Furthermore, we provide a summary of the evidence regarding various infusion protocols and dosages utilized in the treatment of KD patients.
Keyphrases
- coronary artery
- toll like receptor
- immune response
- dendritic cells
- end stage renal disease
- pulmonary artery
- endoplasmic reticulum stress
- oxidative stress
- cell death
- nuclear factor
- newly diagnosed
- ejection fraction
- chronic kidney disease
- high dose
- inflammatory response
- coronary artery disease
- liver failure
- prognostic factors
- peritoneal dialysis
- case control
- transcription factor
- signaling pathway
- regulatory t cells
- heart failure
- low dose
- respiratory failure
- left ventricular
- risk assessment
- young adults
- cell cycle arrest
- cell proliferation
- patient reported outcomes
- pulmonary hypertension
- drug induced