Resistant KD

Additional IVIG shows 71% response & 30% resistance. In such cases Steroid pulse therapy (Methyl Prednisolone) to resistant IVIG cases may give 100% response. Authors feel that we should start combination of drug therapies in in all IVIG resistant cases, in which steroid pulse therapy could be essential to prevent or further damage of coronary arteries (29,47). We don’t want to see further risk in developing coronary aneurysms in recrudescent fever. Management protocol for IVIG resistant group is given in the table as per the personal experiences and may vary from person to person. CRP level of >8mg/dl after initial IVIG are likely to fail additional IVIG. Plasma exchange may be considered as another mode of treatment if you have facility for plasmapheresis under an experienced hematologist to remove circulating immune complexes, thus we can save the cost of IVIG in developing countries and age also matter. Younger children cannot undergo plasmapheresis due to various procedural reasons. Different scoring systems were developed by various authors as per their experiences.

KD Shock Syndrome

The incidence of KD shock syndrome (KDSS) is estimated to be ≈7%. KDSS can be defined as the presence of hypotension and shock requiring the initiation of volume expanders, the infusion of vasoactive agents, or transfer to intensive care units. Shock in KDSS is often moderate, with low lactate values and the need for treatment with inotropic and vasopressor agents. Although hemodynamic instability generally improves quickly once therapy with diuretic agents and vasopressor agents is initiated, a mild degree of ventricular diastolic dysfunction can persist after acute management. The causes of KDSS may involve the release of endogenous molecules that mediate a decrease in peripheral vascular resistance, myocardial dysfunction from myocarditis with or without myocardial ischemia, and capillary leakage, but the exact underlying mechanisms remain unclear. KDSS is often associated with more severe laboratory markers of inflammation and higher risk of coronary arterial dilation. Such cases are also more likely to be resistant to IVIG therapy and to require additional anti-inflammatory treatment.

First line Therapy in Resistant Kawasaki Disease (Modified from AHA)

Second dose of IVIG 2 gram /kg/bwt
IVIG + Prednisolone IVIG: 2 g/kg IV + Prednisolone 2 mg/kg/day, divided every 8 h until afebrile, then prednisone orally until CRP normalized, then taper over 2–3 wk
IVMP Advised not with IVIG but can be tried separately Intra venous methyl prednisolone (IVMP) 30 mg/kg/ body weight over 2-3 hrs x 3days. Then followed by oral 2-4wks
or Infliximab* 5 mg/kg/bwt single infusion x 2hrs + IVMP
or Etnarcept 0.8 mg/kg/bwt/sc/wk +IVMP
or Ulinastatin 20000 -30000 unit/kg/ bwt od IV x 3days + IVMP
Cyclosporin-a 3-8 mg/kg/bwt /day PO x 5-6 days depends on a febrile period
(keep serum level 400-500ng/ml).
Anakinra 2-6 mg/kg/day by subcutaneous injection.

# Plasma exchange and methotrexate/ may be the final choice for refractory KD.

* Infliximab may be considered as preferred first line therapy in resistant KD (desired drug of Indian Society of Kawasaki disease). Steroid may beaded as per the severity of CAL