158 - CICLOSPORIN IS USEFUL IN SEVERE KAWASAKI DISEASE: EXPERIENCE FROM A TERTIARY CARE CENTRE IN NORTH-WEST INDIA

Background: Approximately 15-20% of patients with KD remain refractory to conventional IVIG therapy. This subset of patients has a higher propensity to develop CA abnormalities (CAAs) with severe manifestations including macrophage activation syndrome (MAS), KD shock syndrome (KDSS) and atypical manifestations. We report the role of ciclosporin in 31 children with KD from a tertiary centre in India.

Methods: A review of medical records of all patients who were diagnosed to have KD during the period January 1994 - June 2023 in Paediatric Allergy Immunology Unit, Advanced Paediatrics Centre. Postgraduate Institute of Medical Education and Research, Chandigarh, India was done. Case records of children with KD who had received cyclosporine were analysed in detail.

Results: Of the 1245 patients with KD, 31 patients (22 boys) received ciclosporin. Median age at diagnosis was 2 years (range 0.3-13 years). Indications for using ciclosporin were MAS (2/31); KDSS (5/31), presence of CAAs (27/31); both KDSS and CAA (4/31); and pancreatitis (1/31). Amongst the patients who had CAAs. 8 (26%), 2 (6%) and 17 (55%) had small, medium, and giant aneurysms respectively. All patients had received IVIG (2g/Kg), corticosteroid (methylprednisolone 30 mg/kg/day for 3 days followed by oral taper. The dose of ciclosporin was 5 mg/kg/day, and it was tapered and stopped after a duration of 4 to 6 weeks. One patient died during hospital stay due to refractory MAS (he had also received tocilizumab and etoposide). Dimension of large CAAs (moderate and giant CAAs by z-score) normalised and/or reduced in size in 7/31 (23%) after a median duration of 540 days (range 330-730 days). Small CAAs normalised in 7/8 (88%) patients after a median duration of 10.5 days (range 2-35 days). There were no adverse effects or significant infection during therapy over a cumulative follow up of 21596 patient days.


Conclusions: Our findings suggest that ciclosporin holds promise as adjunctive therapy for intensifying treatment in IVIG non-responders and managing severe KD cases. Importantly, no adverse events were reported during the short course ciclosporin therapy, highlighting its safety profile.