208 - REFRACTORY KAWASAKI DISEASE, A CHALLENGE FOR MEDICINE

Background/

Aim: Opportune diagnosis of KD is a challenge for pediatricians, mainly because there are no specific tests, and clinical criteria originally described in 1967 are still valid. Laboratory changes in the acute phase of the disease are not specific or diagnostic. Timely diagnosis and early treatment can reduce coronary risk from 30% to 5%. However, there are patients whose outcome is not favorable despite properly treatment. The objective of this case is to analyze the treatment used and the presence of coronary lesions in the acute stage of patients with KD.

Materials and methods: Retrospective, observational study in a patient diagnosed with KD.

Results: This is a 2-year-old male at the time of presentation, with fever for 3 days, bilateral conjunctival injection, cervical lymphadenopathy, maculopapular diffuse erythoderma, cracking of lips, edema of hands and feet, who also presented irritability, hyponatremia, increased ALT and lymphopenia, CRP 92mg/L, ESR 30mm/h, for which a diagnosis of KD was integrated, starting management with IVIG 2gr/kg, acetylsalicylic acid 50mg/kg, methylprednisolone 2mg/kg, with initial ECHO without evidence of aneurysms, then in second ECHO there were small aneurysms, so we gave infliximab 5mg/kg and azathioprine 1.7mg/Kg, then the aneurysms continue growing so we initiated a second dose of IVIG 2gr/kg, pulses of methylprednisolone 30mg/kg, however, there was a persistence of progression to medium-sized aneurysms; it was decided to give a dose of cyclophosphamide 750mg/m2/doses and when new progression to giant aneurysms was evident, 5 sessions of plasmapheresis was established, managing to stop the inflammatory process. We continue with follow-up and monitoring with ECHO, currently still being managed with azathioprine and antiplatelet agents with good response.

Conclusion: There is a genetic predisposition that directly influences the response of each patient and the evolution of the disease, regardless of the time of diagnosis and initiation of treatment.

Although it is true that there are no guides that mention schemes endorsed by the study population on the use of other biological agents, so the making of these decisions will depend on the experience of each rheumatologist.

In spite of no studies on the use of plasmapheresis in pediatrics, we strongly believe that this is a method that should be used in patients with refractory and rapid progression of aneurysms. As a national reference center we have been able to verify the response and the end of the inflammatory process in patients diagnosed with KD.