AI Article Synopsis
- - A 12-week-old boy was diagnosed with incomplete refractory Kawasaki disease (KD) and later developed macrophage activation syndrome (MAS) after showing symptoms like irritability, pain, and vomiting for 10 days, despite not meeting all classic KD criteria.
- - Echocardiography revealed serious heart issues like pericardial effusion and severely dilated coronary arteries, leading to the diagnosis of incomplete KD, but initial treatments with intravenous immunoglobulin and aspirin failed.
- - His condition worsened and met the criteria for MAS, at which point treatment with high-dose anakinra was started, resulting in remission of inflammation.
Article Abstract
We report a 12-week-old boy presenting with incomplete refractory Kawasaki disease (KD) complicated with macrophage activation syndrome (MAS). The infant presented with cerebral irritability, pain, tachypnoea and vomiting for 10 days. He did not fulfil any of the classic diagnostic criteria for KD. Pericardial effusion on echocardiography in addition to severe dilatation of the coronary arteries in combination with leucocytosis and raised acute phase reactants led to the diagnosis of incomplete KD. Treatment with intravenous immunoglobulin and aspirin was initiated but without any response. The condition was subsequently refractory to additional treatment with infliximab and high-dose methylprednisolone. His condition worsened, fulfilling the criteria for MAS. High-dose anakinra was initiated, and remission of the inflammation was achieved.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6685371 | PMC |
| http://dx.doi.org/10.1136/bcr-2019-229708 | DOI Listing |
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