Coronary risk factors in Kawasaki disease treated with additional gammaglobulin

Abstract

Aims: To assess the hypothesis that an additional intravenous gammaglobulin (IVGG) infusion, if administered early, may prevent coronary artery lesions (CAL) in patients with Kawasaki disease (KD) who do not respond to initial IVGG therapy. Methods: Forty four KD patients (17 with CAL and 27 without CAL), treated with additional IVGG because of persistent or recrudescent fever after initial IVGG therapy, were studied. Main outcome measures were the presence of CAL by echocardiography and the number of febrile days before and after start of additional IVGG infusion (pre-and post-additional IVGG). Results: In univariate analyses, risk factors for CAL were the number of febrile days pre-additional IVGG, the number of febrile days post-additional IVGG, the number of days that initial IVGG was divided over, the white blood cell count pre-and post-additional IVGG, and the C reactive protein concentration preadditional IVGG. In a multivariate analysis, the only independent risk factor was the number of febrile days pre-additional IVGG (>10 days; odds ratio 7.86; 95% CI 1.44 to 42.8; p = 0.02). Conclusions: Among KD patients with persistent or recrudescent fever after initial IVGG therapy, administration of additional IVGG before the first 10 febrile days was associated with a decreased prevalence of CAL, when compared with the prevalence in those who were retreated later. An additional IVGG infusion, if administered early, may prevent CAL in initial IVGG non-responders.A pproximately 10-20% of patients with acute Kawasaki disease (KD) do not respond to standard intravenous gammaglobulin (IVGG) therapy and have persistent or recrudescent fever after completion of the infusion.

Predictive risk factors for coronary artery abnormalities in Kawasaki disease

“…Since we treated patients with IVGG as soon as the diagnosis was made, only the duration of the post-IVGG fever and total duration of fever could be reduced. About 10% of KD patients treated with IVGG do not respond, and these IVGG-non-responders are another risk group for CAA [1,3,6,12,15,16]. In this study, IVGG-non-responders accounted for 7.7% and shared only high baseline CRP with the CAA group.…”

“…IVGG non-responsiveness is also associated with CAA development [1,3,6,12,15,16]. Therefore, clearly defining and predicting IVGG non-responsiveness should assist of patients at high risk for CAA.…”

Immunoglobulin Resistance in Kawasaki Disease

“…Kawasaki disease children who are resistant or refractory to initial immunoglobulin therapy with recrudescence of fever are thought to be a ''hyperinflammatory phenotype'' and are considered to be at a higher risk for coronary artery aneurysms 3,[16][17][18][19] and other complications. 20,21 Several factors have been reported to be associated with refractory cases, including younger age, 22 male gender, 23 earlier presentation, 3 initial treatment before day 4 of fever, lower platelets, 3,22 lower hemoglobin, 3,24 higher white blood cell 25 and bands counts, 3 elevated C-reactive protein 3,19,22,24,26,27 and erythrocyte sedimentation rate, 18 elevated lactate dehydrogenase, 19,24 abnormal liver function, 3,19,22,23,26,27 lower serum albumin 23,28,29 and certain biomarkers, 30-33 among others.…”

Guía colombiana de práctica clínica para el uso de inmonuglobulinas en el tratamiento de reemplazo e inmunomodulación

“…(GRADE A1) 247,250,251,252,253,254,255 R El uso de IgIV en asociación con ácido acetilsalicílico a dosis antiinflamatoria disminuye de forma significativa la frecuencia de aneurismas coronarios. La dosis recomendada es de 2 g/kg en una sola dosis.…”

“…(GRADE A1) 247,250,251,252,253,254,255 R Los pacientes que continúan con fiebre (EK resistente) después de la primera infusión están en mayor riesgo de aneurismas y deben recibir una segunda infusión con la misma dosis de 2 g/kg. (GRADE A1) 247,250,251,252,253,254,255 Síndrome de choque tóxico El síndrome de choque tóxico estreptocócico (TSS) es una infección invasiva, especialmente por Streptococcus pyogenes. Se asocia con elevada mortalidad, hasta de 70 %, por falla multiorgánica.…”