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Table 3 Summary of Published Series of Patients with Severe Kawasaki Disease Treated with Cyclophosphamide

From: Cyclophosphamide use in treatment of refractory Kawasaki disease with coronary artery aneurysms

Article

# of pnts

Age at Dx

Sex

CYC dose

Route

CYC duration

Prior Tx

Follow up

Outcome

Wallace CA et al, 2000 [9]

2

0.9 yr, 2.7 yrs.

Male

2 mg/kg/day

IV then PO

1.5-7mo

IVIG, IVMP

2.5 yr and 2.8 yrs.

Normal CAs at last follow up

Lucron H et al, 2004 [10]

4

0.3 yr, 0.8 yr, 2 yrs., 4.3 yrs

50% Male

10 mg/kg/day IV, then 2 mg/kg/day PO

IV then PO

IV 2–5 days, PO 6-12mo

IVIG, Plasma-pheresis

8 yrs. and 13 yrs

2/4 deceased, both with myocarditis and CAAs

Briceno-Medina M et al, 2016 [11]

1

12 yrs.

Male

15 mg/kg/day

IV

4 doses

IVIG, IVMP

3 yrs.

+ CAA, no stenosis or thrombosis

Current series, 2020

10

Median 2 yrs

50% Male

10 mg/kg/dose

IV

1–2 doses

IVIG, IVMP, IFX, CsA, ANA

Median 2 yrs. 4mo

Mean (median) z scores at last follow up: LAD 7.5 (5.0), RCA 8.3 (6.0)

  1. Pnts patients, Dx diagnosis, CYC cyclophosphamide, IV intravenous, PO oral, IVIG intravenous immunoglobulin, IVMP intravenous methylprednisolone, CAs coronary arteries, NL normal, CAA coronary artery aneurysms, z > 2.5, IFX infliximab, ANA anakinra, CsA cyclosporine A, LAD left anterior descending (proximal), RCA right coronary artery