Kawasaki’s disease is an acute febrile illness seen in young children which may lead to life-threatening vasculitis with predilection for the coronary arteries. Untreated, 25% of cases may develop coronary artery aneurysms. However with conventional treatment of IVIG and high dose aspirin, only 4% are known to develop coronary artery aneurysm. For patients with immunoglobulin resistant Kawasaki’s disease, retreatment with IVIG, methyl prednisolone, cyclophosphamide and even Infliximab have been found useful. Most of the refractory Kawasaki’s disease respond to 2nd dose of IVIG and rarely require further treatment with intravenous methyl prednisolone. We report 2 cases of Kawasaki’s disease who developed coronary aneurysms inspite of 2 courses of IVIG and subsequently responded to intravenous methyl prednisolone.
First case was an eleven months old boy who presented with fever since 15 days, rash and diarrhea since 6 days. On examination, he had pallor, erythematous rash over trunk and back, palmar & plantar edema and erythema, multiple cervical lymphadenopathy, conjunctival congestion and strawberry tongue with dark red lips. Investigations showed anemia, leucocytosis and thrombocytosis. His C-reactive protein (CRP) was positive and ESR was 35 mm at end of 1 hour. Urine routine showed uncountable pus cells with culture growing E.Coli (108/ml). Echocardiography showed mild dilatation of left coronary artery (2.4 mm). He was treated with IV antibiotics and IVIG (2 gm/kg) along with high dose aspirin. After 15 days, a repeat echocardiography revealed mild aneurysm at the ostium of left coronary artery. He was retreated with IVIG. However, his clinical features persisted even after 10 days after retreatment. He was than treated with IV Methyl Prednisolone (30 mg/kg) along with blood transfusion (in view of anemia) following which his clinical symptoms resolved and laboratory parameters also started normalizing.
Second case was a two and a half year old boy with fever and cold since 15 days, bilateral swelling over wrist since 6 days and rash over trunk and face since 2 days. On examination, he had multiple cervical lymphadenopathy, palmar and planter erythema, bilateral wrist swelling with restriction of movement, erythematous rash over trunk and back and right sided pneumonia. Investigation showed thrombocytosis, leucocytosis, anemia, elevated ESR with positive CRP, X-ray chest showed right lower zone consolidation and echocardiography revealed dilated left coronary artery at the ostium (2.8 mm). He was treated with IV Antibiotics, IVIG (2 gm/kg) and high dose Aspirin (100 mg/kg/d). Even after 10 days after IVIG, his clinical symptoms persisted. He was thus given a second course of IVIG (2 gm/kg). After 8 days after 2nd course of IVIG, a repeat echocardiography revealed aneurysm at the ostium of left coronary artery. He was then treated with IV Methyl Prednisolone (30 mg/kg) to which he responded.
|
