Familial Occurrence of Kawasaki Disease in an Indian Family

Kawasaki disease (KD), formerly known as the mucocutaneous lymph node syndrome, is an acute febrile illness of childhood seen worldwide. It is a vasculitis with a predilection for the coronary arteries, with approximately 20%-25% of the untreated patients experiencing coronary artery abnormalities, including aneurysms. Attempts to find an etiological agent for Kawasaki disease, one of the leading causes of acquired heart disease in children, have not found a definitive answer, although epidemiological studies support infectious origin. Continued research to solve the mystery of KD has found an interesting aspect to the occurrence of the disease in the form of its familial occurrence. Surveys from Japan and North America have reported KD in family members, siblings and offspring, suggesting a possible genetic predisposition for the occurrence of the disease and for the involvement of coronary vessels [1] [2]. There have been reports of recurrence of KD with coronary artery involvement in siblings [3]. There is not much information on the familial occurrence of KD in Indian population. Herewith presenting an Indian child with KD whose cousin had suffered from the same disease earlier on, suggesting familial occurrence of KD in Indian population.

Case 1: The Patient

An 8-year-old male child presented with history of high grade intermittent fever of 5 days, with redness of tongue and peeling of the skin of hands and feet for 3 days. He was the only child born to non-consanguineous parents. He has no significant medical or past history. On examination (Figure 2), he was febrile, with bilateral bulbar conjunctival congestion, strawberry tongue, angular cheilitis, periungual desquamation (Figure 3), bilateral cervical lymphadenopathy and mild hepatomegaly. A diagnosis of KD was made based on his clinical presentation. Following admission, his initial blood investigations showed leukocytosis with normal platelet count, and raised erythrocyte sedimentation rate (ESR) and raised C-reactive protein (CRP). Echocardiogram done at admission revealed dilated left coronary artery. Child was treated with intravenous immunoglobulin (IVIg) and aspirin. Fever subsided subsequently by fourth day of treatment. His condition improved significantly and was later discharged from the hospital. On follow-up after 3 weeks, repeat echocardiogram revealed normal left coronary artery, showing improvement from the previous findings.

Case 2: The Cousin

The maternal cousin of the above mentioned child was admitted with similar complaints 5 months previously at our hospital. The second case was also a male child, aged 4 years, who presented with a history of high grade fever for 7 days. He had no significant medical or past history. On examination, the child had bulbar conjunctival congestion, desquamation of the oral mucosa and peeling of the skin on hands and feet. He also had significant cervical lymphadenopathy. Based on these signs and symptoms, a diagnosis of KD was made, and he was admitted to the hospital for further management. At admission, the blood investigations revealed leukocytosis with normal platelet count, and raised ESR and CRP. Echocardiogram done at admission was normal. The child was also treated with IVIg. He responded well to the treatment, showing improvement over a period of five days.

Familial occurrence of KD has been well documented by the Japanese researchers. Studies from America have documented that the disease is more common among Asians and Asian-American populations [4]. In Japan, the siblings of an index case were found to have 10-fold increased relative risk of KD. The incidence of KD is 2-fold higher in Japanese parents of children with the disease. The incidence of recurrent KD in siblings is 5 to 6 times higher in multi-generational KD families as compared to those with only one affected child.

Familial occurrence points towards possible genetic predisposition and also helps in understanding or predicting the severity and course of the disease, including the risk of coronary artery involvement. It has been reported in previous studies that the disease severity and risk of coronary involvement is higher in those with familial occurrences [5].

This case report highlights the familial occurrence of KD in Indian population.

 Wang CL. Kawasaki Disease: Infection, immunity and genetics. Pediatr Infect Dis J. 2005;24:998-1004.

2- Yanagawa H, Yashiro M, Nakamura Y, Kawasaki T, Kato T. Results of 12 nationwide epidemiological incidence surveys of Kawasaki disease in Japan. Arch Pediatr Adolesc Med. 1995;149:779-783.

3- Matsubara T, Furukawa S, Ino T, Tsuji A, Park I, Yatuba K. A sibship with recurrent Kawasaki disease and coronary artery lesion. Acta Peadiatr. 1994;83:1002-1004.

4- Dergun M, Kao A, Hauger SB, Newburger JW, Burns JC. Familial Occurrence of Kawasaki Syndrome in North America. Arch Pediatr Adolesc Med. 2005;159:876-881.

5- Uehara R, Yashiro M, Nakamura Y, Yanagawa H. Kawasaki disease in parents and children. Acta Pediatr. 2003;92:694-697.