Pediatric Rheumatology Online Journal

Vol 2, No. 2   2004

http://www.pedrheumonlinejournal.org

 

Kawasaki Disease in India

                       

Raju Khubchandani1  M.D.

Susan D’Souza2 D.CH., D.N.B.

 

Affiliations:

1. Consultant

Jaslok Hospital and Research Center, Peddar Road, Mumbai 400026

2. Senior Registrar

Breach Candy Research Hospital B. Desai Road, Mumbai 400026

 

Contact:

Dr. R.P. Khubchandani

31 Kailas Darshan, Kennedy Bridge, Mumbai 400007 INDIA

Tel: 022- 23865522

Fax: 022- 23898362

E-mail: dr_rajukay@hotmail.com

Conflict of interest: none

 

Keywords: Kawasaki Disease

 

 

Abstract:

Despite a high Asian incidence of Kawasaki disease (KD), there is a paucity of reports from India. We report a series of 6 children from Bombay and review published Indian literature (PUBMED search). We highlight certain features and problems of this disease that may be unique to our setting. We describe a new effort in India to educate our medical colleagues about KD and improve diagnosis and treatment.

 

Introduction:

Although KD has been reported from all over the world, Asia has reported a higher incidence in comparison to other continents. Only a paltry 38 cases from India have been reported in the pediatric literature over the last 25 years. This number of reported cases in one of the most populated countries of the world suggests gross under-diagnosis and under-reporting.

   

Case reports and review: 

Over a period of eight months, between March-October 2002, we diagnosed 6 children with KD using established clinical criteria. [1] All children in our series underwent echocardiography studies at diagnosis and at 6 weeks follow up, and received intravenous immunoglobulin (2gm/kg) with standard aspirin therapy. We reviewed Indian case reports utilizing a PUBMED search through March 2003 and found a total of 14 citations. (Table 1) There were 32 enumerated cases, of which 28 cases had been described in detail. This analysis includes the 28 cases described and our series of 6 patients.

Demographic distribution of our series and collated Indian data is shown in Table 2. More than 70% of the children fell in the typical age category of 1–5 years with the youngest child being 80 days old. A high male to female ratio of 10:1 was noted in the overall data. There were 5 atypical / incomplete cases described. Atypical presentations were seen in children outside the 1 to 5 year age range (4 of 5 patients), and 3 of them demonstrated echocardiographic abnormalities.

The prevalence of the diagnostic clinical features seen is summarized in Table 3.  Notably, amongst the diagnostic clinical criteria, lymphadenopathy was seen in 88% in our series. Of the other clinical features described (Table 4), irritability was by far the commonest followed by arthritis and diarrhea in equal measure. One child had reactivation of the BCG vaccination site. Six patients (18%) showed coronary/cardiac anomalies on 2 D echocardiography (Table 5). While 2 patients had developed coronary aneurysms, 3 others demonstrated dilatation of the coronary arteries and one child had mitral regurgitation as an isolated manifestation.

IVIG was not administered to 15 % of cases, and in up to 33% of cases cost constraints led to the use of staggered or incomplete treatment regimes (Table 6). Of the 6 patients with coronary involvement, 5 received IVIG. Three of these children showed complete regression of their cardiac lesions.

 

 Discussion:

In Japan the current reported incidence rate for KD is 120-150 cases per 100,000 children < 5 years. [2] A study conducted in California showed Asians to have the highest incidence as compared to others in the study population. [3] Similarly, a report from Birmingham, UK estimated the annual incidence of KD at 5.5 per 100,000 children < 5 years with the highest incidence being in the Indian subcontinent Asian children (14.6 per 100,000). [4] Despite this, reports from India are scant and the possible reasons for this are discussed below. We suspect that the gender bias in seeking medical attention may be responsible for the high male to female ratio reported from our country.

The incidence of lymphadenopathy seen in our population (88%) is higher than that reported in other studies. [5,6].   We speculate that this may be due to the high background prevalence of recurrent upper respiratory infections and poor dental hygiene in our population. The occurrences of atypical/incomplete KD (15%) and coronary involvement (15%) are slightly lower to those reported elsewhere [5,7]. With just a quarter of the patients receiving appropriate IVIG regime, one would have expected a higher rate of coronary involvement. We suspect that a focused search for coronary anomalies in KD by a cardiologist  who has had experience evaluating KD patients or who has completed pediatric cardiology training may have yielded a higher incidence of coronary involvement. 

          An interesting clinical sign that has been described is erythema and induration at the site of recent BCG immunization. This is an unusual sign that has been seen in young infants and is relatively specific to KD. It must be actively sought in countries where universal BCG immunization still prevails or in migrant infants from such countries. It occurs due to cross reactivity of T cells between specific epitomes of mycobacterium and human heat shock protein. [8] Interestingly, despite India adopting a immunization policy of BCG vaccination at birth, there was only one case (an eighty day old child) with this sign. This low incidence was probably due to the fact that most patients were outside the infancy stage.

          KD is a clinical diagnosis, based on the recognition of a constellation of diagnostic criteria. The lack of a definitive laboratory test makes bedside recognition even more imperative since early recognition and treatment with IVIG has been unequivocally shown to reduce the occurrence of coronary artery aneurysms. [8] The diagnostic clinical criteria of KD evolve sequentially and a diagnosis is often made over a temporal profile. With many of our patients utilizing multiple physicians (ophthalmologist for the red eyes, dermatologist for the rash and the otorhinolaryngologist for the neck swelling!), it is easy to understand how the diagnosis is missed. In our country this is compounded by the high background incidence of far more common conditions with fever and rash such as measles and other viral exanthems.

Furthermore, up to 10-45% of children are known to have an atypical or incomplete course in which diagnosis is possible only with a high index of suspicion, after echocardiography or on autopsy [9]. These atypical cases usually lack rash and lymphadenopathy while mucosal changes are nearly always found. As mentioned earlier, a paucity of trained pediatric cardiologists and poor physician awareness are major obstacles. All of these factors are likely to contribute to missed or delayed diagnosis and consequent delayed therapy. In many of these cases, physicians are faced with the predicament of prescribing the exorbitantly priced and scarce IVIG, often after the disease is beyond the tenth day. This in turn may often lead to the usage of modified IVIG regimes and suboptimal doses and therapy.

            With the high incidence of infectious diseases in India, which can have characteristics in common with KD, we believe that the first case of KD in a physician’s professional career in India may pose a major diagnostic dilemma. There is clearly a need to heighten physician awareness about KD in India and create KD physician interest groups. We successfully established a KD interest group as an activity of the local pediatric association (Bombay) in January 2003. The group disseminates awareness, helps colleagues with diagnosis, provides parent education, and logs cases as a local registry. At the time of writing this article in late 2003 the registry has enrolled 12 cases. Our goal is to establish these interest groups across India and gradually improve the diagnosis and treatment of KD in our country.

 

 

References:

1.      Shulman ST, Jamie De Inoencio, Hirsch R. Kawasaki disease. Pediatr Clin North Amer 1995, 42: 1205-22.

2.      Burns JC, Kushner HI, Bastian JF, Shike H, Shimizu C, Matsubara T, Turner CL. Kawasaki Disease – A brief history. Paediatrics 2000 Aug Vol 106 (2) p e27.

3.      Chang RK. Epidemiologic characteristics of children hospitalized for Kawasaki disease in California.. Paediatr Infect Dis J 2002 Dec; (12): 1150-5.

4.      Gardener-Medwin JM, Dolezalova P, Cummins C, Southwood T. Incidence of Henoch-Schonlein purpura, Kawasaki Disease, and rare vasculitides in children of different ethnic origins. Lancet 2002 Oct 19; 360(9341): 1197-202.

5.      Li T, Kawasaki T, Nakamura Y, Yanagawa H. Epidemiological picture of Kawasaki Disease in Beijing from 1995 through 1999. Pediatr Infect Dis J 2002 Feb;21(2):103-7

6.      Muzzafar MA, Al-Mayouf SM.Pattern of clinical features of Kawasaki Disease. Saudi Med J 2002 Apr 23(4):409-12.

7.      Falcini F, Cimaz R, Calabri GB, Picco P, Martini G et al. Kawasaki Disease in Northern Italy: A multicentric retrospective study of 250 patients. Clinical Exp Rheumatol 2002 May –June 20 (3): 421-6

8.  Brogan PA, Bose A, Burger D, Shingadio D, Tulloh R, Michie C, et al.

      Kawasaki Disease: An evidence based approach to diagnosis, treatment and

      proposal for future   research. Arch Dis Child. 2002;86(4): 286-290.

9.      Kusinska B, Kaluzewska-Wrolelewska M. What do we know about Kawasaki

      disease? Med Sci Monit, 2000;6(6):1227-1231.

 

 

Table 1 Indian citations up to March 2003 (PUBMED search)

 

 

First author

Journal (year)

No of cases described

Taneja A

Indian Pediatr.              1977

1

Nitsure MY

Indian Pediatr.              1988

1

Seshadri MS

J. Assoc. Phys India.   1989

1

Singh S

Indian Pediatr.              1996

1

Narayanan SN

Indian Pediatr.              1997

8

Singh S

Indian            Pediatr.               1997

6

Bavdekar SB

J. Indian Med. Assoc.  1998

1

Mitra S

Indian Pediatr.              2000

1

Paul DK

Indian Pediatr.              2000

2

Ghosh A

Indian Pediatr.              2000

1-(5-enumerated)

Pendse RN

Indian J Pediatr.           2001

2

Mishra D

Indian J Pediatr.           2001

1

Saraf S

Indian J Pediatr.           2001

1

Vijayalakshxmi AM

Indian Pediatr.              2002

1

 

Total cases

32

 

Our series

  6

 

 

 

 

 

Table 2 DEMOGRAPHIC DATA

 

Collated Indian data:

n = 28                  

            

Our series

n = 6

Total

n = 34

Age

< 1 year

1-5 years

> 5 years

Range

 

 4

21

3

80 D – 10 Y

 

0

4

2

3 Y – 6 Y

 

4 (12 %)

25 (74 %)

5 (14 %)

80 D – 10 Y

Sex

Male

Female

 

26

2

 

5

1

 

31 (91%)

3 (9%)

 

D= days

 

 

 

 

 

 

 

 Table 3: Frequency of diagnostic features

 

CLINICAL FEATURES

Collated Indian data: 28

Our series

6

Total

34   

INCOMPLETE/ATYPICAL*

4

1

 5    (15%)

ORAL CAVITY

27

5

32   (94%)

LYMPH NODES

 

24

6

30   (88%)

SKIN

 

26

5

31   (91%)

CONJUNCTIVA

 

23

4

27   (79%)

EXTREMITIES

22

2

24   (71%)

Time to diagnosis (days)

7-77 (specified in 8 cases)

6-14

-

* All males (ages- 80 days, 3 months, 5 years, 7 years, 10 years)

 

 

 

 

 

 

Table 4: Other clinical features

 

 

Collated Indian data:  28

Our series

6

Total

34

IRRITABILITY

 

21

5

         26 (76%)

ARTHRITIS

5

2

7(21%)

DIARRHEA

6

1

7(21%)

ASEPTIC MENINGITIS

2

1

3(9%)

HEPATOSPLENOMEGALY

3

0

3(9%)

PNEUMONIA

2

1

3(9%)

CARDIAC FAILURE

2

0

2(6%)

OTHERS

BCG reactivation (1), syncope (1), palatal palsy (1), neuropathy (1)

Otitis media (1)

-

 

 

 

 

 

 

 

 

 

Table 5: ECHO abnormalities:

 

                      

                                                                                               OTHERS (5/28)                                                                   OURS (1/6)

AGE/ SEX

 

 

1.5 Y/ Male

 

80 D/ Male`

9 M/ Male

10 Y/ Male

3M/ Male

4 Y/ Male

PRESENTATION

T

 

AT

T

 

 

AT

AT

T

CORONARIES

 

Dilatation

 

Aneurysm

Aneurysm