Tareen Page 1

Clinical spectrum of Rheumatic Fever in Baolchistan

Dr Rizwana Tareen*, Dr Mobin Ur-Rehman**, Dr Mujeebullah Tareen***, Binav

Shrestha****
*E-mail:[email protected], Telephone:. **E-mail: [email protected], Telephone:
+923138294497,***E-mail:[email protected],Telephone +923448002324****Email: [email protected], Telephone: +923337957577.
Dr Rizwana Tareen: Assistant Professor, Department of Paediatrics, Unit-I , Bolan
Medical College
Dr Mobin Ur Rehman: Assistant Professor, Department of Paediatrics, Unit-1, Bolan
Medical College, Quetta
Dr Mujeebullah Tareen, Assistant Professor, Department of Cardiology , Bolan
Medical College
Binav Shrestha, Department of Paediatrics

Abstract
Objective: To describe the clinical spectrum of Rheumatic fever patients in Baluchistan.

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Patients and Methods: This study was carried out in the Department of Paediatric
Medicine Bolan Medical Complex Hospital from September 2010 to December 2011. 52
children between 4-14 years of age with positive modified Jones criteria for Rheumatic
fever were included in this study. Patients with other rheumatic conditions and without
supporting evidence of antecedent infection were excluded. Data was collected using a
proforma which included detailed history, examination and lab tests. Auscultation findings
and Doppler echocardiography were used to diagnose carditis. Patients were treated as per
the protocol. Results: 30 patients out of total 52 i.e. 56.769% patients presented with
initial presentation whereas 22 (42.305%) with complaint of recurrent episode.
37(71.51%) presented with carditis. 18(60%) and 19(86.36%) had carditis in initial
presenting group and recurrent presentation respectively with (RR 0.6947 (95%CI 0.49640.9722) P 0.038112. Overall 23.08% had pericarditis. 48.08%, 19.23% and 5.77% had
Mitral regurgitation, combination of Mitral regurgitation with aortic regurgitation and
aortic regurgitation respectively. Mitral regurgitation was statistically significant in the
initial presenting group (RR 3.85 (1.5391-9.6306) P=0.00022. arthritis (65.38%) and
chorea (9.62%) was seen slightly higher in the initial presenting group. Subcutaneous
nodules and erythema marginatum was present in 7.69% and 3.85% respectively. Minor
manifestations i.e. fever (86.54%) arthralgia(21.15%), prolongs PR interval (5.77%) and
sore throat(51.92%) all were higher in initial presenting group. Conclusion: Rheumatic
heart disease remains a main cause of morbidity in the patients diagnosed in recurrent and
initial episodes Rheumatic fever.
Key Words: Rheumatic Fever Baluchistan Mitral Regurgitation

Background
Incidence and prevalence of rheumatic fever and RHD shows very significant global
variation. The greatest burden has been found in sub-Saharan Africa, the lowest in North
America[1]. While the incidence and mortality of ARF and RHD have decreased
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drastically in the affluent industrialized countries of Europe, North America, and in Japan,
the disease is a major health problem in the less affluent, 'developing' countries of Latin
America, the Middle East, Africa, India and Southeast Asia[2, 3]. In the developing
countries of the world, rheumatic fever and rheumatic heart disease remain significant
medical and public health problems [4]. Over the past 20 years, there is no significant
decline in the percentage of rheumatic fever and rheumatic heart disease cases being
admitted to a major government hospitals in India[5, 6]. This high frequency can be
linked to lack of early detection of the disease at primary level, poor management of throat
infections and poor rheumatic fever prophylaxis at community level[7]. High morbidity
and mortality due to Rheumatic heart disease (RHD) associated with females is mainly
because of late diagnosis on one hand and socioeconomic reasons on the other hand. Poor
referral to tertiary care centers leads to delayed diagnosis which results in
complications[7]. Climate and geography appear to bear little relationship to the incidence
and severity of ARF[8]. The application of Jones criteria for diagnosis remains relevant,
though echocardiography is increasingly called upon to 'confirm' clinical diagnosis and
help manage these patients in an appropriate manner[9, 10]. The most effective approach
for control of ARF and RHD is secondary prophylaxis, which is best delivered as part of a
coordinated control programme[11]. This study outlines the clinical profile of patients with
Rheumatic fever in Baluchistan.
Patients and Methods
This cross sectional descriptive study was done at Pediatric Department Bolan Medical
Complex Hospital and it lasted for 14 months. Total of 52 children with acute rheumatic
fever were included who fulfilled the following criteria: children diagnosed as suffering
from rheumatic fever based on Revised Jones criteria, children of both genders and
children between ages 4-14 years. Children with the following features were excluded
from this study: children with evidence of other rheumatic conditions, children without
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supporting evidence of antecedent group-A streptococcal infection, age greater than 14
years and less than four years. After informed consent from the patients data was collected
using a proforma designed for this study. Detailed history of all the patients was taken to
determine antecedent history of sore throat and socio-economic conditions. Thorough
examination was then carried out with special attention towards the cardiac auscultation
findings. Revised Jones criteria with no previous history of acute rheumatic fever was
taken as the criteria for the initial episode of acute rheumatic fever while recurrent episode
was diagnosed if reappearance of 2 major or 1 major and 2 minor manifestations of
rheumatic fever was present along with evidence of streptococcal infection. Every patients
complete blood count, erythrocyte sedimentation rate (ESR), urine analysis, C reactive
protein, chest X-ray, ECG, echocardiography, throat culture and Antistreptolysin O titer
tests were performed to support the physical findings. Auscultation findings and Doppler
(2D Doppler and color Doppler) echocardiography were used to diagnose carditis as per
revised Jones criteria. Carditis was labeled as mild if only precordial murmurs were
present and it was considered severe if signs and symptoms of heart failure and/or
cardiomegaly were seen on chest X-ray. The socioeconomic standard was set arbitrarily
depending upon the monthly income of the family. If income was < Rs.5000/month it was
labeled as lower, between Rs.5000 to Rs.15000/month as lower middle, Rs.15000 to
Rs.50000/month as upper middle and >Rs.50000/month was labeled as higher
socioeconomic class. Bed rest was advised to all the patients until the ESR was near
normal. Benzathine penicillin (0.6 million units for children weighing <27Kg and 1.2
million units for those weighing >27Kg) was administered to all the patients after a test
dose. First dose of Benzathine penicillin was administered following admission in ward
and was repeated at the intervals of 2 and 4 weeks as a secondary prophylaxis. Aspirin was
used for inflammation (100mg/kg/day in 4 divided doses initially and 75mg/kg/day after 2
weeks until ESR returned to normal). Steroids (2mg/Kg/day) were administered in
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conditions of severe carditis. Digoxin (10g/Kg/ day) was given to all the patients with
carditis. Haloperidol (0.25-0.5mg/Kg/ day) was administered for 3 months in patients with
chorea. Data analysis was performed using SPSS for windows version 10 and percentages,
relative risk, confidence interval and p values were calculated for all variables.

Results
Form the total of 52 children included in our study 30 (57.69%) children came to us with
first episode and 22(42.305%) came with complaint of recurrent episode. 55.8% of the
study population fell in the age group of 7 to 11 years and the mean age was 9 years. The
male to female ratio was 1.17:1 with 28(53.8%) male and 24 (46.2%) female participants.
32(61.54%) patients belonged to lower, 16 (30.77%) from lower middle and 4 (7.69%)
patients were from upper middle class. None of the patients were from the upper class.
Among the major manifestations Carditis was present in total of 37 (71.15%) patients 18
(60%) presenting as the initial presentation and 19 (86.36%) presenting in recurrent
presentation (RR 0.6947 (95%CI 0.4964-0.9722) P 0.038112. 12 (23.08%) patients had
congestive cardiac failure during the course of the study, 5(16.67%) in initial presentation
and 7 (31.82) patients in recurrent episode (RR 0.5238 (95%CI 0.1913-1.4341) P
0.200325). Total of 5(9.62%) patients had pericarditis, out of which 2(6.67%) belonged to
the initial episode group whereas 3(13.64%) belonged to the recurrence group (RR
0.4889(95%CI 0.0891-2.683) P 0.6391). Mitral regurgitation was common finding with
25(48.08%) patients affected in total. The initial episode group had significantly high
number of 21(70.00%) cases while the recurrent presenting group had only 4(18.18%)
cases (RR 3.85 (95%CI 1.5391-9.6306) P 0.00022). Mitral regurgitation along with aortic
regurgitation was found in 10(19.23%) patients, 4(13.33%) of them from the initial
episode group and 6(27.27%) of them from the recurrent episode group (RR 0.4889 (95%
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CI 0.1565-1.5276) P 0.29). Aortic regurgitation alone was found in only 3(5.77%) patients,
2 (6.67%) cases in initial episode group and just 1(4.55%) case in the recurrent group (RR
1.4667, 95% CI 0.1418-15.1742, P 1). Mitral stenosis was present in only 2(3.85%)
patients in total all of which reported from the recurrent episode group (9.09%).
Migratory polyarthritis was present in total of 34(65.38%) patients. 22(73.30%) of them
reported from the initial episode group of children and 12(54.40%) form the recurrent
episode group (RR 1.3444(95% CI 0.8674-0.8674) P 0.15939). Total of 5(9.62%) patients
suffered from the chorea. Initial episode group of children had just 4(13.30%) chorea
whereas among the recurrent episode group only 1(4.50%) reported it (RR 2.9333(95% CI
0.3517-24.4664) P 0.381383). Skin lesions found were 4(7.69%) cases of subcutaneous
nodules and 2(3.85%) cases of erythema marginatum.
The minor manifestations which were seen in this study were the fever in 45(86.54%),
arthralgia 11(21.15%), increases PR interval in ECG in 3(5.77%). Out of the total children
suffering from fever 25(83.30%) reported from the initial episode group and rest of the
20(91%) reported from the recurrent group( RR 0.9167 (95% CI 0.7449-1.1281) P
0.68453). arthralgia was seen in 5(16.70%) patients in the initial episode group and
6(27.30%) from the recurrent group( RR 0.6111(95% CI 0.2135-1.7492) P 0.49459).Only
3 patients had PR interval prolongation in ECG, 1(3.30%) from the initial episode group
whereas 2(9.10%) presenting from the recurrent group(RR 0.3667 (95% CI 0.03543.7936) P 0.566968).
Throat swab culture for Hemolytic streptococci was positive in 14 out of 52 patients
(26.92%). Anti Streptolysin O titer was more than 320 Todd units in 48(92.308%)
children. All the patients had Erythrocyte sedimentation rate more than 12mm in first hour
but among them only 46 children had erythrocyte sedimentation rate more than 90mm in
the first hour.
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Discussion
The mean age in our study was 9 years while it was found to be quite old in Africa[12]
while in Australia and Pakistan it was found to be closer to us [13, 14]. In our study the
male female ratio was 1.7:1 which has been seen in Eastern Europe[15] while most studies
show a female preponderance[16]. Most of the patients in this study were poor, and this is
consistent with international studies[17-19] and it is agreed that this disease is a function
of poverty, low socioeconomic status, and barriers to healthcare access[20]. Hence it is not
unusual that these diseases are found in poor and female gender[17]. Carditis was the main
feature present in this study in 71% of the population, Carvalho and workers found it to be
56% [21], while Caldas found it to be 48%[22]. Other studies done in the region confirm
our finding showing a higher frequency of carditis [4, 14]. It could be that genetic factors
are involved and the frequency of carditis is higher in Asian people. Heart failure was the
presentation of carditis in 47% of patients, it is significantly lower than African workers
have shown [12, 23]. Again our findings are closer to other studies done in this part of the
world [24, 25], thus implying the interplay of genetic factors. The frequency of pericarditis
was 13 % and it was similar to other studies done in Asia [26]. None of our patients went
into cardiac tamponade which is documented by other workers [27]. Mitral regurgitation
was the most important finding present in 48% of patients, it is lower then found out by
workers in Ivory Coast [12], but again it was closer to a study in Ankara[28]. This again
may signify hirthto unknown genetic factors. Both Mitral and Aortic valve regurgitation
were found in 19 % of patients, however it was found 23% in an African study[29]. In
another Pakistani study by Masood in Lahore it was shown to be 10%[14]. Pure aortic
regurgitation was found in only 5 % of patients in our study , while in a study in Cameroon
it was found to be 15%[29] and 40 % in on Sudanese study[30]. Therefore, like combined
lesions the frequency of Aortic regurgitation also varied from one geographic location to
another. Pure Mitral Stenosis was found to be present in almost 4% of cases, while another
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Pakistani study found it to be 44%[7]. This is probably due to the fact that this study was
in women, while our study group included children and Mitral stenosis is a late
complication. In Africa the frequency of Pure Mitral stenosis has been found to be much
higher (15%),[29] which can again only be attributed to genetic or socioeconomic factors.
Arthritis was the predominant feature in the first presentation and our finding is reiterated
by numerous studies [10, 31]. Canter suggests that as the frequency of carditis in
rheumatic fever is falling, the frequency of Arthritis and Chorea is increasing[32]. In our
study 13% had chorea, which is consistent with international literature [33] [13]. Our
frequency of subcutaneous nodules was higher than reported in South America [21] and
Italy [34]. Further studies are needed to find this association.
Fever was the most common minor manifestation and this is consistent with international
literature[35]. Arthralgia was the second most common manifestation although it was less
then shown by our Nepali colleagues[24]. Our study did not find any relation of laboratory
tests with the severity of the disease and in this we are supported by international data[25].
Conclusions
Our data show that Rheumatic fever is still an important cause of cardiac morbidity and a
large proportion of the patients already had complications at diagnosis.

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22
30

initial
recurrent

Figure 1: Initial versus Recurrent Presentation of Rheumatic Fever

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70.00%

61.54%

60.00%
50.00%
40.00%

30.77%

30.00%
20.00%

7.69%

10.00%
0.00%

0.00%

Figure 2: Socioeconomic status of Patients in the study

53.80%

46.20%

male

Figure 3: Gender wise distribution of Patients

Rheumatic fever

female

Tareen Page 13

86.364%

73.3%

70.0%
60.0%

54.400%
Initial Rheumatic Fever

Recurrent Rheumatic Fever

31.818%
27.273%
16.7%

18.182%
13.636%

13.3%

6.7%

carditis

ccf

pericarditis

13.3%
9.091%

6.7%
4.545%

mr

MR+AR

AR

4.500%
0.0%
MS

Figure 4: Clinical Features of Patients in initial and recurrent presentations

Rheumatic fever

arthritis

chorea

Tareen Page 14

Minor Manifestations of Rheumatic Fever

91.00%
83.3%
Initial Rheumatic Fever

Recurrent Rheumatic56.7%
Fever
45.40%

27.30%
16.7%
3.3%
fever

arthralgia

9.10%

PR prolonged

sore throat

Figure 5: Minor manifestations of Rheumatic Fever in initial and recurrent presentations

Table 1: Major manifestations of Rheumatic Fever

Manifestation Total Total
Initial
% of Recurrent
% of
Risk 95%
P
%
episode initial
episode
recurrent Ratio
CI
Value
episode
episode
37
71.154
18
60.00%
19
86.364%
0.6947 0.4964- 0.038112
Carditis
CCF

12

Pericarditis

Rheumatic fever

%
23.077
%
9.615%

16.67%

31.818%

0.5238

6.67%

13.636%

0.4889

0.9722
0.19131.4341
0.0891-

0.200325
0.6391

Tareen Page 15
MR

25

MR+AR

10

AR

MS
Arthritis

2
34

chorea

3.846%
65.385
%
9.615%

Subcutaneou
s nodules
Erythema
marginatum
Minor
Manifestation
fever

7.692%

3.846%

45

25

83.30%

20

91.00%

0.9167

arthralgia

11

16.70%

27.30%

0.6111

PR prolonged

86.538
%
21.154
%
5.769%

3.30%

9.10%

0.3667

sore throat

27

51.923
%

17

56.70%

10

45.40%

1.2467

Rheumatic fever

48.077
%
19.231
%
5.769%

21

70.00%

18.182%

3.85

13.33%

27.273%

0.4889

6.67%

4.545%

1.4667

0
22

0
73.30%

2
12

9.091%
54.40%

1.3444

13.30%

4.50%

2.9333

2.683
1.53919.6306
0.15651.5276
0.141815.1742
0.86740.8674
0.351724.4664

0.74491.1281
0.21351.7492
0.03543.7936
0.7162.1705

0.00022
0.29
1

0.15939
0.381383

0.68453
0.49459
0.566968
0.57514