Demographic, clinical, laboratorial, and radiological characteristics of rheumatic fever in Brazil: systematic review

Abstracts

A febre reumática (FR) se caracteriza por um processo inflamatório não supurativo que se instala após uma infecção pelo estreptococo beta-hemolítico do grupo A de Lancefield. Sua prevalência é maior nos países em desenvolvimento como o Brasil. Em nosso país, entretanto, dados epidemiológicos sistemáticos sobre a doença são incompletos e escassos. Estima-se uma prevalência de FR ao redor de 3% entre as crianças e os adolescentes brasileiros. Neste artigo, foi realizada uma revisão sistemática dos principais estudos brasileiros, utilizando como fonte de pesquisa as bases de dados do LILACS, Scielo e Medline, tendo como palavras-chave Febre Reumática e Rheumatic Fever. Foram selecionados 10 trabalhos epidemiológicos e a análise comparativa não demonstrou diferenças em relação a predominância de sexo, quadro clínico, laboratorial e radiológico dos pacientes nas diversas regiões do país.

febre reumática; Brasil; epidemiologia

REVIEW ARTICLE

Demographic, clinical, laboratorial, and radiological characteristics of rheumatic fever in Brazil: systematic review

Luciana Parente Costa^I; Diogo Souza Domiciano^I; Rosa Maria Rodrigues Pereira^II

^IRheumatology Department of USP

^IIProfessor of the Medical School of USP- Rheumatology

Correspondence to

ABSTRACT

Rheumatic fever (RF) is characterized by a non-suppurative inflammatory process that begins after a group A beta

hemolytic streptococci infection. Its prevalence is higher in developing countries, such as Brazil. However, in our country, systematic epidemiologic data on the disease are scarce and incomplete. Rheumatic fever has an estimated incidence of 3% among Brazilian children and adolescents. We undertook a systematic review of the main Brazilian studies using the LILACS, Scielo, and Medline databases searching for expressions like Febre Reumática and Rheumatic Fever. Ten epidemiological studies were selected and comparative analysis did not show a predominance of gender, clinical presentation, and laboratorial and radiological parameters in the different regions of the country.

Keywords: rheumatic fever, Brazil, epidemiology.

RHEUMATIC FEVER

Rheumatic fever (RF) is a late, non-suppurative complication of and oropharyngeal infection with group A beta-hemolytic streptococci. It is estimated that approximately 0.3 to 3.0% of individuals infected with known rheumatogenic strains of Streptococci will develop RF,^1,2 and approximately one to two thirds of them will develop rheumatic heart disease.

The disease is rare in many developed countries, but in developing countries RF continues to be a huge economical and social burden. In Brazil, chronic rheumatic cardiopathy still is the major cause of cardiac disease among children and young adults.

It is estimated a yearly incidence of 500,000 new cases of RF worldwide, leading to a prevalence of more than 15 million cases of rheumatic carditis. Approximately 233,000 people die every year as a consequence of this disorder.³ Latin America has an incidence of 21,000 cases of acute RF every year. Data in Brazil are scarce. In 2002, 5,000 new cases were reported (Brazilian Geographical and Statistical Institute - IBGE, from the Portuguese). Data from the Federal Health Department estimate an incidence of RF of approximately 3% among children and adolescents, being responsible for 40% of the cardiac surgeries in the country.⁴

The objective of the present study was to analyze the demographic, clinical, laboratorial, and radiological characteristics of RF in Brazil by reviewing studies in the LILACS, Scielo, and Medline databases.

METHODOLOGY

The LILACS, Scielo, and Medline databases were searched using the following sites: www.bireme.br, www.scielo.org, and www.ncbi.nlm.nih.gov/pubmed, and the key words: Febre Reumática and Rheumatic Fever. The search yielded 5,508 studies, and all studies in the Scielo database were also in the LILACS database. Studies in Portuguese were than selected, resulting in 334 studies. Of those, only epidemiologic studies that analyzed the demographic, clinical, laboratorial, and radiological characteristics of Brazilian populations with RF, according to Jones' criteria, were considered eligible. Only 11 studies fulfilled all requirements.^5-15 One of those studies⁵ was excluded because the population was described in more details on a later article,⁶ in 1993, resulting in a total of 10 studies.

RESULTS

Of the ten studies analyzed, four studied populations in the state of São Paulo (Ribeirão Preto, São Paulo, and Botucatu),^7-10 one in Santa Catarina (Florianópolis),⁶ one in Goiás (Goiânia),¹¹ one in Rio Grande do Sul (Porto Alegre),¹² two in Paraná (Curitiba),^13,14 and one in Acre (Rio Branco)¹⁵ (Table 1).

The study period ranged from 1972 to 2005, with a predominance of the decades of 1980 and 1990. All studies were retrospective, five of them included only one center,^{9,10,11,13,15} four were multicenter,^6,7,8,12 one focused on data from the County Health Department,¹⁴ and three studies focused on hospitalized patients.^6,7,12 A total of 2,355 patients were analyzed in the ten studies selected, with ages ranging from 3 to 38 years, but only one study evaluated patients older than 17 years¹¹ (Table 1).

Table 2 shows the clinical characteristics based on Jones' major criteria. 'Arthritis' predominated in most populations described, ranging from 21.4 to 84.3% (mean 63.3%). Carditis had a prevalence between 43.4 and 94.3% (mean 52.0%). The two studies in which the frequency of carditis was higher than that of arthritis included one from a Cardiology department (69.7%)¹⁵ and one that included hospitalized patients (94.3%).⁶ The incidence of chorea ranged from 49.4%, in a general pediatrics rheumatology hospital, to 6.1% (mean 27.7%).^13,15

A higher frequency of mitral valve involvement, ranging from 42 to 96.9% (mean 79.5%) was observed in all populations studied; the aortic valve was the second most affected (3.1 to 26.2%, mean 21.1%).^6,8-15 The concomitant involvement of the mitral and aortic valves ranged from 3.1 to 26.2% (mean 20.7%). Only one study reported the involvement of the tricuspid and pulmonary valves with an incidence of 9.2% and 0.55%, respectively.⁸

Minor criteria (Table 3) were observed in 60.2% of the patients of three studies.^6,8,12 Arthralgia had a mean incidence of 42.2%, being described in two studies.^6,8 An increase in the PR interval was reported in two studies, with a mean incidence of 10.4%.^6,15 All studies focused on clinical and laboratorial data^6-9,11-15 and determined the levels of anti-streptolysin O (ASLO) for evidence of a streptococcal infection, with an incidence ranging from 48.7 to 68.1% (mean 68%). Only Gus et al. described oropharyngeal cultures in seven of their patients, which was positive in only one of them.¹² A mean of 65.9% of the patients had elevated inflammatory activity assays. The erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), mucoproteins, α2-globulins, α1-acid glycoprotein, and gamma globulin levels were evaluated (Table 4).

The incidence of echocardiographic changes without clinical carditis in three populations analyzed ranged from 6.7 to 18.3%.^8,9,10 The chest X-ray showed an increase in the cardiothoracic index in 20.2% of the patients described by Borges et al. in 2005¹⁵. Electrocardiographic changes were reported in 55.1% of the patients and the included, besides the increase in the PR interval, sinus tachycardia, sinus bradycardia, sinus arrhythmia, premature ventricular contraction (PVC), overload, atrioventricular blocks, and changes in ventricular repolarization (Table 5).

Studying the incidence of breakouts of RF according to the seasons, three studies demonstrated a predominance during the winter months^11,12,13 and two others during the summer months.^6,9 Comparative analysis of those studies showed that 22.3 to 52.9% of the cases were seen during the winter months, with a mean of 33.0%, and 7.8 to 30.9% of the cases during the summer months, with a mean of 26.5%. Five studies evaluated the incidence of relapses, which ranged from 15 to 34% with a mean of 21.5%.^8,9,11,14,15

DISCUSSION

Analyzing the worldwide epidemiology of RF, a wide reduction in the incidence of acute RF after II World War, explained by greater access to antibiotics, especially in Europe, United States, and Japan, can be observed. This is comparable to what was seen in developing countries like Brazil, but in a smaller scale than that observed in developed nations.¹⁶

This systematic review demonstrated the presence of Brazilian literature on the demographic, clinical, laboratorial, and radiologic aspects of RF in different regions of the country: North, Midwest, South, and Southeast. We found studies published from the decade of 1980 to 2009.

Demographic, clinical, laboratorial, and radiological characteristics were comparable among the different Brazilian studies.

A significant predominance of carditis over arthritis was observed in two studies, possibly due to the characteristics

Demographical, clinical, laboratorial, and radiological characteristics of rheumatic fever in Brazil: systematic review

of the Service (cardiology and hospitalized patients) and not of the region or the decade in which they were undertaken. A predominance of mitral valve involvement followed by the aortic valve, isolated or associated with mitral valve involvement, was demonstrated in all studies. The presence of echocardiographic changes suggestive of rheumatic carditis in patients without cardiac symptoms, with an incidence of 20%, was described in two studies.

The studies do not agree on the predominance of RF according to the season, winter and summer, but it was slightly higher in the winter months when all studies are analyzed. A 20% relapse of RF was observed.

Comparative analysis of the region of the country and the time of the study did not show a predominance of gender, clinical presentation, and laboratorial and radiological characteristics in the study patients.

REFERENCES

• ¹
  Ayoub EM: Acute rheumatic fever and poststreptococcal reactive arthritis. In: Cassidy JT, Petty RE, editors. Textbook of pediatric rheumatology, 4 ed, Philadelphia: Saunders, 2001.
• ²
  Zabriskie JB. Rheumatic fever: the interplay between host, genetics, and microbe. Circulation 1985; 71:1077-86.
• ³
  Carapetis JR, Steer AC, Mulholland EK, Weber M. The global burden of group A streptococcal diseases. Lancet Infect Dis 2005; 5:685-94.
• ⁴
  Ministério da Saúde. Coordenação de doenças crônico degenerativas. Incidência da Febre Reumática no Brasil. Ministério da Saúde: Brasília, 2003.
• ⁵
  Silva ML, Baião TL, Fernandes VR, Teodósio SM, Maciel VL. Febre reumática: manifestações clínicas. Diagnósticos e tratamento em 40 casos. Arquivos Catarinenses de Medicina 1992; 21:209-16.
• ⁶
  Baião TL, Silva ML, Fernandes VR, Back IC, Lins S, Amaral AJ. Febre Reumática. Arquivos Catarinenses de Medicina 1993; 22:119-26.
• ⁷
  Laus E. Contribuição ao estudo da febre reumática na região de Ribeirão Preto. Medicina 1980; 12:7-16.
• ⁸
  Silva CHM. Rheumatic Fever: a multicenter study in the state os São Paulo. Rev Hosp Clin Fac Med S Paulo 1999; 54:85-90.
• ⁹
  Terreri MTRA, Caldas AM, Len CA, Ultchak F, Hilário MOE. Características clínicas e demográficas de 193 pacientes com febre reumática. Rev Bras Reumatol 2006; 46:385-90.
• ¹⁰
  Paulo LT, Terreri MT, Barbosa CM, Len CA, Hilário MO. Is rheumatic fever a more severe disease in pre-school children? Acta Reumatol Port 2009; 34:66-70.
• ¹¹
  Carmo HF, Vilela RG, Alvarenga SL et al Ainda a febre reumática. Rev Bras Reumatol 1994; 34:61-4.
• ¹²
  Gus I, Zaslavsky C, Seger JMP, Machado RS. Epidemiologia da febre reumática . Estudo local. Arq Bras Cardiol 1995; 65:321-5.
• ¹³
  Spelling PF, Nikosky JG, Santos DD. Incidência de moléstia reumática em um serviço de Curitiba - relato de 81 casos. Rev Med Paraná 2000; 58:5-16.
• ¹⁴
  Torres RPA, Cunha CLP, Miyague NI. Estudo de 500 casos de febre reumática na cidade de Curitiba. Divulgação em Saúde para Debate 2000; 19:73-5.
• ¹⁵
  Borges F, Barbosa ML, Borges RB et al Características clínicas demográficas em 99 episódios de febre reumática no Acre, Amazônia brasileira. Arq Bras Cardiol 2005; 84:111-4.
• ¹⁶
  Prokopowitsch AS, Lotufo PA. Epidemiologia da febre reumática no século XXI. Rev Soc Cardiol Estado de São Paulo 2005; 15:1-6.

Endereço para correspondência:

Rosa Maria Rodrigues Pereira

Faculdade de Medicina da Universidade de São Paulo

Av. Dr. Arnaldo, 455, 3º andar, Reumatologia, sala 3.105

São Paulo, SP, CEP: 01246-900 - Brasil

Publication Dates

• Publication in this collection
  17 Nov 2009
• Date of issue
  Oct 2009

History

• Accepted
  07 Aug 2009
• Received
  15 July 2009