Rheumatic fever: the relationship between clinical manifestations and laboratory tests

OBJECTIVES: To determine the relationship between the severity of clinical features of rheumatic fever (RF), and antistreptolysin O titre (ASOT) and/or erythrocyte sedimentation rate (ESR). METHODS: Clinical and laboratory data from 102 children with RF who had been admitted at a university hospital in Tehran between 1992 and 2002 were reviewed retrospectively. In order to categorize the severity of clinical manifestations of disease, patients were divided into three groups. Those with arthritis alone were defined as group A, carditis with or without arthritis as group B and carditis (with or without arthritis) with congestive heart failure as group C. RESULTS: Thirty-one cases were enrolled in group A, 39 in group B, and 32 in group C. We didn't find a significant relationship between the severity of clinical presentation of disease and ASOT (P = 0.89) and ESR (P = 0.24). Seventy-two patients presented with first attack and 30 had recurrences. The frequency of congestive heart failure (CHF) in first attacks was 31.2%vs 68.8% in recurrences (P < 0.0001). No Significant relationship was found between the number of involved valves and ASOT (P = 0.4) or ESR (P = 0.8). CONCLUSION: Variable clinical presentation of disease and increasing intensity of cardiac involvement is not related to the ASOT or ESR levels.     

Clinical, laboratory and echocardiographic profile of acute rheumatic fever in Nepali children

BACKGROUND: Acute rheumatic fever (RF) is a common, preventable health problem in developing countries. Sporadic outbreaks and the prevalence in some indigenous populations have renewed interest in RF in developed countries also. AIMS: To describe the clinical, laboratory and echocardiographic features, outcome and value of echocardiography in detecting valvular disease in RF. METHODS: A prospective, cross-sectional study was conducted over 2 years. Patients under 14 years admitted to the cardiology unit of Kanti Children's Hospital, Kathmandu with RF using the Jones criteria were recruited consecutively. RESULTS: The median age (range) of the 51 patients was 11 (5-14) years, the male:female ratio was 1.6:1 and 39% had a history of a sore throat. Clinical and laboratory features detected were as follows: carditis 92%, arthritis 33%, chorea 8%, subcutaneous nodules 4%, fever 51%, arthralgia 37%, elevated antistreptolysin O titre 94%, elevated CRP 78%, prolonged PR interval 45%, pericardial effusion 22% and cardiac failure 28%. In total, 36 patients (71%) complained of joint pains. A murmur on auscultation was significantly associated with underlying diseased valves confirmed by echocardiography (p=0.001). A murmur was audible in 78.4% and diseased valves were confirmed by echocardiography in 88.2%. The mitral valve was the most commonly involved valve (82%) and mitral regurgitation the commonest lesion (24%). A thickened mitral valve predicted carditis (p=0.007). Five (10%) patients died. CONCLUSION: Inclusion of echocardiographic evidence of carditis and possibly arthralgia as major criteria would improve case detection.     

What is the True Frequency of Carditis in Acute Rheumatic Fever? A Prospective Clinical and Doppler Blind Study of 56 Children with up to 60 Months of Follow-Up Evaluation

BACKGROUND: This study aimed to evaluate prospectively clinical and echocardiographic findings of patients who had rheumatic fever with and without clinical features of cardiac involvement. METHODS: For this study, 56 consecutive patients (mean age, 11.4 years) with acute rheumatic fever diagnosed according to the 1992 modified Jones criteria were evaluated at diagnosis, after 3 and 6 months, then at 2 and 5 years. All assessments were performed blindly and included physical and cardiac examination, electrocardiogram, chest X-ray, and two-dimensional color-flow Doppler echocardiography. RESULTS: Initial clinical carditis was observed for 27 patients (48.2%), all of whom had positive echocardiographic abnormalities. Echocardiographic abnormalities were observed in 11 patients who had arthritis or chorea presentation without initial clinical carditis. Persistence of the abnormalities was observed at a late follow-up evaluation in 72.7% of the cases. Sydenham's chorea was present in 8 patients with clinical carditis and in 10 without this disorder, 8 of whom had echocardiographic abnormalities. CONCLUSION: Patients who had acute rheumatic fever without clinical signs of carditis showed acute and late follow-up echocardiographic abnormalities suggestive of cardiac involvement. Clinicians should be attentive for the presence of cardiac involvement among patients with chorea.     

Primary meningococcal arthritis in pediatrics. Report of nine cases

Primary meningococcal arthritis is an extremely uncommon type of invasive meningococcal disease, with an incidence of 1.5- 1.8% of all paediatric cases of pyogenic arthritis. It is defined as the presence of acute septic arthritis without association with meningitis or the classic meningococcaemia, and isolation of Neisseria meningitidis in synovial fluid and/or blood culture. Typically monoarticular, mostly affects large joints. Prognosis is excellent with appropriated treatment. The aim of this study is to report 9 cases of primary meningococcal arthritis, evaluated at Hospital de Ni?os "Dr. R. Gutiérrez" in a period of 3 years, and to discuss clinical and epidemiologic issues.     

Poststreptococcal reactive arthritis: clinical course and outcome in 15 patients

Patients with Group A beta-hemolytic streptococcal infection and articular disease, who do not fulfill the modified Jones criteria for diagnosis of acute rheumatic fever (ARF), have been classified as having poststreptococcal reactive arthritis (PSRA). We reviewed the clinical characteristics, laboratory findings and outcome of 15 patients with PSRA. None of these patients had clinical evidence of carditis. The pattern of joint involvement was variable and included arthritis in five patients and arthralgia in the remaining ten patients. Nine patients were treated with salicylates for one to 16 weeks; the others recovered spontaneously. Usually, the patients with arthralgia responded promptly to salicylates, while the response was poor in patients with arthritis. One patient with monoarthritis developed carditis nine months after his first arthritis attack. Another patient presenting with monoarthritis later had two additional episodes of poststreptococcal reactive arthralgia. It seems there is a wide spectrum of poststreptococcal rheumatic diseases, and patients with PSRA are also at risk for cardiac disease; therefore, prophylactic antibiotic therapy should be considered in these patients.     

Septic arthritis in childhood

BACKGROUND: The purpose of the present study was to determine whether there was a difference between septic arthritis (SA) combined with osteomyelitis and SA alone with regard to clinical and laboratory findings, such as symptoms on admission, age, sex, joint involvement and isolated micro-organisms, and a relationship between age and joint involvement in SA. In addition, we also aimed to determine the prognostic factors in SA. METHODS: The clinical and laboratory findings of 40 patients who were diagnosed with SA in our hospital were reviewed retrospectively. The diagnosis of SA was made according to the following criteria: immediate joint fluid aspiration (culture and Gram's stain positive, leukocyte count markedly elevated and glucose level low), blood culture positive and positive cultures from other possible sites of infection. RESULTS: Of the 40 patients, 22 were boys, 18 were girls and the male to female ratio was 1.2/1. Patient ages ranged from 6 months to 14 years (mean (+/- SD) 8.44 +/- 4.18 years). The most observed symptoms were fever (52.5%), arthralgia (50%) and joint swelling (45%). Thirty-four (85%) patients had only one joint and six patients (15%) had more than one joint involved. In total, arthritis was diagnosed in 49 joints. The joints diagnosed as having arthritis were the following: knee (n = 18), hip (n = 12), ankle (n = 12), elbow (n = 3), shoulder (n = 2), wrist (n = 1) and interphalangeal joint (n = 1). Of the 40 patients, 21 (52.5%) had SA alone and 19 (47.5%) had arthritis together with osteomyelitis. While arthritis was diagnosed in 27 joints in the group of patients with SA, it was diagnosed in 22 joints in the group of patients with SA combined with osteomyelitis; in the latter, an increase was not observed in the number of joints involved. Joint fluid culture was positive in 22 (55%) patients; the growth of Staphylococcus aureus was observed in 20 cases and Pseudomonas aeruginosa and Staphylococcus epidermidis were isolated in each patient. In contrast, in one patient, arthritis occured during meningococcal meningitis (in this patient, Gram-negative diplococci was isolated from a cerebrospinal fluid culture). Patients with SA combined with osteomyelitis and those with SA alone were compared for symptoms on admission, the history of trauma and antibiotic use, sex, age, fever, joint involvement, anemia, leukocytosis and micro-organisms isolated from joint fluid and blood; there were no significant differences for these parameters between the two groups (P > 0.05). In addition, we found that there was no relationship between age and joint involvement in SA and there was no effect of micro-organisms on mortality. Three of 40 patients died; the mortality rate was 7.3%. Of the three patients who died, two had SA alone and one had SA combined with osteomyelitis. The primary disease was sepsis in these three patients; S. aureus was cultured from blood in two patients and Gram-positive cocci was observed following examination of the joint fluid in the other patient. CONCLUSIONS: We would like to emphasize that SA is mono-articular, frequently localized in the knee, hip and ankle in 85% of patients, joint fluid culture was positive in 55% of patients, bacteria was isolated from one or more cultures of blood, joint fluid, pus or bone in 70% of patients and the most common isolated micro-organism was S. aureus. In addition, it must be pointed out that children younger than 2 years of age with fever, a positive trauma history and/or abnormal joint findings should be carefully examined for SA because the rate of SA was lower (7.5%) than expected in this age group. We also found that the mortality of SA was not influenced by age, joint involvement and bacterial agents, and there was no significant difference in symptoms on admission, the history of trauma and antibiotic use, sex, age, fever, joint involvement,anemia, leukocytosis and micro-organisms isolated from joint fluid and blood between patients with SA     

Acute rheumatic fever in the young: changing prevalence and pattern

Acute rheumatic fever (ARF) is rare in young people. A group of 28 patients is described who were hospitalized during the years 1960–1984. Patients were divided into two groups: children who were admitted between 1960 and 1966 and those admitted between 1966 and 1984. The annual incidence of ARF declined from 1.17 to 0.44 cases per 10,000 population at risk. The incidence of arthritis declined in parallel to the decrease in number of children presenting with ARF, whereas the incidence of carditis remained the same.Abbreviations ARF acute rheumatic fever - RF rheumatic fever     

Clinical and morphologic parallels in children with rheumatoid mono- and oligoarthritis

Altogether 88 children with rheumatoid arthritis were examined. Of these, 36 patients were with an injury to one articulation and 52 with an injury to one to three articulations. The clinical features of the articulation syndrome, the data obtained as a result of studying synovial fluid and of the morphological picture of synovial biopsy specimens made it possible to denote rheumatoid mono- and oligoarthritis in children as benign varieties of the disease with primarily local (in articulations) inflammatory process. The difference found was only qualitative as regards the intensity of the clinico-laboratory signs (more pronounced in patients suffering from oligoarthritis). This fact provides basis for making the diagnosis of monoarticular or oligoarticular rheumatoid arthritis.     

What is the outcome of rheumatic carditis in children with Sydenham's chorea?

We evaluated the echocardiographic features of 69 children diagnosed with Sydenham's chorea at the first attack of acute rheumatic fever. By echocardiography, carditis was detected in 71% of cases and silent carditis was shown in 28.9% of cases at initial presentation. Most patients had mild or moderate valvular regurgitation. Sixty-three cases were followed from 1-10 years. The improvement rate in valvulitis in cases with silent carditis (29.4%) was not different than in cases with clinical carditis (18.5%) (p > 0.05). Persistence of valvular pathologies occurred in 72.2% of cases with carditis in the long-term follow-up (> 2 years). Most patients (88.8%) complied with secondary prophylaxis, so relapse of carditis was exclusively prevented in our patients. Recurrence of chorea was identified in 20.6% of cases and was not associated with clinical or laboratory evidence for streptococcal reinfection. Patients with chorea usually had mild carditis, and carditis showed resolution. Relapse of carditis in our population was exclusively prevented with secondary prophylaxis. Recurrence of chorea was not rare, despite regular treatment with benzathine penicillin.     

The first attack of acute rheumatic fever in childhood--clinical and laboratory profile

One hundred consecutive cases of 'first attack' of acute rheumatic fever were studied. There were 52 males and 48 females, constituting 1.12% of total hospital admissions. Nearly 10% of children were below the age of 5 years, stressing the early onset of rheumatic fever in tropics. Only 47% gave a definite history of overcrowding at home. Sore throat was present in 67%, overt arthritis in 66%, carditis in 57%, arthralgia alone in 22% of which 45.45% had carditis. Small joint involvement was noticed in 23% of cases of which 73.91% had carditis. Only 33.33% had congestive cardiac failure. Ten per cent of children had chorea, while subcutaneous nodules were seen in 4% of cases, all of whom had associated carditis. Erythrocyte sedimentation rate (ESR) showed good correlation with clinical profile. Throat cultures were positive for beta hemolytic streptococci only in 12% of cases. Anti-streptolysin 'O' (ASO) titre showed significant titres on 68% of cases, anti-deoxyribonuclease "B" (ADN-B) in 69.32%, antibody to group A carbohydrate (ACHO) in 70.65%. ASO, ADN-B, and ACHO titres together gave 87.5% positivity while estimations in paired sera showed ASO 79.54%, ADN-B 82.27% and ASO, ADN-B together 99.92% significant titres. Study of blood groups showed A group children to be more vulnerable to rheumatic fever (37.5%) and rheumatic carditis (47.37%). Mortality in the present study was nil.     

Rheumatic chorea in northern Australia: a clinical and epidemiological study.

To describe the epidemiology and clinical features of Sydenham's chorea in the Aboriginal population of northern Australia a review was conducted of 158 episodes in 108 people: 106 were Aborigines, 79 were female, and the mean age was 10.9 years at first episode. Chorea occurred in 28% of cases of acute rheumatic fever, carditis occurred in 25% of episodes of chorea, and arthritis in 8%. Patients with carditis or arthritis tended to have raised acute phase reactants and streptococcal serology. Two episodes lasted at least 30 months. Mean time to first recurrence of chorea was 2.1 years compared with 1.2 years to second recurrence. Established rheumatic heart disease developed in 58% of cases and was more likely in those presenting with acute carditis, although most people who developed rheumatic heart disease did not have evidence of acute carditis with chorea. Differences in the patterns of chorea and other manifestations of acute rheumatic fever in different populations may hold clues to its pathogenesis. Long term adherence to secondary prophylaxis is crucial following all episodes of acute rheumatic fever, including chorea, to prevent recurrence.     

Rheumatic chorea in northern Australia: a clinical and epidemiological study

To describe the epidemiology and clinical features of Sydenham's chorea in the Aboriginal population of northern Australia a review was conducted of 158 episodes in 108 people: 106 were Aborigines, 79 were female, and the mean age was 10.9 years at first episode. Chorea occurred in 28% of cases of acute rheumatic fever, carditis occurred in 25% of episodes of chorea, and arthritis in 8%. Patients with carditis or arthritis tended to have raised acute phase reactants and streptococcal serology. Two episodes lasted at least 30 months. Mean time to first recurrence of chorea was 2.1 years compared with 1.2 years to second recurrence. Established rheumatic heart disease developed in 58% of cases and was more likely in those presenting with acute carditis, although most people who developed rheumatic heart disease did not have evidence of acute carditis with chorea. Differences in the patterns of chorea and other manifestations of acute rheumatic fever in different populations may hold clues to its pathogenesis. Long term adherence to secondary prophylaxis is crucial following all episodes of acute rheumatic fever, including chorea, to prevent recurrence.     

Juvenile rheumatoid arthritis of the knee: evaluation with contrast-enhanced color Doppler ultrasound

BACKGROUND: Contrast-enhanced color Doppler ultrasonography is a non-radiation-bearing tool that can be of value for assessment of inflammatory and vascular synovial changes in juvenile rheumatoid arthritis (JRA). OBJECTIVES: To evaluate the effect of contrast-enhanced color Doppler ultrasound (US) in the evaluation of synovial changes in the knees of children with JRA. MATERIALS AND METHODS: Sagittal color Doppler sonograms of 31 knees in 22 patients with JRA and of 10 knees in 5 control subjects were obtained before (at baseline) and after (at peak contrast phase) intravenous injection of SHU 508. Images were assessed for overall mean pixel intensity within the synovial tissue and for peak enhancement ratios [[(mean pixel intensity values at maximum contrast enhancement-unenhanced mean pixel intensity values)/unenhanced mean pixel intensity values] x 100]. The joints were classified into three groups by clinical/laboratory criteria: group A (active disease in the knee), n = 9; group B (quiescent disease with serum chemistry levels of active disease), n = 12 and group C (remission disease), n = 10. RESULTS: Mean color pixel intensity values were markedly increased by the use of US contrast agents in groups A (P = 0.004) and B (P = 0.0001), did not reach statistical significance in group C (P = 0.06) and remained essentially unchanged in the control group (P = 0.25). Enhancement ratios for the three groups of JRA patients were not different (P = 0.38) (mean +/- SD, 720% +/- 402 for group A, 731% +/- 703 for group B and 314% +/- 263 for group C). CONCLUSION: Contrast-enhanced color Doppler imaging holds promise for the detection of active synovial inflammatory disease in subclinical cases of JRA, thereby allowing earlier treatment and improved clinical outcome.     

Prognosis of children with poststreptococcal reactive arthritis

Patients with Group A beta-hemolytic streptococcal infection and articular disease who do not fulfill the modified Jones criteria for a diagnosis of acute rheumatic fever (ARF) have been classified as poststreptococcal reactive arthritis/arthralgia. We reviewed the initial clinical characteristics and outcome of 12 poststreptococcal reactive arthritis/arthralgia patients. During the initial episode all had arthritis or arthralgia and a documented streptococcal infection. None had carditis and none received prophylactic antibiotic therapy during an average follow-up of 17 months (range, 6 to 42 months). One patient developed classic ARF with valvulitis 18 months after the initial episode. Two children had later episodes of arthritis and two had at least one additional episode of arthralgia. Poststreptococcal reactive arthritis/arthralgias seems to be part of the disease spectrum of ARF and therefore the use of prophylactic antibiotic therapy to prevent subsequent development of ARF and carditis in these patients should, perhaps, be reconsidered.     

Serum mucoprotein tyrosine in rheumatism

Summary and Conclusions 75 cases of rheumatism in children with clinical manifestations such as arthritis, chorea, carditis and arthritis with carditis were studied for serum mucoprotein values. The values were raised consistently in all the cases. Even cases of rheumatic chorea showed a significant rise in mucoproteins while other tests for judging rheumatic activity may be non-contributory. From the Department of Pediatrics, M. G. Medical College, Indore.     

Acute osteomyelitis and septic arthritis in children

OBJECTIVE: To review the clinical presentation, clinical management and organisms responsible for acute haematogenous osteomyelitis (AHO) and septic arthritis (SA) in the post Haemophilus influenzae type B (Hib) vaccine era and to evaluate current Australian antibiotic guidelines for these conditions. METHODS: A retrospective chart review of children less than 16 years of age presenting to The Children's Hospital at Westmead in the period from January 1998 to July 2002 with an ICD discharge code consistent with AHO or SA. RESULTS: During the 4 1/2-year period 120,511 children were admitted to The Children's Hospital at Westmead. There were 102 cases of AHO and 47 cases of SA during this time. An organism was identified either by blood culture or tissue biopsy in 45% of children with AHO and 38% with SA. Staphylococcus aureus was the most common identifiable causative organism accounting for 76% of isolated organisms in AHO and 39% of isolated organisms in SA. Methicillin-resistant S. aureus (MRSA) was responsible for 9% of AHO and 6% of SA cases. There were no cases due to Haemophilus influenzae or Kingella kingae during the study period. The majority (66%) of children with AHO were managed non-operatively with intravenous and then oral antibiotics. Thirty-five (34%) children had operative treatment to drain pus. In contrast, 74% of the patients with SA had one or more surgical procedures performed to drain pus from involved joints. CONCLUSIONS: Staphylococcus aureus remains the most common organism causing AO and SA; however, community-acquired methicillin-resistant strains are now occurring. Haemophilus influenzae is no longer a common cause of SA. Our study supports the current Australian antibiotic guidelines that recommend flucloxacillin alone as the empiric treatment of choice of both AHO and SA in children fully immunised against Hib. However the possibility of community-acquired MRSA should be considered, particularly in high risk groups such as indigenous Australian children or children from regional areas with a high rate of community-acquired MRSA.     

Some uncommon manifestations of rheumatic fever

Summary 7 cases with unusual and uncommon manifestations of rheumatic fever have been reported and discussed. 3 patients had rheumatic pneumonia; one of them had associated rheumatic peritonitis, 1 had subcutaneous nodules without any cardiac involvement and 1 had chorea with arthritis. There were 2 patients with rheumatic carditis showing a change in the murmurs on follow-up. From the Departments of Paediatrics and Cardiology, Postgraduate Institute of Medical Education and Research, Chandigarh, 11.     

Childhood acute rheumatic fever in Ankara, Turkey

Patients with acute rheumatic fever (ARF) admitted to a tertiary hospital in Ankara between January 1999 and July 2002 were studied cross-sectionally to verify the clinical profile and were followed during the acute period. All patients were investigated for throat culture, streptococcal serologic study, C-reactive protein, and erythrocyte sedimentation rate, and telecardiograms, electrocardiograms and echocardiographic study were done. During the study period, 129 attacks of ARF were observed: 118 were initial attacks and 11 were recurrences. Age on admission was 11.2 +/- 2.73 years (mean +/- SD, range: 6-21 years). Polyarthritis alone was present in 42 cases (33%), carditis alone in 33 (25%), combined carditis and polyarthritis in 36 (28%), combined carditis and chorea in 15 (12%) and chorea alone in 3 (2%). Nineteen (14%) patients with isolated arthritis and pure chorea had silent mitral and/or aortic regurgitation. Carditis was a dominant presenting manifestation, but appeared to be mild or moderate. The present study indicates that ARF is still a significant problem in Turkey. The observation that 8.5% of the attacks were recurrent reaffirms the need for more effective secondary prevention programs.