Manifestation Of Acute Rheumatic Fever Research Articles

Index of Suspicion

Index of Suspicion

Sydenham’s chorea

Sydenham's chorea (SC) is a manifestation of acute rheumatic fever (ARF). Although the incidence of SC has declined significantly, particularly in developed areas, it remains the most common cause of acute chorea in children worldwide. Recent data show that SC accounts for almost all cases of chorea in children in the United States. As there is no specific laboratory marker of this condition, the diagnosis relies on a careful clinical history and laboratory assessment to rule out alternative causes. Morbidity is primarily related to cardiac lesions in ARF. It is recommended that all patients with suspected SC submit to a cardiologic evaluation. Neurologic features encompass motor signs, among which chorea is the most prominent, and nonmotor symptoms such as obsessive-compulsive behavior and attention-deficit/hyperactivity disorder. The first-line treatment of SC is valproic acid. Patients who do not respond to this drug or who present with severe chorea (particularly chorea paralytica, in which the muscle tone is so decreased that patients are bedridden) should be treated with risperidone. Other dopamine receptor-blocking drugs, such as haloperidol, may also be useful. There is growing evidence that immunosuppressive treatment, such as intravenous methylprednisolone followed by a tapering course of oral prednisone, is effective. This option has been used in patients who failed to respond to (or did not tolerate) the previously mentioned therapies. Plasmapheresis and intravenous immunoglobulin are regarded as experimental treatments. Obsessive-compulsive behavior associated with SC is not usually as severe as in other conditions such as Tourette's syndrome. Finally, because at least 20% of patients with SC experience recurrent attacks of ARF, they carry a high risk of developing severe cardiac lesions. These patients require prophylaxis against streptococcus infection, using penicillin or sulfa drugs.

Jones criteria and underdiagnosis of rheumatic fever

The authors attempt to determine whether typical clinical and laboratory manifestations of acute rheumatic fever (ARF) are in accordance to what has been traditionally described and how useful the Jones criteria are for diagnosis. Data from 81 cases of ARF were retrospectively collected. 5 to 15 years of age and diagnosis of ARF confirmed by 2 or more rheumatologists, sustained for at least 6 months and two or more visits. Girls had more chorea (23/50.0% vs. 5/14.3%)(p< 0.0001). Cardiovascular (65/80.2%) and joint involvements (63 / 77.8%) were the most frequent manifestations. Fever was noted in roughly half of the patients. Arthritis was more frequent than arthralgia (47/58.0% vs. 16/19.8%, respectively) (p< 0.0001); however, no specific pattern of joint involvement was found to be more prevalent. Mitral insufficiency was the most frequently detected echocardiographic sign (53 / 93.0%) and its association with aortic insufficiency was noted in 27 / 47.4% patients. Only 24 / 29.6% patients fulfilled Jones criteria for ARF requiring an evidence of previous group-A streptococcal infection (GASI). When compulsory GASI was disregarded, this number rose to 71/87.7% patients (p< 0.0001). Girls were more affected by chorea; heart valves and joints were equally affected and represented the major clinical features; no specific pattern of joint involvement (eg.: migratory arthritis) could be labeled as typical; and strict adherence to Jones criteria, with compulsory documentation of a previous GASI, may lead to underdiagnosis of ARF.

Continued high caseload of rheumatic fever in western Pennsylvania: Possible rheumatogenic emm types of streptococcus pyogenes

To evaluate the occurrence of cases of acute rheumatic fever (ARF) in western Pennsylvania although there has been a marked reduction of cases of ARF in the United States overall. From 1994 to 2003, the cases of ARF evaluated at Children's Hospital of Pittsburgh were reviewed. In addition, throat cultures were performed on a subset of these children and their family members beginning in 1995. Molecular typing was performed on isolates of the group A streptococcus (GAS) recovered, using field inversion gel electrophoresis (FIGE) and emm typing. There were 121 new cases of ARF from 1994 to 2003. Of these, 57% were male. The median age was 10 years. The majority of children (57%) had carditis with or without another manifestation of ARF. The results of throat cultures were available for 231 persons; 36% (30/84) of the children with ARF and 14% (20/147) of family members were positive for GAS. Eight emm types were observed (emm 1, 2, 6, 12, 18, 28, 75, and 89). Data suggest that emm type 12 may be a rheumatogenic strain. ARF remains a problem in western Pennsylvania. Identification of emm types associated with cases should enlighten vaccine development.

Did Gustav Mahler have Sydenham's chorea?

Sydenham's chorea (SC), a major manifestation of acute rheumatic fever (RF), is characterized by chorea and other motor and non-motor features. Among the latter are behavioral symptoms, including obsessive-compulsive disorder. Although SC is typically a self-limited condition, up to 50% of patients may evolve with persistent chorea. There is evidence that Gustav Mahler had a movement disorder, but its nature remains undetermined. There are witnesses describing him as having facial dyskinesia and a gait disorder consistent with chorea. His conducting performance was notorious for obsessive attention to details of the staging and musical production. Mahler was diagnosed with a valvulopathy in 1907 and died of subacute bacterial endocarditis in 1911. It is possible that the composer suffered from RF in childhood with carditis and SC, which may left him with valvulopathy, obsessive-compulsive disorder, and persistent chorea.

Evaluation of subclinical valvar disease in patients with rheumatic fever

Carditis is the only manifestation of acute rheumatic fever that leads to permanent disability. Hence, its diagnosis is of paramount importance. Recently, it has been reported that Doppler echocardiography has disclosed subclinical valvar regurgitation in some patients with acute rheumatic fever manifested as isolated arthritis or pure chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is not clear. We aimed, therefore, prospectively to investigate the potential to diagnose patients with subclinical carditis. We examined 40 patients, aged from 7 to 16 years, with Doppler evidence of mitral and aortic regurgitation, but in the absence of any pathologic murmur. The major findings satisfying the Jones criterions were arthritis in 29 patients, chorea in 10 patients, and arthritis and erythema marginatum in one patient. Of the patients, 33 had mitral regurgitation, 6 patients had combined mitral and aortic regurgitation, and one patient had aortic regurgitation. The patients were followed over a mean period of 18.1 +/- 13.9 months, the valvar regurgitation disappearing in 23 (57.5%). No significant differences were observed in the resolution of the valvitis between those treated with acetylsalicylic acid, steroids, or those receiving no treatment. It is noteworthy, nonetheless, that patients treated with steroids were the fastest to recover from valvitis (p < 0.05). Based on our study, we suggest that subclinical valvitis demonstrated by echocardiography should now be accepted as adequate evidence for the diagnosis of carditis, and become a major diagnostic criterion for acute rheumatic fever. When managing this group of patients with subclinical disease, treatment with steroids seems to have a role in promoting early resolution of the valvitis.

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.

Epitopes of streptococcal M proteins that evoke antibodies that cross-react with human brain.

There is evidence suggesting that Sydenham's chorea, which is a major manifestation of acute rheumatic fever, may be mediated by streptococcal antibodies that cross-react with the brain. Our studies were undertaken to determine whether streptococcal M protein, the major virulence factor of group A streptococci, evoked antibodies that cross-react with human brain. Rabbits were immunized with pepsin-extracted M protein from rheumatogenic type 6 streptococci. Immune sera were screened for the presence of antibodies that cross-reacted with human brain by indirect immunofluorescence tests and immunoblot analyses. Type 6 M protein evoked antibodies that cross-reacted with several brain proteins and antibody binding to these proteins was completely inhibited by type 6 M protein and partially inhibited by types 5 and 19 M proteins, suggesting that these heterologous M proteins contain conserved brain-cross-reactive epitopes. Using synthetic peptides from several serotypes of M proteins, the conserved brain-cross-reactive epitopes were localized to a decapeptide contained within the covalent structure of the B repeat region of type 6 M protein. These peptides also inhibited brain-cross-reactive antibodies in the serum of a patient with active Sydenham's chorea. Our data indicate that streptococcal M proteins contain brain-cross-reactive epitopes that could potentially be involved in the pathogenesis of Sydenham's chorea.

Chorea as a manifestation of rheumatic fever--a 30-year survey (1960-1990).

Sydenham chorea, a major manifestation of acute rheumatic fever, has been the most common form of acquired chorea during childhood. Despite the recent dramatic decline in both incidence and severity of rheumatic fever in our area, the frequency of carditis was unchanged. This study investigated retrospectively the incidence of chorea in the last three decades (1960-1990) in our area. During the 30 years of the survey, 28 patients with Sydenham chorea were treated in our centre of whom 10 were seen between 1960-1970, 17 between 1970-1980, and only one patient between 1980-1990.

Immunopathology of rheumatic pericarditis

Immunologic mechanisms have been implicated in the endocarditis, myocarditis, and arthritis of acute rheumatic fever. However, pericarditis, a major manifestation of acute rheumatic fever with a clinical incidence of 5-10%, has not been studied. We have evaluated a patient who had this febrile disease with arthritis and pericarditis. Pericardial tissue showed extensive deposition of IgG, IgM, and C3 both on the pericardial surface and within the walls of pericardial blood vessels. Our findings of immunoglobulins and complement within the inflamed pericardium suggest an immune-mediated mechanism for the pericarditis of acute rheumatic fever.

Rheumatic Fever in the Adult

The clinical manifestations of acute rheumatic fever were evaluated in a study of 35 adult patients. Migratory polyarthritis occurred in all but one. None had chorea, erythema marginatum, or subcutaneous nodules. Carditis occurred in eight, manifested by pericarditis in four and by development of new significant murmurs in four. Two patients died during their acute attacks, and nine exhibited new valve damage on follow-up. The results of the study indicate that rheumatic fever represents a continuous spectrum from childhood to adulthood, in which the incidence of carditis, though diminishing, remains significant. In addition, cardiac involvement may occur de novo in the adult, resulting in permanent damage or even death. This would have important implications regarding penicillin prophylaxis.

Abdominal pain in acute myocarditis and pericarditis.

Severe abdominal pain, often accompanied by gastrointestinal symptoms and fever, may be the first and most prominent symptom of myocardial or pericardial disease. This has been well recognized in adults, and examples of myocardial infarction masking as acute cholecystitis or perforated peptic ulcer have been frequently recorded. Acute pericarditis has similarly been confused with a number of acute surgical diseases. With the exception of abdominal pain as a frequent manifestation of acute rheumatic fever, the counterpart of these experiences in childhood has not been emphasized. The cases selected for this study fit largely into the categories of acute interstitial myocarditis or acute idiopathic pericarditis. In addition 2 cases of acute bacterial pericarditis were included. Cases with involvement of the myocardium or pericardium as a part of a widespread septic disease were omitted. Cases of rheumatic heart disease, diphtheritic myocarditis, endocardial fibroelastosis, congenital heart disease, and idiopathic myocardial hypertrophy were excluded.

EVALUATION OF THE STREPTOCOCCAL DESOXYRIBO-NUCLEASE B AND DIPHOSPHOPYRIDINE NUCLEOTIDASE ANTIBODY TESTS IN ACUTE RHEUMATIC FEVER AND ACUTE GLOMERULONEPHRITIS

Antibody titers for two recently described streptococcal antigens, desoxyribonuclease B (DNase B) and diphosphopyridine nucleotidase (DPNase) have been compared with antistreptolysin O (ASO) titers in patients with acute rheumatic fever, in patients with acute nephritis, and in normal controls. Like the ASO, elevated titers for the two new antibodies are commonly found in patients with complications of streptococcal infections. The titers for anti-DPNase tend to be higher in acute nephritis than in acute rheumatic fever. These two new anti-body tests are particularly useful in providing evidence of a preceding streptococcal infection in those patients with manifestations of acute rheumatic fever or acute nephritis who fail to show an elevated ASO titer.

Studies on acute rheumatic fever in the adult. II. The rebound phenomenon.

Excerpt INTRODUCTION In a previous publication1the clinical and laboratory manifestations of acute rheumatic fever in 30 adult patients were studied. Treatment of these patients consisted of adreno...

Purpuric manifestations of rheumatic fever and acute glomerulonephritis

1. 1. Three cases of symptomatic purpura have been presented. In two of these the underlying factor was considered to be acute rheumatic fever, in one of which there was evidence of simultaneous nephritis. The primary cause in the third case was acute glomerulonephritis. 2. 2. The various cutaneous manifestations of acute rheumatic fever and acute glomerulonephritis have been outlined. 3. 3. The nephritic manifestations of rheumatic fever have been discussed together with the relationship between these two conditions.

Production of Hypoprothrombinemia and Hypoeoagulability of the Blood with Salicylates.

Conclusions1. The oral administration of acetylsalicylic acid and sodium salicylate to human beings in daily doses of 20 to 80 grains (1.3 to 5.3 g) consistently produced hypo-prothrombinemia and hypocoagulability of the blood. 2. The administration of vitamin K with the salicylate prevented the development of hypoprothrombinemia and prolongation of the coagulation time. 3. It is theoretically possible that the not unusual hemorrhagic manifestations of acute rheumatic fever may be due in some cases, at least in part, to the large doses of salicylates so commonly administered.