Delayed diagnosis of acute rheumatic fever in adults: a forgotten cause of febrile polyarthritis

Acute rheumatic fever is a nonsuppurative sequela of upper respiratory tract infection with group A streptococci. We describe our recent experience with the diagnosis and management of 3 cases of acute rheumatic fever to highlight the delays that may arise in the diagnosis of this condition. In adults, febrile polyarthritis is the most common presentation of acute rheumatic fever. Increased awareness on the part of the physician is necessary to ensure both prompt and accurate diagnosis of this cause of febrile polyarthritis.     

Acute articular rheumatism in the child in 1997

Nine cases of rheumatic fever were seen from 1982 to 1996. The diagnosis was based on Jones criteria. Four of eight children had carditis characterized by mitral regurgitation with or without aortic regurgitation and/or atrioventricular conduction disturbances. The outcome was favorable in all the patients who had carditis initially; one of the patients without initial carditis developed permanent cardiac lesions during a recurrence with carditis. In industrialized countries, the incidence of rheumatic fever declined starting early in the XXth century, then dropped sharply after World War II, and is now extraordinarily low (mean annual incidence, 0.5/100,000 schoolage children). In developing countries, by contrast, rheumatic fever was recognized only after World War II and remains endemic (mean annual incidence, 100 to 200/100,000 schoolage children), contributing a substantial proportion of cases of cardiovascular disease. The diagnosis is difficult and rests on clinical grounds since there is no specific laboratory test. Diagnostic delays are potentially serious. Acute attacks should be managed as therapeutic emergencies. Prevention of recurrences rests on long-term antimicrobial therapy. Rheumatic fever is a disease process resulting from an inappropriate immune response to pharyngitis due to a beta-hemolytic group A streptotoccus (BHAS). A low standard of living may be a factor in developing countries but fails to explain the epidemic flares seen in these areas or the residual background incidence in industrialized countries. A role of host-related susceptibility to the disease has not been demonstrated. The type-specific surface M protein, the main factor associated with high virulence, carries a specific epitope on its distal portion. Rheumatogenic strains have been identified; most produce mucoid colonies. At a given point in time, within a given serotype, the virulence of a specific strain increases. Temporal and spatial variations of observed types contribute additional complexity. Adhesion of the organisms is followed by release of streptococcal degradation products that share antigenic determinants with human tissues including the heart, the synovium, and the neurons. The hyaluronate capsule and M protein of the organisms are capable of initiating immune responses; their presentation to CD4+ T-cells results in lymphokine production, an acute phase humoral response, and a cell-mediated response potentially responsible for permanent valvular damage. In France, the standard of care is to prescribe antimicrobial therapy to all patients with pharyngitis or tonsillitis without performing tests to identify the causative agent. The introduction of tests for the rapid recognition in routine clinical practice of BHAS, which account for only 20 to 30% of all cases of pharyngitis and tonsillitis, should allow a more rational approach to the treatment of these infections. Reserving antimicrobial therapy to those patients with BHAS should not result in an increase in the incidence or rheumatic fever.     

Strategies for correcting the delta F508 CFTR protein-folding defect

Several scores based on symptoms and signs have been developed to assess the presence of heart failure. The goal of this study was to compare six heart failure scores in non-hospitalised subjects and to determine their usefulness in population based research. The scores were applied to 54 participants of a population based study. All underwent a complete medical examination, including chest X-ray, electrocardiography and Doppler echocardiography. Using all information available, a cardiologist, unaware of the results of the scores, clinically classified participants as having no, possible or definite heart failure. Sensitivity, specificity, predictive values and receiver operating characteristics were calculated, using the cardiologist's assessment as a gold standard. The cardiologist judged definite or possible heart failure to be present in 17 persons. All scores had a high sensitivity for the detection of definite heart failure, whereas the study of men born in 1913 and Walma's score had the highest sensitivity for the combination of possible and definite heart failure. Gheorgiade's and the Boston score had the highest positive predictive values. In conclusion, five of the six scores we studied are broadly similar in the detection of heart failure. The men born in 1913 score relies heavily on the assessment of dyspnea, resulting in a relatively large number of false positives. Although the scores are useful in detecting manifest heart failure, objective measurements of cardiac function appear necessary to reduce the false positive rate and accurately detect early stages of heart failure.     

Low-grade fever of protracted course. Retrospective study of 30 cases and reflections for a low-cost management

The objects of this paper were the following: 1) to establish in a group of persistent (over four weeks) low grade fever (LGF) patients the percentage of cases in which a definite diagnosis could not be made; 2) where a definite diagnosis could be made, to describe the most commonly occurring diseases; 3) to follow up the uncertain diagnosis cases for at least two years. Thirty cases of persistent LGF were retrospectively studied. They did not include drug hyper-dysthermia and temporary and/or metabolic vasomotor reactions. The data suggest that: 1) two thirds of persistent LGF are likely not to be definitely diagnosed; 2) some certain diagnoses were: dental granulomas, mycobacteria infections, thyroiditis, factitious fever, rheumatic polymyalgia, Hodgkin's lymphoma and pulmonary thromboembolism; 3) in 14/19 undiagnosed cases the fever subsided permanently, without any treatment, within one year, whereas it persisted in 5/19, but no deterioration of the overall clinical status was observed; 4) although some of the undiagnosed cases were examined elsewhere, a certain diagnosis was never achieved in spite of their undergoing sophisticated and expensive clinical, laboratory and X-ray tests. Therefore it is concluded that: 1) persistent LGF should be managed more conservatively than fever of unknown origin so as to preserve resources; 2) some diseases should be included in the differential diagnoses from the beginning of the initial clinical work up; 3) undiagnosed LGF fever either subsides and returns to normal within one year or the fever persists, but no deterioration of the clinical and performance status is likely to occur.     

Utility of an integrated clinical, echocardiographic, and venous ultrasonographic approach for triage of patients with suspected pulmonary embolism

The potential role of ultrasound techniques in diagnosing acute pulmonary embolism (PE) has been investigated in severe cases with hemodynamic compromise, but is still unclear for the whole clinical spectrum of patients with suspected PE. The aim of this study was to assess the utility of an integrated bedside evaluation for PE based on the combination of a clinical score, 2-dimensional echocardiography, and color venous duplex scanning. A group of 117 consecutive patients with suspected PE was assessed using a clinical likelihood score, echocardiography, and venous duplex scanning in order to obtain a preliminary diagnosis of PE, which was subsequently compared with the final diagnosis obtained by lung perfusion scintigraphy and angiography. A preliminary diagnosis of PE was made in 70 patients; a final diagnosis of PE was made in 63 patients, of which 56 had and 7 did not have a preliminary diagnosis of PE. The preliminary diagnosis therefore showed 89% sensitivity and 74% specificity, with a total accuracy of 82%. In patients with massive PE, sensitivity and negative predictive values of the preliminary diagnosis were 97% and 98%, respectively. Echocardiography was poorly sensitive (51%) but highly specific (87%) for PE. Thus, the integration of clinical likelihood, echocardiography, and venous duplex scanning provides a practical approach to patients with suspected PE, allows the rapid implementation of appropriate management strategies, and may reduce or postpone the need for further instrumental evaluation of more limited access.     

Early diagnosis of acute intestinal ischaemia: contribution of colour Doppler sonography

The aim of this study was to evaluate the contribution of colour Doppler sonography in the diagnosis of acute intestinal ischaemia. In a two years experience, all patients admitted for acute abdominal pain in our emergency department were evaluated with colour Doppler sonography of the abdomen. The final diagnosis based on clinical evolution, endoscopic or surgical findings and further radiological investigations was compared to the sonographic results. Therapy and final outcome of the patients with acute intestinal ischaemia were also evaluated. In twenty-one patients a final diagnosis of acute intestinal ischaemia (mesenteric ischaemia (n = 13) and ischaemic colitis (n = 8)) was made. Intestinal ischaemia was correctly diagnosed by initial clinical and biological data and further confirmed by sonography in eight cases (mesenteric ischaemia (n = 2) and ischaemic colitis (n = 6)). Eleven other cases were detected by suggestive colour Doppler sonography features (mesenteric ischaemic (n = 10) and ischaemic colitis (n = 1)). Sixteen of the 21 patients had a final favourable outcome (mesenteric ischaemia (10/ 13) and ischaemic colitis (6/8)). We conclude that sonography has a place in the diagnosis of acute intestinal ischaemia and has to be integrated in the diagnostic algorithm of this acute condition. By this way, this diagnosis may be suspected earlier and may allow a prompt and adapted treatment with possible improvement in survival rate.     

Fever in the returned traveler

The most important cause of fever in the returned traveler is malaria. All febrile patients in which malaria is epidemiologically possible require urgent evaluation for P. falciparum malaria, which can be rapidly fatal in the nonimmune patient. Early diagnosis and therapy can prevent severe morbidity and mortality. Other less common causes of undifferentiated fever include acute schistosomiasis, the enteric fevers, rickettsial diseases, leptospirosis, and dengue fever. Early empiric therapy for suspected leptospirosis and the rickettsial infections is encouraged to decrease morbidity and mortality. About a quarter of febrile patients do not have an etiologic agent determined for their illness but recover without sequelae. Patients with fever and hemorrhagic manifestations within 3 weeks of their return need to be isolated for the remote possibility of a highly transmissible agent. Although the febrile traveler is always a challenge, the real world differential diagnosis is limited and a systematic approach via the history, physical examination, and selected laboratory tests is usually sufficient to confirm the diagnosis or eliminate potentially serious infections.     

Pigmented villonodular synovitis of the temporomandibular joint with invasion of middle cranial fossa

Forty six attacks of acute rheumatic fever (ARF) in forty patients were diagnosed between November 1987 and August 1995. Thirty four were initial attacks and 12 were recurrences. Arthritis was the commonest feature, 84.8%. Carditis occurred in 65.2% of the group, 67.6% of the initial attacks and 58.3% of the recurrences; however, the frequency of moderate/severe carditis was higher in recurrences, 25% versus 11.8%. Of those with carditis, mitral regurgitation occurred in 93.3%, aortic regurgitation in 16.7% and significant tricuspid regurgitation in 6.7%. Mitral stenosis was not encountered. No mortality occurred during ARF. Chorea, erythema marginatum and subcutaneous nodules were infrequent. These data are similar with those from a previous study which demonstrated the mild nature of ARF in Saudi Arabia, but showed higher frequency of carditis and suggested the frequency of carditis was not significantly higher during recurrences as compared to frequency of moderate/severe carditis.     

Aortic stenosis. Occurrence, pathogenesis, diagnostic strategy and therapeutic indications

Incidence, pathogenesis, diagnostic strategy and indications for treatment. Aortic stenosis is a serious disease which should be diagnosed early because of the good operative results. For this reason it is important to be aware of the disease particularly in the elderly and in patients with a history of rheumatic fever (e.g. immigrants). The diagnosis should be suspected in the patient with one or more of the three following symptoms: dyspnoea, angina pectoris and syncopes, and who has a systolic ejection murmur at the base of the heart with transmission to the neck and a reduced or absent second heart sound. First priority next to routine examinations (stethoscopy, ECG, x-ray of the chest) is referral to echocardiography. The echocardiographic results in combination with the patient's history and the clinical examination almost always form a sufficient basis for the timing of the operation. Indications for operation are given.     

Clinical and echocardiographic findings in patients with suspected acute aortic dissection

OBJECTIVES: The objective of this study was to define the range of clinical presentations, echocardiographic findings, and underlying final diagnoses in patients with clinically suspected acute aortic dissection. METHODS AND RESULTS: This study was designed as a retrospective review of clinical and echocardiographic data in consecutive patients evaluated for clinically suspected acute aortic dissection. The study population consisted of 75 studies in 74 consecutive patients referred for urgent or emergency evaluation because of signs and symptoms suggesting acute aortic dissection. A history and physical examination designed to elicit the cause of chest pain, evidence of congestive heart failure, and other cardiovascular abnormalities was performed in each patient. All patients underwent transesophageal echocardiography by experienced operators. Routine 12-lead electrocardiograms and chest radiographs were available for review in the majority of patients. Magnetic resonance imaging or computed tomography was performed in only 5 (6%) and 34 (44%) patients, respectively. Contrast aortography was performed in 21 (27%) patients. For the entire patient cohort, the most prevalent symptom was chest pain alone (n = 31; 41%) or chest pain in conjunction with back pain (n = 23; 31%). Classic "tearing" pain was an infrequent symptom. Syncope or other neurologic findings were present in 15 (20%) patients. Acute aortic dissection was responsible for 34 (45%) of the 75 presentations, with 31 (41% of total evaluations, 92% of dissections) involving the ascending aorta (Stanford type A, DeBakey type 1 or 2). Alternate major cardiovascular diagnoses, including acute myocardial infarction, primary valvular disease, or pericardial disease, were established in 12 (16%) cases. Aortic pathology, other than dissection, was found in 15 (20%) cases. Transesophageal echocardiography established the diagnosis responsible for the symptoms in 61 (81%) cases. CONCLUSIONS: Symptoms in patients with acute aortic dissection are more variable than commonly recognized. Transesophageal echocardiography is an accurate primary diagnostic tool in patients with clinically suspected acute aortic dissection. It allows rapid diagnosis of dissection and can identify alternate cardiovascular pathology responsible for the symptoms in a significant number of patients without acute dissection.     

C-reactive protein as a marker for acute coronary syndromes

BACKGROUND: For several years, acute coronary syndromes have been perceived as causing the most hospital admissions, and even hospital mortality. The syndrome of unstable angina frequently progresses to acute myocardial infarction but its pathogenesis is poorly understood, and prognosis determination is still problematic. We tested the hypothesis that measurement of the C-reactive protein in patients admitted for chest pain could be a marker for acute coronary syndromes. METHODS AND RESULTS: We studied 110 patients admitted with suspected ischaemic heart disease, but without elevated serum creatine-kinase levels at the time of hospital admission. Patients were subsequently divided into two groups based on their final diagnosis: group 1 comprised patients with unstable angina; group 2 patients with acute myocardial infarction. We measured the C-reactive protein at the time of hospital admission. The concentration of C-reactive protein was elevated in 59% of the patients with a final diagnosis of acute myocardial infarction, and in 5% of the patients with a final diagnosis of unstable angina, (P < 0.001). CONCLUSION: This study indicates that C-reactive protein levels measured at the time of admission in patients with suspected ischaemic heart disease could be a marker for acute coronary syndromes, and helpful in identifying patients at high risk for acute myocardial infarction. Measurement of C-reactive protein may have practical clinical significance in the management of patients hospitalized for suspected acute coronary syndromes.     

Craniosynostosis: is a prenatal diagnosis possible?

We report the case of a 62-year-old man with massive pulmonary embolism and severe hemodynamic impairment. Transthoracic Doppler echocardiography was fundamental in confirming the diagnosis by direct visualization of intra-atrial thrombus and signs of right chamber overload. It allowed prompt administration of thrombolytic drug and follow-up monitoring. Doppler echocardiography is a non-invasive, available technique and its early application should be considered in the evaluation of patients with suspected massive pulmonary embolism.     

Case management study: polyarthritis with fever

In most cases, a thorough initial evaluation will reveal the cause of fever and polyarthritis. However, in some patients the initial diagnosis may be unclear and, as time passes, the characteristic clinical patterns emerge. Recurrent attacks are suggestive of other conditions such as crystal-induced arthritis, Lyme disease, and Mediterranean fever. In rheumatoid arthritis and Reiter's syndrome, the fever resolves and the articular findings predominate with the passage of time. Similarly, Still's disease is initially diagnosed on the basis of clinical criteria, and later confirmed by the evolution of chronic polyarthritis. Diagnostic approaches for the evaluation of patients presenting with acute arthritis have been published and are readily available (2,8,9). The most reliable way to establish the diagnosis for a rheumatic disease is thoughtful and thorough evaluation by an experienced clinician (3,10). Certain discriminating features and confirmatory tests can aid in the diagnosis of polyarthritis with fever (Tables 2 and 3).     

The effect of prevention and other factors in the decrease in rheumatic fever

933 children with rheumatic fever hospitalised in Clinic in the period of 40 years (from 1995 to the end of 1994) were analysed in order to establish the fluctuation of patients number and changes in clinical appearance. Patients were divided in 4 groups: I. the patients hospitalised from 1955-1964 (472 patients), II. from 1965-1974 (307), III. from 1975-1984 (135), IV. from 1985-1994 (19). The data for Republic Croatia show the steady fall of the number of hospitalised children with rheumatic fever. Carditis with polyarthritis were present in the largest part of our patients (446 namely 47.8%). The number of recurrences fell equally with the number of patients with rheumatic fever. However, ratio between the number of patients with rheumatic fever and the number of recurrences did not change essentially, that was 11-15.85% in the periods considered. The percent of hospitalised children with fixed rheumatic heart disease was 4.23% in the I. period to as much as 15.5% in the III. period from totally hospitalised children with rheumatic fever. In the last time the disease became mild in its development. All children with suspicion on rheumatic fever should be hospitalised. The prophylaxis, primary or secondary, should be carried out intramusculary with benzithine penicillin G, as proved as the best, in order to ensure that the child virtually received the prophylaxis.     

Vaccinating guinea pigs with recombinant glycoprotein D of herpes simplex virus in an efficacious adjuvant formulation elicits protection against vaginal infection

PURPOSE: This study was designed to develop improved criteria for the diagnosis of infective endocarditis and to compare these criteria with currently accepted criteria in a large series of cases. PATIENTS AND METHODS: A total of 405 consecutive cases of suspected infective endocarditis in 353 patients evaluated in a tertiary care hospital from 1985 to 1992 were analyzed using new diagnostic criteria for endocarditis. We defined two "major criteria" (typical blood culture and positive echocardiogram) and six "minor criteria" (predisposition, fever, vascular phenomena, immunologic phenomena, suggestive echocardiogram, and suggestive microbiologic findings). We also defined three diagnostic categories: (1) "definite" by pathologic or clinical criteria, (2) "possible," and (3) "rejected." Each suspected case of endocarditis was classified using both old and new criteria. Sixty-nine pathologically proven cases were reclassified after exclusion of the surgical or autopsy findings, enabling comparison of clinical diagnostic criteria in proven cases. RESULTS: Fifty-five (80%) of the 69 pathologically confirmed cases were classified as clinically definite endocarditis. The older criteria classified only 35 (51%) of the 69 pathologically confirmed cases into the analogous probable category (p < 0.0001). Twelve (17%) pathologically confirmed cases were rejected by older clinical criteria, but none were rejected by the new criteria. Seventy-one (21%) of the remaining 336 cases that were not proven pathologically were probable by older criteria, whereas the new criteria almost doubled the number of definite cases, to 135 (40%, p < 0.01). Of the 150 cases rejected by older criteria, 11 were definite, 87 were possible, and 52 were rejected by the new criteria. CONCLUSION: Application of the proposed new criteria increases the number of definite diagnoses. This should be useful for more accurate diagnosis and classification of patients with suspected endocarditis and provide better entry criteria for epidemiologic studies and clinical trials.     

Transesophageal echocardiography in mitral prosthesis dysfunction: usefulness and limitations in the evaluation of mitral insufficiency

This study was performed to test the usefulness of transesophageal echocardiography in the diagnosis and assessment of pathological mitral regurgitation in patients with mitral valve prostheses. Doppler color flow imaging by transesophageal echocardiography was compared to the transthoracic echocardiography and angiographic and surgical assessment. We analyzed the influence of the spatial configuration of the jet on the semiquantitative assessment of mitral regurgitation. We studied 71 patients with prostheses in mitral position which were submitted for transesophageal echocardiography examination. 51 of these patients were found to have a pathological prosthetic regurgitation that was confirmed in 21 cases by left ventriculography and in 4 during cardiac surgery. Transesophageal echocardiography Doppler color flow imaging identified a regurgitant jet in 31 patients (60.7%). There was complete agreement with the quantitative assessment of regurgitation by angiography or surgery in 36% of the cases. All patients with prosthetic insufficiency observed by angiography or during cardiac surgery were confirmed by transesophageal echocardiography. Complete agreement in grade of severity by transthoracic echocardiography was found in 84% of cases. There was a difference in grade of severity of mitral regurgitation in only 4 patients. Regurgitant jets were classified by transesophageal echocardiography color Doppler in two groups: free jets and impinging wall jets. 21 cases presented a free jet and 31 excentrically directed impinging wall jet of mitral regurgitation. There was complete agreement with hemodynamic assessment of severity in all patients with regurgitant free jets (11/11). In presence of jet wall there was understimation of mitral regurgitation in 28.5% (4/13).(ABSTRACT TRUNCATED AT 250 WORDS)     

Value of clinical features in the diagnosis of enteric fever

There is no objective data on the value of individual clinical symptoms or signs in the diagnosis of enteric fever in a febrile patient. The purpose of the study was to assess the value of some clinical and simple laboratory features in the diagnosis of enteric fever. One hundred & six patients with microbiologically confirmed enteric fever and 170 patients with other established febrile illnesses were included in the evaluation. History of stepladder pattern of rise of temperature, loose motions, relative bradycardia and coated tongue proved to be powerful markers of enteric fever with high specificity (100%, 94.71%, 94.71%, 94.12% respectively), positive and negative predictive values. Headache, hepatomegaly and splenomegaly were moderately powerful. ESR and WBC count appeared to have little value in the diagnosis of enteric fever. Pattern of onset and loose motions did not discriminate between typhoid and paratyphoid fever. Most of these patients had illness persisting beyond one week by which viral infections and infectious enterocolitides were largely excluded. Elucidation of power of these markers in distinguishing enteric fever from other febrile illnesses with the help of better designed prospective studies would lessen our dependence on expensive and time consuming laboratory investigations.     

The dynamics of the tissue oxygen regimen in patients with circulatory encephalopathy under the influence of oxygen baths]

AIM: To compare the proposed diagnostic criteria of subacute infectious endocarditis (SIE) to criteria developed by von Reyn et al. and by Duke Endocarditis Service. MATERIALS AND METHODS: 69 SIE cases and suspected recurrences have been analysed for patients observed in the Therapeutic Clinic of the Moscow medical University in 1990-1997. RESULTS: According to the authors' criteria accurate and tentative SIE diagnosis were made in 82 and 18% of patients, respectively. The other two diagnostic approaches in this situation increase the percentage of presumptive diagnosis and decrease that of the definite one. CONCLUSION: The criteria proposed by the authors are more sensitive in diagnosis of definite SIE, are less dependent on echocardiography quality and bacteriological diagnosis.     

Seroprevalence of B19 parvovirus infection in patients with acute polyarthritis

Over a period of 29 months, from January 1991 to December 1994, all cases of acute polyarthritis seen at the Rheumatology Service in our Institution were studied to determine the seroprevalence of parvovirus B19 (B19) infection. The variables studied included: age and sex of patients, presence of fever and rash, Anti-B19 IgM and IgE serological determinations (ELISA, Mardix Lab.), follow-up time and final diagnosis. The study included 36 patients (22 women and 14 men, mean age 34 +/- 19 years). Thirteen and seven patients had fever and cutaneous rash, respectively. Anti-B19 IgM serology was positive in 4 patients; in 2 of them IgG seroconversion was confirmed. The mean follow-up time was 14 +/- 9 months. Final diagnoses included undifferentiated polyarthritis, rheumatoid arthritis, B19 polyarthritis, systemic lupus erythematosus, and miscellaneous in 19, 7, 4, 2, and 4 patients, respectively. Seroprevalence of B19 infection in acute polyarthritis in our area was 11%, approximately.     

Institute of Rheumatology and progress of antirheumatic therapy

The paper deals with the achievements of the Institute of Rheumatology in antirheumatic therapy, among them there are methods of objective assessment of antirheumatic drugs, the first use of antimalarials in the treatment of chronic rheumatic fever, discovery of immunodepressive properties of these drugs, specification of the mechanism of action of several NSAIDs. Antilymphocytic globulin, salazopyridazine and the alkylating drug dopan were used for the first time in therapy of rheumatic diseases. Administration of the most potent NSAIDs diclofenac or indomethacin to patients with acute rheumatic fever proved to be as effective as prednizolone. Special attention is paid to the combination treatment of rheumatoid arthritis with NSAIDs. The concurrent administration of aurannofin and methotrexate was shown to cause a more rapid development of clinical improvement than monotherapy with either drug. A combination of gold aurothiomalate and hydroxychloroquine and that of low doses of D-penicillamine and cyclophosphamide had no advantages over monotherapy. Revealing the therapeutical potential of antibodies to interferon-gamma in the treatment of rheumatic arthritis and psoriatic arthritis was the most important achievement of recent years. These studies open new vistas for anticytokine treatment of rheumatic diseases.