Ann Thorac Surg 2004;78:699-701
© 2004 The Society of Thoracic Surgeons
Case report
Recurrent acute rheumatic fever: a forgotten diagnosis?
Isaac S. Kadir, FRCSa*,
Thomas A. Barker, MRCSa,
Bernard Clarke, FRCPb,
Helen Denley, MRCPathc,
Geir J. Grötte, FRCSa
a Departments of Department of Cardiothoracic Surgery, Manchester, UK
b Department of Cardiology, Manchester, UK
c Department of Pathology, Manchester Royal Infirmary, Manchester, United Kingdom
Accepted for publication June 23, 2003.
* Address reprint requests to Dr Kadir, Department of Cardiothoracic Surgery, Manchester Royal Infirmary, Oxford Rd, Manchester, M13 9WL UK
e-mail: ikadir{at}aol.com
 |
Abstract
|
|---|
The incidence of acute rheumatic fever has seen a dramatic decline over the last 15 to 20 years in most developed countries and treatment of this disease has changed little since. The ease of travel and immigration and the cosmopolitan nature of many cities mean that occasionally the disease will come to the attention of clinicians not familiar with its presentation, resulting in delayed diagnosis and treatment. We present a case of recurrent acute rheumatic fever in a patient who was initially thought to be suffering from acute bacterial endocarditis on her previously diseased rheumatic aortic valve. This culminated in her undergoing urgent aortic valve replacement during a phase of the illness that should have been treated with high dose anti-inflammatory medication. Therefore, clinicians should be aware of this condition and include it in their differential diagnosis of the febrile patient with a previous history of rheumatic fever. We briefly discuss the diagnostic dilemma of patients suffering from this condition and in differentiating it from acute endocarditis.
|
Introduction
|
|---|
At the dawn of the new millennium acute rheumatic fever continues to be a major health problem in many parts of the world with approximately one million new cases yearly in India. In developing countries it accounts for 35% to 40% of cardiovascular disease-related hospital admission and is the predominant indication for cardiac surgery [1]. Although considered to be a disease of developing countries, rheumatic fever persists even among the prosperous population in developed countries. Rheumatic heart disease, the sequel to acute rheumatic fever is an important predisposing condition for acute endocarditis. Not infrequently the clinical manifestation of recurrent rheumatic heart disease mimics that of acute endocarditis, giving rise to a diagnostic dilemma. We present such a case in which a patient initially thought to have acute endocarditis underwent surgery. Operative and histologic findings of a pancarditis and later manifestation of the rheumatic activity eventually resulted in a revised diagnosis of recurrent rheumatic fever and successful drug treatment.
A 20-year-old Bangladeshi female who had immigrated to England 20 months previously was referred to our department with a provisional diagnosis of acute infective endocarditis. At the age of 11 years she developed fever, chest pains, and arthralgia following a sore throat and was treated for acute rheumatic fever and followed up thereafter. She received intramuscular penicillin injections for the next 5 years for prophylaxis against recurrent streptococcal infection. At the age of 16 she was diagnosed as having rheumatic mitral and aortic valve disease. On arriving in England she was seen monthly for 6 months by a cardiologist who confirmed severe aortic regurgitation and mild mitral stenosis. She had occasional episodes of paroxysmal supraventicular tachycardias.
Six weeks prior to her current presentation she had complained of a sore throat and her family practitioner had prescribed a course of antibiotics, which she failed to complete. Not surprisingly, as she was a member of a large extended family, her two brothers also had sore throats around the same time and they too were treated with antibiotics. Three weeks later she had difficulty walking as a result of swollen painful feet and ankles. Her knees, although not swollen, were also exquisitely painful. This was initially treated with naproxen with little effect but diclofenac relieved her symptoms within 24 hours. At around the same time she also complained of abdominal pains and epistaxis. There were no complaints of any neurologic symptoms, skin or subcutaneous lesions. Two weeks later she attended the cardiology outpatient department for a routine check up, and echocardiography revealed her valves to be unchanged from previous visits. Shortly after, a week prior to admission, she complained of extreme fatigue, fever, chest pain and dyspnea. Importantly, she had not travelled out of the country since arriving in England, and had not undergone any invasive medical or dental procedures.
She was admitted to her local hospital where she was noted to be pyrexial (38.8°C) with a pulse rate of 90 per minute and a blood pressure 120/80 mm Hg. Examination findings were consistent with overt congestive cardiac failure and initial laboratory findings included a hemoglobin 7.7 g/dL, white cell count (WCC) 12.1x109/L, C-reactive protein (CRP) 209 mg/L, and erythrocyte sedimentation rate (ESR) 112 mm/first hour. Her chest roentgenogram revealed congested lung fields consistent with cardiac failure. Electrocardiogram showed first-degree heart block. A transthoracic echocardiography the following day revealed moderate aortic and mitral regurgitation, and a dilated left ventricle. No vegetations were seen. An initial diagnosis of acute infective endocarditis was thought to be highly likely and antibiotics (benzylpenicillin and gentamicin) and antifailure medication commenced prior to transfer to the regional cardiac center.
Following transfer, transoesophageal echocardiography revealed a severely regurgitant nonstenotic trileaflet aortic valve with the suspicion of a vegetation. There was mild mitral stenosis and the left ventricle was mildly dilated and hypertrophied with good overall contractility. There were no aortic root abscesses. The antibiotics were changed following microbiologic advice (amoxycillin, vancomycin, and gentamicin) and she was observed for a further 48 hours. By this time five sets of blood cultures were sterile and as inflammatory markers were rising (WCC 14x109/L, CRP 326 mg/L) with persistent congestive heart failure despite appropriate medical treatment, surgical intervention was indicated.
At operation the pericardial cavity was completely obliterated with fleshy adhesions and its entire contents was one homogenous mass. The pericardium was thickened (3 to 4 mm) with few areas of calcification. The epicardium was edematous and grossly thickened (3 mm). It was intensely inflamed, erythematous and deeply injected. At this point the surgeons suspected carditis from recurrent rheumatic fever. Following a laborious dissection, cardiopulmonary bypass was established and aortic valve replacement performed using a 19-mm St Jude bileaflet mechanical valve. There was no naked eye evidence of infection on the aortic valve nor any vegetations or abscesses. The valve was clearly rheumatic and the non-coronary cusp was seen to be prolapsing into the left ventricle. The aortic annulus was thickened and edematous. Although the postoperative course was uneventful the antibiotic regime initiated prior to her operation was continued on microbiologic advice. The throat swab and all the blood cultures sent previously were sterile. All atypical serology were negative and ASO titer was above 1 in 1000 dilution.
No organisms were seen on microscopy of the valve and all cultures of the valve were negative. Histopathologic examination of the valve, pericardium, and epicardium demonstrated nonspecific acute inflammation with polymorph infiltration, marked edema, and focal fibrin formation. The valve showed full thickness inflammation and special stains for bacteria and fungi were negative. Electron microscopy revealed abundant fibrin and polymorph infiltration with no evidence of any organisms or their remnants to be present. Although no Aschoff bodies were seen, the above appearance strongly favored a diagnosis of acute rheumatic heart disease. Precautionary antibiotics were continued in the postoperative period while the various results were awaited. Her inflammatory markers continued to climb; by the third postoperative day, the CRP was 611 mg/L, WCC 18.8x109/L, and there was a persistent pyrexia. The antibiotics were changed to clarithromycin and rifampicin following which the CRP, WCC, and pyrexia started to decrease gradually. The patient developed a painful migratory effusive polyarthritis involving her knees and interphalangeal joints on both limbs in the postoperative period. A rheumatology review suggested that the clinical presentation was highly characteristic of recurrent acute rheumatic fever and treatment with high dose aspirin (1 g/day) and prednisolone (15 mg/day) commenced after which there was a gradual resolution of all symptoms and a rapid fall in inflammatory markers (WBC 4.1x109/L, CRP 16 mg/L). She was discharged 9 weeks following admission on oral penicillin V.
|
Comment
|
|---|
Rheumatic fever (RF) is a condition that usually follows a pharyngeal infection with a virulent strain of group A streptococcus [2]. The disease is characterized by damage to connective tissue in a variety of organs, most commonly heart, joints, central nervous system, and subcutaneous tissue. In the 1940s, T. Duckett Jones adopted these manifestations of acute rheumatic fever and proposed a criterion for its diagnosis [3]. As laboratory medicine evolved these criteria were subsequently modified to include such findings that include elevated C-reactive protein, erythrocyte sedimentation rate, white blood count, and positive microbiologic culture.
While active carditis remains a major criterion its diagnosis can be extremely difficult especially when it is the isolated manifestation of the disease or when it occurs on preexisting rheumatic heart disease. Because valvulitis constitutes the sine qua non of rheumatic carditis, recurrent carditis on a background of previous rheumatic valvular disease often gives rise to a changing or new murmur. It is this fact together with the associated systemic manifestation of fever, lethargy, and chest pain that explain why it is sometimes indistinguishable from the clinical manifestation of acute endocarditis. Whereas echocardiography has been demonstrated to be more sensitive than clinical examination in demonstrating valvular involvment in the early and subclinical phase of rheumatic fever, its use ultimately has not proved to be superior. In addition, the ability of Doppler echocardiography to detect subclinical recurrence of carditis in the presence of preexisting rheumatic heart disease remains obscure [4]. Doppler echocardiography, on the other hand, is not specific for either rheumatic carditis or endocarditis as is amply illustrated in this case. What appeared to be an endocarditic vegetation was in fact a prolapsing noncoronary leaflet of the aortic valve into the left ventricular outflow tract; a manifestation of active valvulitis. In the original Jones criteria for the diagnosis of rheumatic fever a previous history of rheumatic fever or evidence of rheumatic heart disease was a major criterion [3]. This was later down graded to a minor manifestation and subsequently removed all together. It is to be noted, however, that the Jones criteria is more pertinent to the diagnosis of a first episode of rheumatic fever and any new cardiovascular manifestation in a patient with a previous history of rheumatic fever should heighten our suspicion that we are indeed witnessing a recurrence. The so-called "minor" criteria in these recurrent cases assumes more than minor importance. In this case the patient had two major criteria (carditis and polyarthritis) and all the designated minor criteria (raised ESR/CRP and electrocardiogram evidence of PR interval prolongation). She also suffered from epistaxis and abdominal pains that, although not minor criteria, are strongly associated with the diagnosis.
The mitral valve was not visualized at surgery due to the extreme difficulty in mobilizing the heart. Echocardiography confirmed fairly normal looking mobile mitral leaflets. Mitral valve area by pressure half-time revealed only mild mitral stenosis and this may even be artificially elevated in view of her severe aortic regurgitation.
Secondary prevention of rheumatic fever in the form of continuous antibiotic prophylaxis with penicillin orally or intramuscularly is recommended throughout the world as streptococci infections resistant to penicillin has never been proven [5]. The duration of this prophylaxis depends on a clinical decision based on severity of attack, presence of rheumatic heart disease, prevalence of rheumatic fever in the patient population, and the intention of the patient to travel to undeveloped countries. As this patient has had at least two episodes of carditis, including a life-threatening episode, she is at high risk of developing a recurrence and antibiotic prophylaxis will be lifelong.
The case highlights an episode of acute recurrent rheumatic fever in a young female in a developed country. While infective endocarditis is more likely in this population, the clinical features and diagnostic algorithm is often forgotten resulting in inappropriate and delayed treatment. Furthermore, the importance of secondary prophylaxis even in what appears to be low-risk population is clearly apparent from this case.
|
References
|
|---|
- Vijaykumar M., Narula J., Reddy K.S., Kaplan E.L. Incidence of rheumatic fever and prevalence of rheumatic heart disease in India. Int J Cardiol 1994;43:221-228.[Medline]
- Stollerman G.H. Rheumatic fever. Lancet 1997;349:935-942.[Medline]
- Jones T.D. Diagnosis of rheumatic fever. JAMA 1944;126:481-484.
- Abernathy M., Bass N., Sharpe N., et al. Doppler echocardiography and the early diagnosis of carditis in acute rheumatic fever. Aust N Z J Med 1994;24:530-535.[Medline]
- McLaren M.J., Markowitz M., Gerber M.A. Rheumatic heart disease in developing countries, the consequence of inadequate prevention. Ann Intern Med 1994;120:243-245.[Free Full Text]
Top
Abstract
Introduction
