Author: Nikil Naveel Chand University of Fiji - Umanand Prasad School of Medicine & Health Sciences Year 3 MBBS Student.
Acute rheumatic fever (ARF) is an acute inflammatory disease of children and young adults caused by infection with pharyngeal strains of group A beta hemolytic streptococcus type 1, 3, 5, 6 and 18 (Carroll, Morse, Mietzner & Miller, 2016). The disease is called the “poor man's disease as its more prevalent in communities where overcrowding and poor sanitation is common (Ralston, Penman, Strachan, Hobson, Britton & Davidson, 2018). The diagnosis of acute rheumatic fever (ARF) requires professionals who are aware of the diagnostic sign’s.
Acute rheumatic fever is a result of the host response to group A streptococcus antigen that cross react with the hosts proteins (Kumar & Clark, 2017). CD4 + helper T cell and plasma antibodies fight against Streptococcus M protein. Antibodies later attack M protein in Cardiac Mitral valve due to similar protein structure which is known as Molecular mimicry (Robbins, Cotran, Kumar, Abbas & Aster, 2015). Antibody binding can activate complement, as well as recruit Fc-receptor bearing cells (neutrophils and macrophages); cytokine production by the stimulated T cells leads to macrophage activation (Robbins et al., 2015).
It usually takes 14-19 days for an individual to develop acute rheumatic fever from the day of infection (Carroll et al., 2016). ARF is a multisystem disorder affecting the circulatory system, integumentary system, nervous system, and the skeletal system. The most common presenting signs will be fever, anorexia, lethargy and a past history of strep throat infection.
Rheumatic fever causes pancarditis but the condition declines statically as the individual ages (UpToDate, 2020) (Ralston et al., 2018).
Breathless is manifested due to the ongoing heart failure or pericardial effusion.
Palpation is present due to pancarditis.
Presence of a systolic murmur due to mitral regurgitation. Carey Coombs murmur may be present.
Pericardial rub presence upon auscultation.
ECG readings will show a ST and T wave change.
(Kumar & Clark, 2017).
Histology – In ARF, focal lessons will be found on the internal heart walls termed Aschoff bodies consisting of foci of T lymphocytes and occasional plasma cell and plump activated macrophages called Antischkow cells (pathognomonic for RF) (Robbins et al., 2015).
Major manifestation occurs when streptococcal antibody titer is high. It shows acute symptoms such as painful, asymmetric and migratory inflammation of the large joints. The involved joints are presented as red, swollen and tender for approximately 4 weeks .
(Ralston et al., 2018).
Small lumps and bumps under the skin. Most prominent at bony prominences. Their presence strongly points to carditis (Ralston, 2018). The nodules measure about few millimeters to 2cm in size, are firm and painless (UpToDate, 2020).
Presents with pink or faintly red, non-pruritic rash involving the trunk and sometimes the limbs but not the face (UpToDate, 2020). It may persist intermittently for weeks to months, even after successful treatment of ARF (UpToDate, 2020).
St Vitus Dance or Sydenham’s, occurs 3-6 months after the onset of RF (Ralston, 2018). Emotional lability may be the first feature and is typically followed by unnecessary involuntary choreiform movements of the hands, feet or face. Speech may be sudden and halting. Spontaneous recovery usually occurs within a few months (Millichap, 1993).
A diagnosis of ARF is reached by a medical professional by following the JONES criteria.
Prolonged PR Interval
Anamnesis of Rheumatism
To make a diagnosis of ARF, there has to be a positive throat culture growing GAS or elevated anti-streptolysin O titer and there has to be 2 major criteria present or one major criteria and 2 minor criteria present.
Goals of treatment - The four major goals of treatment are:
Symptomatic relief of acute disease manifestations (eg, arthritis).
Eradication of group A beta-hemolytic Streptococcus (GAS).
Prophylaxis against future GAS infection to prevent progression of cardiac disease.
Provision of education for the patient and patient’s caregivers.
Symptomatic Treatment of Acute Rheumatic Fever
The symptoms of acute rheumatic fever should be managed as follows:
Paracetamol and/or codeine should be used to manage arthritis, fever, and other acute symptoms until the diagnosis of ARF is confirmed.
Once confirmed, high dose salicylates (aspirin) are the preferred choice, although other NSAIDs (e.g. ibuprofen) may be equally effective. Steroids may rarely be needed. Use: Aspirin 60-100 mg/kg/day, orally, given in 4 divided doses (maximum of 8 g/day) for 1-2 weeks, then wean according to clinical response and inflammatory markers (CRP, ESR): usual duration 6-8 weeks but longer duration may be required.
Carditis resulting in heart failure is treated with standard therapies (diuretics, ACEI), and cardiac arrhythmias that may develop are treated accordingly. Steroids have been used, but conclusive evidence of their efficacy is limited. Where a decision is made to use steroids in severe carditis, use: Prednisolone 1-2 mg/kg/day to a maximum dose of 80 mg daily: therapy is usually continued for 1-3 weeks; where >1 week is required, wean by 20-25% each week.
In severe disease, bed rest is recommended.
Chorea is usually managed conservatively, however carbamazepine and sodium valproate may be used for control.
(Cardiovascular Therapeutic Guidelines, 2015)
All patients with RF are given long term prophylaxis for prevention of recurrence.
Prophylaxis is given in the form of benzathine penicillin 1.2 million units if weight is more than 20kg by deep IM into the gluteal region once in 4 weeks. If the weight is less than 20kg than use 0.6 million units single dose once in 4 weeks (Kumar & Clark, 2017).
As an alternative, penicillin V may be given at a dose of 500mg orally twice a day for adults and 250mg orally twice a day for children. Patients allergic to penicillin should be given erythromycin 500mg (child 12.5mg per kg up to 500mg) twice daily for 10 days (UpToDate, 2020).
Prophylaxis is life-long in the presence of rheumatic carditis and chronic rheumatic heart disease. In other cases, prophylaxis should be given until the patient reaches the age of 40 years or for 5 years after the last attack of RF, whichever is the longer period. Prophylaxis for RF does not exempt patients with chronic rheumatic valvar heart disease from requiring prophylaxis for infective endocarditis.” (Kumar & Clark, 2017).
Caregivers and Patient Education
It is critical that patients and their caregivers receive adequate information about ARF and RHD. The most critical advice is the information about continuing their prophylaxis even though they are not showing any signs and symptoms.
It is essential to educate patients about the importance of treating sore throats early and recommending a non-penicillin antibiotic (eg, clindamycin) for this if the patient is taking penicillin prophylaxis.
Awareness on oral health and hygiene is important in these patients because they are at high risk of developing complication such as infective endocarditis. Thus, patients should receive education about dental care. Caregivers of patients with carditis should be encouraged to inform their doctor or dentist prior to any interventional procedure so that appropriate antibiotic prophylaxis can be given.
Given the risks of ARF/RHD in pregnancy and labor, every female of childbearing age receiving secondary prophylaxis should also be given detailed family planning/contraception advice as an integral part of the education program. During pregnancy, the patients should consult their obstetricians and inform them of their prophylactic treatment
Statistically about 50 percent of the patients will develop chronic rheumatic valve heart disease after 15 years. The most affected valve in this condition is mitral valve, followed by aortic valve, the tricuspid valve are rarely affected (Ralston et al., 2018).