Rhematic Fever

• Delayed nonsuppurative sequalae of group A streptococcus pharyngeal infection

Pathophysiology:

• Need:
  • GAS Pharyngitis
    • Only 20-40% of pharyngitis episodes are associated with GAS infection
    • The remainder are caused by viruses or bacteria for which antibiotic treatment is not recommended
  • Genetic susceptibility
• Mechanism:
  • Molecular mimicry
  • GAStrep Pharyngitis induces a Type 3 hypersensitivity reaction
• Immune Response:
  • Antibodies against M protein of certain strains cross-react with glycoprotein antigens in the heart, joints, nerves, and other tissues
  • Only a minority of infected patients develop Rheumatic Fever (RF)
• Consequences of Recurrence:
  • Carditis:
    • Worsens with each recurrence; damage is cumulative
  • Embolization:
    • From mural thrombi
  • Infective Endocarditis:
    • Risk increases on deformed valves
  • Chronic Rheumatic Heart Disease:
    • Murmurs
    • Hypertrophy
    • Dilation
    • Heart failure
    • Arrhythmias

• Delay in Onset:
  • There is typically a 2-4 week delay between the initial streptococcal infection and the onset of RF due to the time required for the immune response to cause tissue damage.

High-Risk Groups for Acute Rheumatic Fever (ARF) and Rheumatic Heart Disease (RHD):

1. Aboriginal People and Torres Strait Islanders:
   • Especially those living in rural or remote settings.
2. Children:
   • Predominantly seen in children aged 5–14 years.
   • Recurrent episodes can occur well into the 40s.
3. Epidemiology:
   • ARF incidence: >30/100,000 per year in 5–14 year olds.
   • RHD all-age prevalence: >2/1000.

High-Risk Groups for Rheumatic Fever:

1. Age: Most common in children aged 5 to 15 years.
2. Geographic Location: Higher incidence in developing countries and areas with poor access to healthcare.
3. Socioeconomic Factors: Poverty, overcrowding, and poor living conditions
4. History of Rheumatic Fever: Previous episode of rheumatic fever increases the risk of recurrent episodes, especially if prophylactic antibiotics are not taken.
5. Family History: Genetic predisposition may play a role. A family history of rheumatic fever can increase an individual’s risk.
6. Environmental Factors: Close contact with individuals who have GAS infections.Poor hygiene and lack of access to medical care for proper treatment of streptococcal infections.

Reasons for Increased Risk of Rheumatic Fever:

1. Age:
   • Immune System Development: Children aged 5 to 15 years are more susceptible to streptococcal infections due to the ongoing development of their immune systems.
2. Geographic Location:
   • Endemic Areas: In developing countries and specific endemic regions, healthcare infrastructure may be insufficient, leading to delayed or inadequate treatment of streptococcal infections.
   • Climate and Environmental Factors: In some regions, climatic conditions and environmental factors may facilitate the spread of streptococcal bacteria.
3. Socioeconomic Factors:
   • Poverty: Low-income families often face barriers to accessing timely and adequate healthcare.
   • Overcrowding: Crowded living conditions facilitate the transmission of infectious diseases, including GAS infections.
   • Nutrition: Poor nutrition can weaken the immune system, making individuals more susceptible to infections.
4. History of Rheumatic Fever:
   • Immune Response: Individuals with a previous episode of rheumatic fever have an altered immune response that makes them more susceptible to recurrence if exposed to GAS infections again.
   • Lack of Prophylaxis: Without regular prophylactic antibiotics, the risk of recurrence remains high.
5. Family History:
   • Genetic Predisposition: Genetic factors may predispose certain individuals to an exaggerated immune response to GAS infections, leading to rheumatic fever.
   • Shared Environment: Families living in close quarters may have shared environmental risk factors, such as exposure to the same strains of streptococcus.
6. Environmental Factors:
   • Hygiene Practices: Poor hygiene and sanitation facilitate the spread of GAS infections.
   • Access to Healthcare: Limited access to healthcare services means that infections are often untreated or inadequately treated, increasing the risk of complications.
7. Healthcare Infrastructure:
   • Availability of Medical Care: In regions with limited healthcare infrastructure, there may be a lack of medical professionals, medications, and diagnostic tools necessary for the timely treatment of streptococcal infections.
   • Public Health Initiatives: Lack of public health initiatives and awareness programs can result in delayed recognition and treatment of GAS infections.

History and Examination

Preceding Illness:

1. Symptoms:
   • Typically follows streptococcal pharyngeal infection.
   • Symptoms develop 2-4 weeks after infection.
2. Cultures:
   • Throat cultures may be taken to identify group A streptococcus.
3. Timing:
   • Symptoms appear 2-4 weeks post-infection.

Vital Signs:

1. Fever:
   • Low-grade fever.

Cardiovascular System (CVS):

1. Symptoms:
   • Chest pain or discomfort.
   • Dyspnea.
2. Signs:
   • New onset mitral regurgitation (MR) or other murmurs.
   • Pericardial rub.
   • Palpitations.
   • Heart failure.

Rheumatological:

1. Symptoms:
   • Pain more than clinical inflammation.
   • Migratory arthritis involving large joints, usually starting in the legs.
   • Each joint affected for < 1 week but often overlap.

Rash:

1. Erythema Marginatum:
   • Non-pruritic rash with pale centers on the trunk and proximal limbs.

Central Nervous System (CNS) – Sydenham Chorea:

1. Symptoms:
   • Abrupt, purposeless, nonrhythmic involuntary movements, worse on one side.
   • Muscular weakness (milking sign).
   • Emotional instability.

Dermatology:

1. Subcutaneous Nodules:
   • <2 cm, firm, painless nodules over bony surfaces or near tendons.
2. Erythema Annulare:
   • Non-pruritic rash with pale centers on the trunk and proximal limbs.

Differentials

• Septic arthritis (including gonococcal arthritis)/   osteomyelitis
• Reactive arthritis
• Viral arthritis (CMV, EBV, rubella vaccination, hepatitis)
• Rheumatic fever
• Juvenile idiopathic arthritis
• Rheumatoid arthritis, IBD, SLE, vasculitis,  sarcoidosis
• Haemarthrosis
• MSK injury/ trauma/ fall (perthes,  SUFE)
• Gout/ pseudogout
• Non-­accidental injury

Diagnosis & Investigations

Currently, there is no specific laboratory test to definitively diagnose Acute Rheumatic Fever (ARF). As such, diagnosis is a clinical decision, guided by the recognition of major and minor criteria as defined by the Jones Criteria.

Confirming Diagnosis Using the Jones Criteria

Definite Initial Episode of ARF requires one of the following:

• Two major criteria, or
• One major criterion plus two minor criteria,
  with evidence of a preceding Group A Streptococcal (GAS) infection.

Definite Recurrent Episode of ARF in patients with a history of ARF or Rheumatic Heart Disease (RHD) requires:

• Two major criteria, or
• One major criterion and one minor criterion, or
• Three minor criteria,
  with evidence of a preceding GAS infection.

Major Criteria:

1. Carditis
2. Arthritis
   • High-Risk Groups: Aseptic monoarthritis
   • Low-Risk Groups: Polyarthritis
3. Chorea
4. Erythema marginatum
5. Subcutaneous nodules

Minor Criteria:

1. Arthritis
   • High-Risk Groups: Monoarthralgia
   • Low-Risk Groups: Polyarthralgia or aseptic monoarthritis
2. Fever
3. Elevated Erythrocyte Sedimentation Rate (ESR) or C-reactive Protein (CRP)
4. Prolonged PR interval on an electrocardiogram (ECG)

Evidence of Preceding Streptococcal Infection:

• Positive throat culture for Group A β-haemolytic streptococcus
• Positive rapid streptococcal antigen test
• Elevated or rising streptococcal antibody titres (most commonly antistreptolysin O; others include anti-DNAse B, streptokinase, antihyaluronidase)

Investigations:

• Evidence of preceding streptococcal infection:
• All suspected cases of ARF (except those with chorea or low-grade subacute carditis) should have elevated serum streptococcal serology:
  • ASOT (Antistreptolysin O titer) + anti-DNase B titres
    • if available (repeat 10–14 days later if first test not confirmatory)
    • The false negatives rate is 20-30%, reduce false negative with anti-DNase B titre 
    • False positives can result from liver disease and tuberculosis.

• Rapid antigen test
  • Turnaround time of 1-3 hours
  • sensitivity of 86% and specificity of 96%
  •  cost only $5-$10 compared with $30 for throat cultures
  • But unfortunately DOES NOT attract an MBS rebate.
• Bloods – ESR/CRP, FBC (chronic anaemia)
• ECG (heart block)
• U/A (GN for CTD)
• Imaging:
  • Serial CXR (cardiomegaly due to carditis, monitor course)
  • Echo (acute carditis, valvular regurgitation/stenosis, chamber size and function, pericardial effusion)
• Tests for alternative diagnoses, depending on clinical features
  • Repeated blood cultures, if possible endocarditis
  • Joint aspirate (microscopy and culture) for possible septic arthritis
  • Copper, ceruloplasmin, antinuclear antibody, drug screen for choreiform movements
  • Serology and autoimmune markers for arboviral, autoimmune or reactive arthritis

Immediate Management

Goals: Goals: symptomatic relief + eradication of GAS + prophylaxis

Symptomatic Relief

• Aspirin:
  • Children: 80-100 mg/kg/day
  • Adults: 4-8 g/day
  • Continue until symptoms resolve and ESR/CRP levels normalize.
• Antihistamines: For rash relief.
• Cardiac Involvement:
  • Severe carditis may cause cardiomegaly, congestive heart failure (CHF), or 3rd-degree heart block.
  • Management follows acute pulmonary oedema (APO) protocols (e.g., Frusemide, ACE inhibitors).
  • Prednisone: 2 mg/kg/day orally for 1-2 weeks, then tapered over 2 weeks for severe carditis.
  • Valve Surgery: Considered for heart failure due to refractory regurgitant lesions, with valve repair preferred over replacement.
• Chorea (if present):
  • Usually self-limiting and may not require treatment.
  • If needed:
    • Carbamazepine: 7-20 mg/kg/day, typically given three times daily (tds).
    • Valproic Acid: 15-20 mg/kg/day, may be increased to 30 mg/kg/day, given tds.

Antibiotic Therapy

• All Cases:
  • IM Benzathine Penicillin G (preferred):
    • ≥20 kg: 900 mg (1,200,000 units)
    • <20 kg: 450 mg (600,000 units)
  • Alternative (if IM injection not possible): Oral Phenoxymethylpenicillin (Penicillin V) for 10 days:
    • Children: 250 mg twice daily (bd)
    • Adolescents and Adults: 500 mg bd
  • Penicillin Allergy: Use Erythromycin:
    • Children: 20 mg/kg up to 800 mg bd for 10 days
    • Adults: 800 mg bd for 10 days

Management for Family Contacts

• Throat swab for GAS culture.
• If positive, initiate appropriate penicillin therapy.

General Care Recommendations

• Rest: Strict bed rest is often unnecessary; include planned rest periods.
• Age-Appropriate Activities: Encourage suitable activities based on age.
• Family Involvement: Educate family members on the condition and engage them in care.
• School Notification: Inform teachers about the patient’s care requirements.

Discharge Planning

• Plan for transfer to a primary care facility with scheduled follow-up appointments.

Drugs used for rheumatic fever

Indication	Drug options listed in order of preference	Comment
Eradication of inciting streptococcal infection	1. Benzathine benzylpenicillin G 1,200,000 units child <20 kg: 600,000 units ≥20 kg: 1,200,000 units intramuscularly, single dose OR	Streptococcal infection may not be evident by the time acute rheumatic fever manifests (e.g. cultures often negative), but eradication therapy for possible persisting streptococci is recommended.Intramuscular penicillin is preferred as streptococcal eradication therapy due to better adherence and its subsequent ongoing use in secondary prophylaxis.
	2. Phenoxymethylpenicillin 500 mg child: 15 mg/kg up to500 mg orally, every 12 hours for 10 days OR
	3. For patients with penicillin hypersensitivity (non-severe): cefalexin 1 g child: 25 mg/kg up to 1 g orally, every12 hours for 10 days OR	Between 3% and 30% of group A streptococcus isolates internationally are resistant to macrolide antibiotics (e.g., azithromycin).
	4. For patients with immediate penicillin hypersensitivity: azithromycin 500 mg (child: 12 mg/kg up to 500 mg) orally, daily for 5 days
Initial analgesia while awaiting diagnostic confirmation:mild to moderate painsevere pain	Paracetamol 1000 mg (in children: 15 mg/kg) orally, every four hours as needed up to a maximum of 60 mg/kg/day or 4000 mg/day	Initial analgesia is preferred during diagnostic uncertainty to avoid the masking effect that anti-inflammatory use can have on migratory joint symptoms, fever and concentrations of inflammatory markers.
	Tramadol immediate-release 50–100 mg (in children: 1–2 mg/kg) orally, every four hours as needed up to a maximum of 400 mg/day	Tramadol (or codeine) is usually avoided in children<12 years of age due to variable metabolism. Use only when strong analgesia is essential and cautious monitoring is available.
Symptomatic management of arthritis/arthralgia after confirmation of acute rheumatic fever diagnosis	1. Naproxen immediate-release 250–500 mg (in children: 10–20 mg/kg/day) orally twice daily, up to a maximum of 1250 mg dailyOR	Naproxen may be safer than aspirin and convenient due to twice-daily dosing and the availability of oral suspension.Ibuprofen is well tolerated and readily available, but there are less data and experience with its use for acute rheumatic fever than those associated with naproxen.The dose of NSAIDs needed for acute rheumatic fever is generally higher than the dose recommended for other conditions; therefore, it may be appropriate to start at the higher dose range.Due to the rare possibility of Reye’s syndrome in children, aspirin may need to be discontinuedduring intercurrent acute viral illness; thus, influenza vaccination is strongly recommended to reduce the likelihood of this case.
	2. Ibuprofen 200–400 mg (in children: 5–10 mg/kg) orally three times daily, up to a maximum of 2400 mg dailyOR
	3. Aspirin 50–60 mg/kg/day orally, in 4–5 divided doses in adults and children. Dose can be escalated up to a maximum of 80–100 mg/kg/day in 4–5 divided doses
Symptomatic management of moderate to severe chorea	1. Carbamazepine 3.5–10 mg/kg per dose orally twice daily	Treatment of Sydenham chorea should be considered if movements interfere substantially with normal activities.
	2. Sodium valproate 7.5–10 mg/kg per dose orally twice daily
Symptomatic management of very severe chorea or chorea paralytica	In addition to an anticonvulsant drug, consider adding a corticosteroid:· Prednisolone 1–2 mg/kg up to a maximum of 80 mg orally once daily
Symptomatic management of carditis	Paediatric dosing:· Furosemide (frusemide) 1–2 mg/kg orally as a single dose, then 0.5–1 mg/kg (to a maximum of 6 mg/kg) orally every 6–24 hours· Spironolactone 1–3 mg/kg (initially) up to 100 mg orally in 1–3 divided doses daily. Round dose to a multiple of6.25 mg (a quarter of a 25-mg tablet)	Treatment of heart failure may be required for severe, acute carditis. Seek advice from a specialist cardiologist.
	· Enalapril 0.1 mg/kg orally in 1 or 2 divided doses daily, increased gradually over 2 weeks to a maximum of1 mg/kg orally in 1 or 2 divided doses daily. Alternative ACE inhibitors: captopril, lisinopril	The choice of ACE inhibitor will vary depending on the clinical situation. Seek advice from a specialist cardiologist.
	Adult dosing:· Furosemide (frusemide) 20–40 mg orally or intravenously as a single dose followed by 20–40 mg orally or intravenously every 8–12 hours. Ongoing dose adjustment is based on clinical progression and renal function.· Spironolactone may be added for patients with limited or no response to loop diuretic; 12.5–200 mg orally once daily with dose escalation based on clinical and electrolyte responses.· Nitrate therapy may be added for patients with limited or no response to diuretic therapy and systolic blood pressure greater than 90 mmHg. Intravenous or topical glyceryl trinitrate may be used.ACE inhibitor therapy with perindopril or ramipril is recommended in patients with moderate or severe left ventricular systolic dysfunction, unless contraindicated.	The management of acute carditis follows the same principles as those for the management of acute heart failure. This table provides a guide to the initial management of acute heart failure due to acute carditis in adults. Seeking advice from a specialist cardiologist early is strongly recommended.
	Digoxin 15 micrograms/kg orally as a single dose, then5 micrograms/kg after 6 hours, then 3–5 micrograms/kg (in adults: 125–250 micrograms) orally, daily	Digoxin is rarely used for the treatment of acute carditis. Seek advice from a specialist cardiologist.
Disease-modifying (immunomodulatory) treatments	Prednisolone 1–2 mg/kg up to a maximum of 80 mg orally, once daily	Considered for use in selected cases of severe carditis, despite meta-analyses in which the overall benefit was not evident.
Secondary prophylaxis	1. Benzathine benzylpenicillin G by deep intramuscular injection 1,200,000 units (≥20 kg) or 600,000 units (<20 kg) *OR2. Phenoxymethylpenicillin (penicillin V) 250 mg orally twice dailyOR3. For patients with penicillin hypersensitivity (non-severe) or immediate penicillin hypersensitivity:erythromycin 250 mg orally twice daily	Every 28 days. †Every 21 days for selected groups. ‡Intramuscular penicillin is preferred due to greater effectiveness in head-to-head trial and better adherence.

NSAID non-steroidal anti-inflammatory drug

* For children weighing less than 10 kg, a dose of 600,000 units is still generally recommended, but seek paediatric advice for careful planning of the secondary prophylaxis regimen.

† Patients on 28-day regimens can be recalled from 21 days to help ensure that injections are given by day 28.

‡ Benzathine benzylpenicillin G given every 21 days may be considered for:

  • patients who have breakthrough acute rheumatic fever despite complete adherence to a 28-day regimen

  • patients who are at a high risk of adverse consequences if acute rheumatic fever occurs (have severe rheumatic heart disease or a history of heart valve surgery).

Source: modified from reference 2 with permission

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Long-Term Management

Complications of ARF

• Rheumatic Heart Disease (RHD):
  • Progression: Carditis worsens over time with cumulative valve damage, typically developing 10-20 years after initial ARF.
  • Valve Involvement:
    • Mitral Valve: Most commonly affected.
    • Aortic Valve: Less common.
    • Mitral Stenosis (MS): A key chronic finding requiring surgical intervention in some cases (e.g., balloon valvuloplasty).
  • Complications:
    • Embolization: Due to mural thrombi.
    • Infective Endocarditis: Increased risk on deformed valves.
  • Chronic RHD Manifestations: Murmurs, hypertrophy, dilation, heart failure, arrhythmias.

Education and Long-Term Secondary Prophylaxis

• Understanding ARF: Educate patients and families about ARF’s causes and the importance of early treatment for sore throats and skin infections.
• Risk Awareness: Inform family members of their increased risk compared to the general population.
• Long-Term Prophylaxis: Emphasize the importance of adherence to secondary prophylaxis to prevent ARF recurrences and associated cardiac damage.
  • Benzathine Penicillin G: 1.2 million units IM every 3-4 weeks.
  • Oral Phenoxymethylpenicillin: If IM route is unsuitable.
• Duration of Prophylaxis: Minimum 10 years after the last ARF episode or until age 21, whichever is longer.

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Primary Prophylaxis

• Target Population: Individuals at risk of ARF following a GAS infection.
• Treatment of Streptococcal Pharyngitis:
  • Timing: Initiate treatment within 9 days of symptom onset.
  • Benzathine Penicillin G (IM Single Dose):
    • Children:
      • ≥20 kg: 900 mg
      • 15-20 kg: 675 mg
      • 10-15 kg: 450 mg
      • 6-10 kg: 337.5 mg
      • 3-6 kg: 225 mg
    • Adults: 900 mg
  • Alternative (Oral Phenoxymethylpenicillin):
    • Children: 10 mg/kg up to 500 mg, bd for 10 days.
    • Adults: 500 mg, bd for 10 days.
  • Penicillin Allergy: Use Erythromycin Ethyl Succinate.

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Secondary Prophylaxis for Rheumatic Fever

Objective: To prevent recurrent episodes of Acute Rheumatic Fever (ARF) and further cardiac damage in individuals with a history of rheumatic fever, particularly those who have developed Rheumatic Heart Disease (RHD).

Background: ARF is an immune-mediated response triggered by a previous infection with Group A Streptococcus (GAS). It is not a persistent infection itself. A new GAS infection can provoke another immune response, leading to recurrent ARF episodes. Without prophylaxis, recurrence is common, particularly in the years following the initial episode, which can lead to progressive and cumulative heart valve damage, resulting in RHD.

Target Population: Patients with a history of ARF, particularly those with established RHD.

Goal: Prevent recurrent ARF episodes that can cause progressive and often irreversible damage to heart valves, especially the mitral and aortic valves, resulting in RHD or worsening existing valvular disease.

Risk Considerations:

• Recurrences are most frequent within the first five years following the initial ARF episode, particularly among children and adolescents.
• Unlike diseases with effective vaccines, ARF prevention relies on antibiotic prophylaxis to prevent initial or recurrent GAS infections.

Initiation of Prophylaxis:

• GAS pharyngitis is the main trigger for ARF, and long-acting antibiotics provide sustained protection against streptococcal infections.
• Regular prophylaxis has been shown to significantly reduce the risk of ARF recurrence and subsequent valve damage.
• Prophylaxis should begin immediately following the resolution of the initial ARF episode.
• Children and adolescents are at the highest risk for repeated ARF attacks. By the early 20s or 30s, the immune response to GAS often decreases, reducing the risk of recurrence. However, individuals with severe valvular disease may require longer or lifelong prophylaxis.

First-Line Treatment

• Benzathine Penicillin G (IM injection):
  • Dosage: 1.2 million units administered intramuscularly every month.
  • Special Cases: For patients with severe carditis or those who have undergone valvular surgery, consider administration every 3 weeks.

Second-Line Treatment (Alternative)

• Phenoxymethylpenicillin (Penicillin V):
  • Dosage: 250 mg taken twice daily (BD).
  • Used if intramuscular (IM) injections are not possible or if the patient refuses IM administration.

Note: Adherence to secondary prophylaxis must be carefully monitored to ensure effective prevention of recurrent ARF.

Duration of Secondary Prophylaxis

• All individuals with a history of ARF or RHD:
  • Minimum of 10 years after the most recent ARF episode or until the patient reaches 21 years of age (whichever is longer).
• Extended Duration Considerations: For patients with severe RHD or those at higher risk, prolonged prophylaxis may be recommended beyond these guidelines based on individual risk assessment.

Improving Adherence to Secondary Prophylaxis for ARF and RHD

Challenges:

• Sociological factors significantly impact the efficacy of secondary prophylaxis.
• In remote Aboriginal and Torres Strait Islander communities, limited availability and the acceptability of health services present major barriers.
• Personal factors, such as refusal of injections, pain associated with injections, or a lack of knowledge and understanding of ARF and RHD, play a comparatively smaller role.

Strategies to Improve Adherence:

• Personalized Care: Creating a sense of belonging to the clinic can enhance patient adherence.
• Recall Systems: Establish systems that extend beyond community boundaries to ensure consistent follow-up and care.
• Organizational Approaches: Use patient registers for effective tracking and management of care.
• Routine Review and Care Planning: Conduct regular reviews and establish comprehensive care plans tailored to patient needs.
• Recall and Reminder Systems: Implement automated or manual systems to remind patients of upcoming appointments and injections.
• Dedicated Local Staff: Assign local staff members specifically to coordinate secondary prophylaxis efforts.
• Aboriginal Health Workers: Leverage their expertise, community knowledge, and language skills to enhance care delivery.
• Staff Training and Awareness: Enhance healthcare staff’s knowledge and skills related to ARF and RHD diagnosis and management.
• Minimizing Staff Turnover: Reduce turnover to ensure continuity and stability of care.
• Reducing Injection Pain: Adopt measures to minimize the discomfort associated with injections, improving patient compliance.

By addressing these factors, the effectiveness of secondary prophylaxis programs can be significantly improved, particularly in high-risk populations such as ATSI communities.

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Procedures Requiring Endocarditis Prophylaxis for Patients with Rheumatic Heart Disease (RHD):

Dental, Oral, and Respiratory Tract Procedures:

1. Dental extractions
2. Periodontal procedures
3. Dental implant placement
4. Gingival surgery
5. Initial placement of orthodontic appliances
6. Surgical drainage of dental abscess
7. Maxillary or mandibular osteotomies
8. Endodontic surgery and instrumentation
9. Placement of orthodontic bands
10. Intraligamentary local anaesthetic injections
11. Surgical repair or fixation of a fractured jaw
12. Tonsillectomy/adenoidectomy
13. Rigid bronchoscopy
14. Surgery involving the bronchial mucosa
15. Sclerotherapy of oesophageal varices
16. Dilatation of oesophageal stricture

Genitourinary and gastrointestinal procedures

1. Surgery of the intestinal mucosa or biliary tract (except for endoscopy, biopsy and percutaneous endoscopic gastrostomy)
2. Endoscopic retrograde cholangiography
3. Prostate surgery
4. Cystoscopy and urethral dilatation
5. Vaginal delivery in the presence of infection, or prolonged labour or prolonged rupture of membranes
6. Surgical procedures of the genitourinary tract in the presence of infection (e.g. urethral catheterisation, uterine dilatation and curettage, abortion, sterilisation and placement or removal of intrauterine contraceptive devices)

ADDITIONAL INFORMATION

Symptoms in detail:

Arthritis

• Several large joints in quick succession: knees, ankles, elbows, wrists
  • Legs first
  • Each for short time (< 1 week) but overlap 🡪 migratory
• Earliest symptomatic manifestation
• Symptoms – pain > inflammation
• Investigations
  • Xray – may show effusion
  • Joint aspirate – sterile inflammatory fluid
• Treatment
  • Aspirin or NSAIDs

Carditis

• Pancarditis – pericardium, epicardium, myocardium, endocardium
• Symptoms:
  • Chest discomfort, pleuritic chest pain, pericardial rub
  • Exam –
    • Murmurs – new/changing, MR most common
• If severe valve damage + myocarditis causing myocardial dysfunction
  • Can 🡪 heart failure
  • Most life-treathening clinical syndrome
  • Treat as APO
• Investigations
  • ECG – heart block (all degrees)
  • CXR – cardiomegaly
  • Echo – acute carditis, valvular regurgitation/stenosis, chamber size and function, pericardial effusion
  • Antimyosin scintigraphy – 80% sensitive for carditis (any cause)

Chorea

• Sydneyham, chorea minor, “St Vitus dance”
• Abrupt, purposeless, nonrhythmic involuntary movements + muscular weakness + emotional disturbance

• Clinical signs: 
  • More marked on one side
  • Cease during sleep
  • Weakness revealed when ask to squeeze emaminer’s hands 🡪 milking sign
  • Emotional – inappropriate, crying, restless, psychosis
  • Neurological – no sensory changes, diffuse hypotonia
  • Can take to up 8 months to manifest

Subcutaneous nodules

• Firm and painless, overlying skin not attached or inflamed
• < 2cm wide
• Over bony surface or prominence or near tendons
•  Present for < 4 weeks
• Usually only in patients with carditis

Erythema Marginatum/annulare

• Evanscent non-pruritic rash
• Pink/faintly red
• Trunk, sometimes proximal limbs
• Extends outwards while skin in centre return to normal