Chapter 23 – Rheumatologic Emergencies

Chapter 23 Rheumatologic Emergencies

Joyce Hui-Yuen and Noé Romo

Acute Rheumatic Fever

Acute rheumatic fever (ARF) is a non-suppurative sequela of Group A Streptococcus (GAS) pharyngitis that can affect the heart, joints, subcutaneous tissue, and central nervous system. ARF occurs primarily between 5 and 15 years of age and is rare in children under three. The estimated incidence is 1–3% in patients who receive inadequate treatment for GAS pharyngitis and it is very uncommon when appropriate treatment is given.

Clinical Presentation and Diagnosis

ARF typically presents 2–4 weeks after an acute infection with GAS, which can sometimes be asymptomatic. There is no single test for the condition and the diagnosis is based on clinical features embodied in the Jones Criteria. Evidence of a recent GAS pharyngitis and the presence of two major, or one major and two minor, features are required for diagnosis. The criteria are described in Table 23.1.

Table 23.1 Jones Criteria and the diagnosis of acute rheumatic fever

Evidence of GAS1
Throat culture and rapid strep test are positive in 25% of cases
Streptococcal Ab (ASLO, anti-DNase B, streptokinase, antihyaluronidase) is more sensitive
Major criteria2
(J)oints (polyarthritis) Pain, erythema and swelling of large joints that “migrates”
Persists for up to one week in each joint
Pain is disproportional (↑↑ severe) to the physical findings
(O) Carditis Endocarditis is most common; also peri-, epi-, and myocarditis
Chest pain, friction rub, aortic and mitral regurgitation murmurs
(N)odules (subcutaneous) Firm, symmetric, 1–2 cm, painless nodules
Located over bony prominences or tendons, commonly the elbow
(E)rythema marginatum Non-pruritic, evanescent, macular serpiginous rash with raised erythematous borders
Central part of each lesion returns to normal as the rash spreads or resolves
Typically on the trunk and inner thighs, upper arms
(S)yndenham chorea Abrupt, purposeless, involuntary movements
Associated with weakness and emotional lability
Minor criteria3
Arthralgia Significant pain in a single joint with minimal findings of inflammation
Cannot use as a minor criteria if polyarthritis used as a major
Prolonged P–R interval
Elevated acute phase reactants: ESR, CRP, WBC

1 Required for all criteria.

2 Need two major alone, or one major and two minor.

3 Need two minor along with one major.

Recent advances in echocardiography (ECHO) have made the detection of subclinical carditis (valvular damage detected only on ECHO) readily available. However, the use of ECHO for the diagnosis of ARF in a patient who has no clinically audible murmur is still controversial. Auscultatory findings remain the basis for the diagnosis of carditis.

Joint involvement is both a major and minor criterion. Migratory arthritis is defined as arthritis in an affected joint that resolves before another joint is affected. Since migratory polyarthritis (major criterion) often improves dramatically with the use of NSAIDS, corticosteroids, or aspirin, only arthralgia (minor criterion) may be evident. Therefore, delay symptomatic treatment if a definite diagnosis of ARF has not been established. In patients presenting with arthritis, other etiologies must be ruled out (see pp. 700703), especially septic arthritis.

Post-streptococcal reactive arthritis (PSRA) occurs in patients who have arthritis and evidence of a preceding GAS infection, but do not fulfill the Jones Criteria. Symptoms can occur as early as ten days after GAS infection, but can last two months (longer than ARF). Although it is a known entity, it is not clear whether it is truly separate from ARF. In contrast to ARF, the arthritis typically does not respond dramatically to aspirin or NSAIDs, and patients do not present with carditis. Nonetheless, give ARF prophylaxis for one year after diagnosis of PSRA.

The World Health Organization’s revised Jones Criteria include several exceptions to the criteria presented above for the following circumstances:

  • Recurrent attack of rheumatic fever (RF): requires two minor features and evidence of recent GAS infection in a patient with established rheumatic heart disease.

  • Rheumatic chorea or insidious onset of rheumatic carditis: evidence of GAS infection is not required.

  • Chronic valvular lesions of rheumatic heart disease: no other criteria are required.

ED Management

Since the diagnosis is based on the Jones Criteria, obtain a CBC, CRP, ESR, chest radiograph, ECG, throat culture, rapid Streptococcus swab, ASLO, and anti-DNAse B antibodies. The throat culture and rapid strep tests may be negative as the GAS pharyngitis can resolve, even without antibiotic treatment. When the ASLO and anti-DNAse B are performed simultaneously, however, evidence of a preceding GAS infection can be found in >90% of patients.

Once the diagnosis is certain, treat migratory polyarthritis with aspirin 50–75 mg/kg/day div tid or qid or naproxen 15–20 mg/kg/day div bid for 2–4 weeks. Arthritis in ARF is exquisitely sensitive to NSAIDS and resolves shortly after institution of therapy.

Treat mild to moderate carditis with high-dose aspirin 80–100 mg/kg/day div qid for 4–8 weeks, depending on response, then gradually discontinue. Give prednisone (2 mg/kg/day) for severe carditis and congestive heart failure, but first consult a pediatric cardiologist.

Chorea resolves spontaneously over 2–3 weeks and most patients do not need to be medicated. Treat severe symptoms with haloperidol, but consult a pediatric neurologist prior to instituting therapy.

Once the diagnosis is established, the eradication of any existing streptococcal infection or carriage is necessary (Table 23.2). Secondary prophylaxis is critical for preventing disease recurrences (Table 23.3). Carditis is the single most important prognostic factor for ARF, but only valvulitis leads to permanent damage. Therefore, patient age and the presence of valvulitis determines the length of prophylaxis.

Table 23.2 Primary prevention of rheumatic fever

Antibiotic1 Dose
Penicillin G benzathine ≥27 kg: 1,200,000 units IM × 1
< 27 kg: 600,000 units IM × 1
Penicillin VK 250 mg tid or qid or 500 mg bid PO × 10 days
Amoxicillin 50 mg/kg daily PO × 10 days
First-generation cephalosporin 20 mg/kg/day PO × 10 days (dosing frequency varies)
Erythromycin ethylsuccinate 40 mg/kg/day PO div bid or tid × 10 days

1 Trimethoprim, sulfonamides, and tetracyclines are not effective for eradicating GAS infections

Table 23.3 Secondary prophylaxis of rheumatic fever

Antibiotic Dose
Penicillin G benzathine IM 1.2 million units IM q 4 weeks
Penicillin V 250 mg bid PO
Sulfadiazine or sulfisoxazole <27 kg: 0.5 g/day PO
≥27 kg: 1 g/day PO
Penicillin-allergic Erythromycin 250 mg bid PO


  • Pediatric rheumatologist within one week

  • Primary care monthly for antibiotic prophylaxis; other pediatric specialists (cardiologist, neurologist) based upon symptomatology

Indications for Admission

  • Suspected acute rheumatic fever

  • Recurrent episode of rheumatic fever


Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation. 2015;131(20):18061818.

Reyhan I, Goldberg BR, Gottlieb BS. Common presentations of pediatric rheumatologic diseases: a generalist’s guide. Curr Opin Pediatr. 2013;25(3):388396.

Uziel Y, Perl L, Barash J, Hashkes PJ. Post-streptococcal reactive arthritis in children: a distinct entity from acute rheumatic fever. Pediatr Rheumatol Online J. 2011;9(1):32.

Van Driel ML, De Sutter AI, Habraken H, Thorning S, Christiaens T. Different antibiotic treatments for group A streptococcal pharyngitis. Cochrane Database Syst Rev. 2016;9:CD004406.

Yanagawa B, Butany J, Verma S. Update on rheumatic heart disease. Curr Opin Cardiol. 2016;31:162168.


Arthritis results from synovial inflammation due to infectious and noninfectious causes (Table 23.4). In contrast, arthralgia is pain or tenderness without swelling.

Table 23.4 Common etiologies of joint pain in children

Diagnosis Differentiating features
Acute rheumatic fever Pain is out of proportion to physical exam findings
Classic migratory polyarthritis involves knees, ankles, elbows, and wrists
Gonococcal arthritis Subacute onset
Initially, pauciarticular and often migratory; can be monoarticular
Adolescents may have tenosynovitis and papular or vesiculopustular rash
Lyme disease Acute onset of swelling and tenderness without warmth or erythema, most often involving the knee
Can be extremely painful
Mostly monoarticular, sometimes migratory, rarely polyarticular
Can persist for weeks to months and recur
Post-infectious Subacute onset 2–3 weeks after an illness (URI or gastroenteritis) Duration <6 weeks
Mono or asymmetric oligoarthritis commonly in knees and ankles
Moderate pain, minimal swelling, no erythema, low-grade or no fever
Septic arthritis Most common at 1–3 years of age in one large joint (knee, hip)
Abrupt onset of painful, persistent and progressive arthritis
Erythema, warmth, swelling, pain, and decreased range of motion (especially infants)
Absence of joint effusion on ultrasound has a high negative predictive value for septic arthritis
Toxic synovitis Occurs in as many as 3% of 2–10-year-olds
Good response to NSAIDS
Can last for 2 weeks, but resolves without complications
Dermatomyositis Frequently involves the small joints of the hands
IBD Acute onset of a persistent oligoarthritis involving large joints, including hips
JIA Arthritis lasting >6 weeks in a child <16 years old
Typically less painful than swelling suggests
Worse in the morning, with associated stiffness
Kawasaki disease Subacute onset polyarthritis involving the small joints of the hands and feet
Serum sickness Arthralgia/arthritis of the knees, ankles, shoulders, wrists, spine, and temporomandibular joint
Associated with myalgias, urticaria, angioedema, and hematuria
SLE Subacute onset of symmetric polyarthritis involving peripheral joints of the hands or feet
Large joints may be involved during disease exacerbation
Despite severe pain, arthritis is nondestructive and may be persistent or intermittent
Leukemia/ Gradual onset of a persistent mono-, poly-, or migratory arthritis
bone tumors Pain out of proportion to physical findings and may be worse at night, can awaken child from sleep
Can be associated with fever, weight loss, and anemia
Benign growing Pain is mild, generally localized to long bones
pains Pain can wake child from sleep but easily resolved with massage and reassurance
RSD Pain severely out of proportion with examination
Affected joint/limb in preferred position of comfort
Often the affected limb is cool to touch.
Fracture Point tenderness, can be associated with some peri-articular swelling
Hypermobility No objective arthritis, usually only arthralgias
Can affect upper and/or lower extremities
Traumatic arthritis Acute or chronic onset of intermittent pain that is worse with activity
Typically one joint is involved (elbow, shoulder, knee, hip)
May have an associated fracture, ligamentous injury, bursitis, or tenovitis

Sep 22, 2020 | Posted by in EMERGENCY MEDICINE | Comments Off on Chapter 23 – Rheumatologic Emergencies
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