CONSENSUS STATEMENT
Poststreptococcal arthritis
P OSTSTREPTOCOCCAL ARTHRITIS (PSA) IS A POORLY UN -
derstood clinical syndrome that has generated much
controversy and for which clear diagnostic criteria and thera-
peutic recommendations are lacking. Like acute rheumatic fe-
ver (ARF), PSA is a reactive arthritis characterized by a
pharyngeal streptococcal infection, a symptom-free interval
and subsequent aseptic inflammation of one or more joints.
However, unlike ARF, the risk of other postinfectious complica-
tions is not clear. Interest in various aspects of group A strep-
tococcal infection has been rekindled recently as a result of
changing trends in both its suppurative and postinfectious
complications. With an apparent increase in the incidence of
ARF in certain parts of North America (1,2), the diagnostic cri-
teria have been revisited and refined (Table 1) (3). Further-
more, a change in streptococcal virulence has been
suggested by clusters of cases of ARF and by the emergence
of cases of severe invasive disease (4,5). In this climate of en-
hanced interest in streptococcal infections and their complica-
tions, it is timely to examine some of the diagnostic and
therapeutic controversies surrounding PSA.
POSTSTREPTOCOCCAL ARTHRITIS
The term ‘poststreptococcal arthritis’ was introduced in
1959 (6) to denote patients who had arthritis following pharyn-
geal infection with beta-hemolytic streptococcus, but in whom
other major criteria of ARF were absent (Table 1). The term is
used inconsistently in the literature to indicate various con-
stellations of signs and symptoms. In its original sense, PSA is
used to designate the condition of patients in whom prolonged
polyarthritis that does not respond to acetylsalicylic acid (ASA)
occurs approximately 10 days following upper respiratory
tract infection with group A beta-hemolytic streptococcus
when other signs of ARF are absent. In most reported series of
PSA, however, cutaneous or cardiac disease was present in a
significant proportion of patients. None of the 12 patients re-
ported by Goldsmith and Long (7) had carditis. However, one
had a pericardial effusion and four had urticarial or maculo-
papular rashes; an additional four had cutaneous hyperesthe-
sia. Of the 16 children with PSA reported by Gibbas and
Broussard (8), seven had pericarditis and at least three had
valvular disease. Six of seven patients reported by Emery et
al (9) had evidence of carditis. Neither chorea nor nodules
Correspondence and reprints: Infectious Diseases and
Immunization Committee, Canadian Paediatric Society, 401 Smyth
Road, Ottawa, Ontario K1H 8L1. Telephone (613) 737-2728, Fax
(613) 737-2794
CAN J INFECT DIS VOL 6 NO 3 MAY/JUNE 1995
TABLE 1
The modified Jones criteria, 1992
Major criteria
Carditis
Polyarthritis
Erythema marginatum
Subcutaneous nodules
Chorea
Minor criteria
Clinical findings
Arthralgia
Fever
Laboratory findings
Elevated acute-phase reactants
Erythrocyte sedimentation rate
C-reactive protein
Prolonged PR interval
Supporting evidence of antecedent group A streptococcal in-
fection
Positive throat culture or antigen test
Elevated or rising streptococcal antibody titre
The diagnosis of acute rheumatic fever is supported by evidence of preceding
streptococcal infection, as well as by the presence of either two major criteria or
one major and two minor criteria. (Adapted from reference 3)
was reported in any of these series. It is likely that some pa-
tients identified in the literature as having PSA actually suf-
fered from ARF.
It is not certain whether PSA represents a mild or early form
of ARF, or whether it is an entirely separate entity. In support of
the first possibility is that outbreaks of PSA and ARF occur at
the same time (10) and that at least some children with PSA
have been shown to develop full-blown ARF (11). In support of
the possibility that PSA and ARF are distinct disorders are the
differences in interval between infection and disease, the dif-
ferences in response to ASA and the differences in pattern of
affected joints (Table 2). In children with ARF (12), but not in
those with streptococcal pharyngitis or poststreptococcal
glomerulonephritis (13), there is a very strong association
with the B-cell alloantigen D8/17 (12). The occurrence of
other manifestations of ARF, such as isolated chorea, may be
associated with this antigen (14), and the same association
may be found in children with isolated PSA (15); such an asso-
ciation would support the view that PSA is a limited form of ARF.
It may be most accurate to regard PSA as a kind of ARF (with or
133
Consensus statement

TABLE 2 sis established is clearly ARF and appropriate therapy should

Comparison of acute rheumatic fever and poststrepto- be initiated. Since PSA is so rare, the risk of nonsuppurative
coccal arthritis complications associated with first or recurrent attacks re-
ARF PSA mains to be clearly established; expert advice should be
Epidemiology sought in the diagnosis and therapy of patients thought to
Age (years) 5-15 3-15 have either condition. Furthermore, patients initially identified
Sex ratio (male:female) 1:1 1:1 as having PSA might, after prospective evaluation, prove to
Chorea 0-30% 0 have ARF.
Erythema marginatum 0-13% 0 In the absence of a clear approach to prophylaxis and
Carditis 30-90% * treatment of children with PSA and because the condition of
Nodules 0-8% 0 patients initially thought to suffer from PSA is frequently asso-
Arthritis ciated with cardiac disease, it seems prudent to treat such pa-
Flitting +++ – tients as if they have ARF until more precise information is
Persistent – +++ available. One reasonable approach is to initiate antibiotic
Large joints +++ +++ prophylaxis as recommended for ARF. If, after three months,
Small joints ± +++ there is no evidence of either carditis or chorea, antibiotic pro-
Salicylate response +++ inconsistent phylaxis may be discontinued.
Deforming – ±
Erosive – PITFALLS IN THE DIAGNOSIS OF
Preceding streptococcal +++ +++
STREPTOCOCCAL INFECTION
infection
Other diseases that should be considered in the differen-
Approximate interval 21 10
tial diagnosis of patients thought to have PSA include juvenile
between infection and
disease (days) rheumatoid arthritis, spondyloarthropathies, acute strepto-
Association with B-cell +++ * coccal arthritis and reactive arthritis secondary to enteric
alloantigen D8/17 (Yersinia, Campylobacter, Salmonella species) or genitouri-
* Not known; ARF Acute rheumatic fever; PSA Poststreptococcal arthritis nary tract (Chlamydia species) infections. Although clinical
criteria help to differentiate these disorders, the diagnosis of
PSA hinges on the correct identification of patients with pre-
ceding group A streptococcal infection. The accurate diagno-
without cardiac involvement) in which the arthritis differs sig- sis of streptococcal infection is difficult for a number of
nificantly from the arthritis of typical ARF in the following ways reasons, including the following:
(Table 2): • A substantial number of healthy children are chronically
• PSA begins sooner (approximately 10 days) after colonized with group A streptococci; therefore, recovery
streptococcal infection than does ARF (approximately 21 of the bacterium from the throat does not necessarily
days); indicate acute infection.
• PSA is cumulative, rather than flitting, and persistent, • Group A streptococci may no longer be present in the
rather than fleeting; throat when nonsuppurative complications such as PSA
• PSA responds poorly to ASA, in contrast to the rapid, appear, as a result of either effective antimicrobial
dramatic response that is usually seen in the case of therapy or spontaneous clearance.
ARF. • Serological evidence of recent streptococcal infection
Substantial controversy exists regarding the optimal ther- (documented by a two dilution or greater rise in titres of
apy for patients with PSA; this stems from concerns about the antistreptolysin-O [ASO] or anti-DNase B, or by means
risk of other nonsuppurative complications upon recurrence. of other streptococcal antibody tests) is not present
In particular, some authorities believe that the risk of carditis immediately after an acute streptococcal infection and
mandates the use of prophylactic antimicrobial therapy, such may require weeks or months to appear.
as that recommended for patients with a history of ARF, to pre-
vent group A streptococcal reinfection (10). In one study (11) QUESTIONS THAT NEED TO BE ANSWERED
of 12 patients with PSA who did not receive antimicrobial pro- Clinical criteria for establishing the diagnosis of PSA have
phylaxis, five experienced recurrent disease. Of the five, two been suggested, but a number of questions regarding the op-
had arthritis, two had arthralgia and one experienced classic timum diagnostic and therapeutic strategies remain unan-
ARF with carditis, migratory arthritis and subcutaneous nod- swered. Issues that need to be addressed include the
ules. In a classic description of scarlatinal arthritis, Crea and following:
Mortimer (16) suggested that patients may experience an ill- • Is PSA a clinical entity distinct from ARF or are they
ness that does not satisfy the diagnostic criteria for ARF but manifestations of the same disease?
puts them at the same risk of acquiring cardiac valvular dis- • To what extent are patients with PSA at increased risk of
ease. If a patient thought to have PSA develops evidence of developing other manifestations of ARF, particularly
carditis, whereby the Jones criteria are satisfied, the diagno- carditis?

134 CAN J INFECT DIS VOL 6 NO 3 MAY/JUNE 1995


• Do patients with PSA require antibiotic prophylaxis to
prevent recurrences (analogous to patients with ARF)?
• What are the present incidences of PSA and ARF?
RECOMMENDATIONS
1. To establish the diagnosis of PSA, antecedent infection
with group A streptococcus must be established, either
by culture of the bacterium from the throat or by
demonstration of an elevation or a fourfold rise in ASO or
anti-DNase B titre.
2. If group A streptococcus is recovered from the throat,
specific antibacterial therapy should be given.
3. The diagnosis of PSA should be made only after careful
historical and clinical evaluation for nonsuppurative
streptococcal complications or other causes of
polyarthritis.
4. In view of reports that patients thought to have PSA
experience recurrences and may be at increased risk of
developing carditis, antibiotic prophylaxis, as
recommended for patients with ARF, may be considered
on a short term basis. If, after further evaluation, there is
no evidence of carditis or chorea, prophylaxis may be
discontinued.
5. A Canadian registry of patients with PSA should be set up
to assess the prevalence, risks and outcome and to
establish an optimum therapy of this rare disease.
REFERENCES
1. Congeni B, Rizzo C, Congeni J, et al. Outbreak of acute
rheumatic fever in northeast Ohio. J Pediatr 1987;111:176-9.
2. Veasy LG, Wiedmeier SE, Orsmond GS, et al. Resurgence of
acute rheumatic fever in the intermountain area of the United
States. N Engl J Med 1987;316:421-7.
3. Special Writing Group of the Committee on Rheumatic Fever,
Endocarditis, and Kawasaki Disease of the Council on
Cardiovascular Disease in the Young of the American Heart
Association. Guidelines for the diagnosis of rheumatic fever.
Jones criteria, 1992 update. JAMA 1992;268:2069-73.
4. Stevens DL, Tanner MH, Winship J, et al. Severe group A
streptococcal infections associated with a toxic-shock-like
syndrome and scarlet fever toxin A. N Engl J Med 1989;321:1-7.
5. Demers B, Simor AE, Vellend H, et al. Severe invasive group A
streptococcal infections in Ontario, Canada: 1987-1991. Clin
Infect Dis 1993;16:792-800.
6. Friedberg CK. Rheumatic fever in the adult: criteria and
implications. Circulation 1959;19:161-4.
7. Goldsmith DP, Long SS. Poststreptococcal disease of childhood:
a changing syndrome. Arthritis Rheum 1982;25(Suppl):S18.
(Abst)
8. Gibbas DL, Broussard DA. Post streptococcal reactive
polyarthritis (PSRA): rheumatic fever or not? Arthritis
Rheum1986;29(Suppl):S92.
9. Emery H, Wagner-Weiner L, Magilavy D. Resurgence of
childhood post-streptococcal rheumatic syndromes. Arthritis
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Rheum 1987;30(Suppl):S80.
10. Fink CW. The role of the streptococcus in post-streptococcal
reactive arthritis and childhood polyarteritis nodosa. J
Rheumatol Suppl 1991;29:14-20.
11. De Cunto CL, Giannini EH, Fink CW, et al. Prognosis of children
with post-streptococcal reactive arthritis. Pediatr Infect Dis J
1988;7:683-6.
12. Khanna AK, Buskirk DR, Williams RC Jr, et al. Presence of
non-HLA B-cell antigen in rheumatic fever patients and their
families as defined by a monoclonal antibody. J Clin Invest
1989;83:1710-6.
13. Gibofsky A, Khanna A, Suh E, et al. The genetics of rheumatic
fever: Relationship to streptococcal infection and autoimmune
disease. J Rheumatol Suppl 1991;30:1-5.
14. Feldman BM, Zabriskie JB, Silverman ED, et al. Diagnostic use
of B-cell alloantigen D8/17 in rheumatic chorea. J Pediatr
1993;123:84-6.
15. Zemel LS, Hakonarson H, Diana DJ, et al. Poststreptococcal
reactive arthritis (PSRA): a clinical and immunogenetic analysis.
J Rheumatol 1992;19
(Suppl 33):120.
16. Crea MA, Mortimer EA Jr. The nature of scarlatinal arthritis.
Pediatrics 1959;23:879-84.
Infectious Diseases and Immunization Committee
Members: Drs William Albritton, Provincial Laboratory of Public
Health, Edmonton, Alberta; François Boucher, Département de
pédiatrie, Centre hospitalier de l’Université Laval, Ste-Foy, Québec;
Gilles Delage, directeur scientifique, Laboratoire de santé publique
du Québec, Sainte-Anne-de-Bellevue, Québec; Elizabeth Ford-
Jones, Division of Infectious Diseases, The Hospital for Sick Chil-
dren, Toronto, Ontario; Michael Hall
(director responsible), Janeway Child Health Centre,
St John’s, Newfoundland; Susan King, Division of Infectious
Diseases, The Hospital for Sick Children, Toronto, Ontario; Noni
MacDonald (chair), Department of Pediatrics, Children’s
Hospital of Eastern Ontario, Ottawa, Ontario; David P Speert (prin-
cipal co-author), Division of Infectious and
Immunological Diseases, Department of Pediatrics,
University of British Columbia, Vancouver, British Columbia.
Ex-officio member: Dr Frank R Friesen (chair, Committee on
Committees), Manitoba Clinic, Winnipeg, Manitoba.
Consultants: Drs Ronald Gold, Division of Infectious Diseases,
The Hospital for Sick Children, Toronto, Ontario; Victor
Marchessault, Department of Pediatrics, Children’s Hospital of
Eastern Ontario, Ottawa, Ontario; Ross E Petty (principal
co-author), Division of Rheumatology, Department of
Pediatrics, University of British Columbia, Vancouver,
British Columbia.
Liaisons: Public Health, Dr Jacqueline Carlson, Disease Control
and Epidemiology, Public Health Branch, Toronto, Ontario; Ameri-
can Academy of Pediatrics, Dr Larry Pickering, Eastern Virginia
Medical School, Children’s Hospital of the King’s Daughters, Nor-
folk, Virginia; Centre for Vaccine Evaluation,
Dr David Scheifele, Division of Infectious Diseases, Research Cen-
tre, BC’s Children’s Hospital, Vancouver, British Columbia;
Epidemiology, Dr John Waters, Alberta Health,
Communicable Disease Control, Edmonton, Alberta
135