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Author: Samit M. Shah, University of Illinois
College of Medicine at Urbana-Champaign, Class of 2012
Introduction: Rheumatic heart disease is
characterized by the priming of the immune system by a group A
streptococcal infection leading to host autoimmunity. This
antecedent infection may be asymptomatic and omitted from a
presenting history. This is a case of acute rheumatic fever
masquerading as inflammatory polyarthritis.
Case Presentation: A 41-year-old male initially
presented to an outside hospital with painful joints, fevers, and
chills for three weeks. Further investigation revealed
leukocytosis, elevated ESR, and an elevated rheumatoid factor (RF).
Shortly thereafter, he presented to our hospital with disabling
medium and large joint pain and morning stiffness lasting for four
hours. He was apparently bitten by a tick recently but denied rash,
facial paralysis, or systemic illness. Examination revealed
tachycardia and inflammatory polyarthritis with sparing of the
small joints. Laboratory evaluation showed an elevated ESR and CRP
while serology for anti-nuclear antibodies, HIV, and Lyme Disease
were negative. Echocardiogram revealed normal left ventricular (LV)
systolic function, insignificant mitral valve regurgitation (MR),
and no evidence of vegetations. Indomethacin provided symptomatic
relief and the patient was discharged with a preliminary diagnosis
of rheumatoid arthritis. Four days later he returned with sudden
onset dyspnea at rest and productive cough of one day duration.
Exam revealed tachycardia and bibasilar crackles. Laboratory
evaluation showed elevated BNP (727 pg/ml) and D-dimer (1961 ng/ml)
while imaging of the chest showed bilateral lower lobe infiltrates
with pleural effusions. Bronchoscopy, cultures for acid fast
bacteria, fungi, pneumocystis, fungal serology, and cytology
studies were unremarkable. Repeat serology was negative for RF and
anti-CCP antibodies. His symptoms improved with diuresis and he was
discharged. Two weeks later he reported worsening polyarthralgia
and exam revealed hypotension, tachycardia, a new third heart
sound, and a maculopapular rash on his abdomen. Titers for
anti-streptolysin O (ASO) and anti-DNAase-B antibodies were found
to be significantly elevated. A repeat echocardiogram showed global
LV systolic dysfunction and moderate MR, significantly worse from
the previous study, confirming the diagnosis of acute rheumatic
Discussion: 19 million people worldwide suffer
from rheumatic heart disease but 95% of cases occur in developing
countries. Recent epidemiological data shows a resurgence of
rheumatic heart disease in some parts of the United States after
nearly disappearing for the past three decades. This case
demonstrates the importance of considering this forgotten disease
in the context of its changing epidemiology. Diagnosis is based on
multiple clinical features combined with evidence of a group A
streptococcal infection. The major Jones diagnostic criteria
include migratory polyarthritis and carditis with acute valvular
dysfunction, subcutaneous nodules, erythema marginatum, and
Syndenham's chorea. Minor criteria include fever, arthralgia,
elevated ESR or CRP, prolonged PR interval, elevated ASO or DNAase
titers, and prior rheumatic fever.
September Issue of IMpact