Winning Abstracts from the 2011 Medical Student Abstract Competition: A Zebra in the Prairie: Inflammatory Polyarthritis and Sudden Dyspnea in Central Illinois
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 fever.
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.