Review Question - QID 217153

This patient with a recent history of pharyngitis, Sydenham chorea (uncontrollable writhing), migratory arthritis, pericarditis, subcutaneous nodules, erythema marginatum, fever, and arthralgias most likely has acute rheumatic fever.

Acute rheumatic fever is an immune-mediated complication of untreated Streptococcus pyogenes pharyngitis. The major clinical features of acute rheumatic fever are the JONES criteria: Joint (migratory arthritis), ♥️ (myocarditis, pericarditis), Nodules (subcutaneous), Erythema marginatum, and Sydenham chorea. Other minor clinical features include fever, arthralgias, elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), and prolonged PR interval. The treatment of acute rheumatic fever is long-term therapy with long-acting intramuscular penicillin G to prevent recurrent disease and worsening of rheumatic heart disease. It is crucial to prevent acute rheumatic fever by treating patients with Streptococcus pyogenes pharyngitis with a course of oral penicillin.

Gewitz et al. review a revision of the JONES criteria by the American Heart Association. They discuss how Doppler echocardiography is a modality that can be used to help evaluate for this disease. They recommend using the revised criteria in order help standardize diagnosis of rheumatic fever.

Figure/Illustration A shows the torso of a patient with erythema marginatum. This is classically described as an evanescent, nonpruritic, non-tender rash with a pale-pink center (yellow arrow) surrounded by a slightly raised red outline (blue arrow) that can either be annular (purple circle) or have an irregular elongated shape (orange circle).

Incorrect Answers:
Answer 1: Chagas disease is caused by Trypanosoma cruzi, a protozoan prevalent in South and Central America. Acute infection with T. cruzi is usually asymptomatic, but may rarely present with nonspecific symptoms and swelling at the site of inoculation. Severe acute disease occurs in less than 1% of patients but may manifest with acute myocarditis, pericardial effusion, pericarditis, and meningoencephalitis. Although this patient has a pericardial friction rub, the findings of erythema marginatum, Sydenham chorea, and recent pharyngitis are more suggestive of rheumatic fever.

Answer 2: Coxsackie B virus is a cardiotropic virus that is the most common cause of infectious myocarditis and pericarditis. Infection with this pathogen can cause signs of heart failure after a viral prodrome. Treatment includes interferons, pleconaril, and acyclovir.

Answer 3: Lyme disease is caused by the spirochete Borrelia burgdorferi and is transmitted by the Ixodes tick. Lyme disease can present acutely with erythema migrans, an annular rash with a bulls-eye appearance. Late complications of Lyme disease can include complete heart block and arthritis. Treatment includes doxycycline and amoxicillin

Answer 4: Parvovirus B19 infection in children classically causes the “slapped cheek” rash of erythema infectiosum. In addition, it may cause arthritis, arthralgias, and myocarditis. Treatment is supportive as the virus usually resolves by itself. In rare cases, it can lead to aplastic anemia.

Bullet Summary:
Patients with acute rheumatic fever can present with migratory arthritis, carditis, subcutaneous nodules, erythema marginatum, Sydenham chorea, fever, arthralgias, prolonged PR intervals, and arthralgias in the setting of previous Streptococcus pyogenes pharyngitis.