Review Question - QID 221245

This patient with Sydenham chorea, migratory arthritis, pericarditis, subcutaneous nodules, erythema marginatum, fever, and arthralgias most likely has acute rheumatic fever. This disease is associated with previous Streptococcus pyogenes pharyngitis.

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 or 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 antibiotics.

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 to help standardize the diagnosis of rheumatic fever.

Figure/Illustration A is a clinical photograph showing an evanescent rash with a pale-pink center surrounded by a slightly raised red outline (blue circles). These findings are consistent with the erythema marginatum rash seen in acute rheumatic fever.

Incorrect Answers:
Answer 1: Antibodies against snRNPs describe anti-Smith antibodies, which are a specific antibody for systemic lupus erythematosus (SLE). Although patients with SLE can present with joint pain, rash, and carditis, the rash is a characteristic malar rash across the face, not a pink rash on the trunk.

Answer 2: Positive rheumatoid factor is usually associated with rheumatoid arthritis, a form of inflammatory arthritis. Patients often complain of morning stiffness that improves throughout the day. Symmetrical involvement of the small joints of the hand is often seen. Carditis and erythema marginatum are not typical presentations of rheumatoid arthritis.

Answer 3: Previous tick-borne illness would be consistent with Lyme disease, which 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 bullseye appearance. Late complications of Lyme disease can include complete heart block and arthritis.

Answer 5: Previous viral illnesses, such as a parvovirus B19 infection, can also result in a rash and arthralgia. The rash is a characteristic “slapped-cheek rash,” and the arthralgia is similar to that of rheumatoid arthritis in that it presents with morning stiffness and symmetric involvement of the small joints of the hands.

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 infection.