Case Presentation: A 70-year-old man with a history of human immunodeficiency virus (CD4 count 500) and alcoholism presented with 5 days of fever and cough, associated with headache and worsening altered mental status. He was febrile to 100F, tachycardic, hypotensive, extremely irritable, photophobic, and with positive nuchal rigidity. Laboratory findings showed a leukocytosis to 25. Two sets of blood cultures grew Streptococcus pneumoniae. Chest radiograph revealed left upper lobe opacity. Lumbar puncture was attempted but unsuccessful. Empiric antibiotics were initiated for treatment of pneumonia and meningitis. The patient clinically improved with return of his mental status to baseline. Repeat blood cultures showed clearance of bacteria. However, due to persistent leukocytosis, echocardiogram was performed on hospital day 7 and revealed a small aortic valve vegetation with severe aortic regurgitation. Valvular surgery was recommended and urged but the patient adamantly refused. He subsequently developed evidence of heart failure with lower extremity edema and shortness of breath. Repeat echocardiogram several days later demonstrated enlargement of the aortic valve vegetation, severe aortic regurgitation, and new biventricular hypokinesis. The patient ultimately passed away from sequelae of acute decompensated HF several days later.
Discussion: Austrian syndrome is an uncommon and deadly triad of pneumonia, meningitis, and endocarditis secondary to Streptococcus pneumoniae that was first described in 1957 by the American infectious disease physician, Robert Austrian. While Streptococcus pneumoniae is still the leading cause of community acquired pneumonia and meningitis, it accounts for less than 3% of native valve endocarditis since the advent of penicillins. It typically affects older men with a history of alcoholism and/or immunocompromised state due to hyposplenism, making this patient population more susceptible to disseminated disease. Infection begins with pneumonia in over 80% of cases, followed by development of meningitis and endocarditis. Endocarditis involves the aortic valve in 75% of cases, frequently with rapid valvular destruction leading to HF requiring urgent valve replacement. Mortality in patients receiving medical treatment with antibiotics alone is reported to be as high as 60% compared to 32% in those treated with a combined medical-surgical approach. Due to its rarity, diagnosis is often delayed and can have fatal consequences. This case serves as a reminder to clinicians that there should be a low threshold to look for a vegetation suggestive of endocarditis in immunocompromised patients being treated for Streptococcus pneumoniae pneumonia and/or meningitis.
Conclusions: Austrian syndrome is a rare and deadly clinical entity caused by Streptococcus pneumoniae that manifests as a triad of pneumonia, meningitis, and endocarditis. It has a predilection for elderly men with a history of alcoholism and/or immunocompromised state. Streptococcus pneumoniae tends to seed the aortic valve, leading to aortic insufficiency. Without early recognition and appropriate intervention, aortic insufficiency can lead to rapidly progressive heart failure resulting in death. Clinicians should maintain a low threshold for an echocardiogram to look for endocarditis in immunocompromised patients found with Streptococcus pneumoniae pneumonia and/or meningitis.