Austrian syndrome

Austrian syndrome is a medical condition first described by Robert Austrian in 1957. The classical triad consists of pneumonia, endocarditis, and meningitis, all caused by Streptococcus pneumoniae. It is associated with alcoholism, due to the presence of hyposplenia (reduced splenic functioning), and can be seen in males between 40 and 60 years old.[1]

Signs and symptoms

Signs of Austrian syndrome usually begin in Caucasian males over 40. Austrian syndrome triad include pneumonia, endocarditis, and meningitis. A study showed that middle aged men with alcohol issues made up the majority of patients with Austrian Syndrome.[2]

Symptoms of pneumonia include cough producing mucus, shortness of breath, chest pains during cough.

Symptoms of endocarditis include fever, muscle aches, swollen feet, shortness of breath.

Symptoms of meningitis include headache, confusion, sudden fever, sensitivity to light.

Mechanism/Pathophysiology

Osler's triad also known as Austrian Syndrome was first introduced in the 19th century. Streptococcus Pneumoniae is what causes an Osler’s triad of meningitis, pneumonia and endocarditis. The portal of entry for this triad is said to be the lungs which was then followed by meningitis and endocarditis. The major risk factors are heavy alcohol use, old age, splenectomy, immunosuppression, etc. Endocarditis typically involves the aortic valve. The native aortic valve is the most frequent site of vegetation for streptococcus pneumoniae, and is considered the most common cardiac lesion.[3]

Causes

The main cause of Osler’s triad (Austrian Syndrome) is streptococcus pneumoniae which is usually associated with heavy alcohol use. Named Osler's triad because it's an association of pneumonia, meningitis, and endocarditis.

Drinking excessive amounts of alcohol puts an individual at risk. It was determined that alcoholism completed the tetrad of associated conditions.

The patient who was the oldest recorded patient with Austrian syndrome had a history of health concerns such as hypertension, diabetes malleus, etc. increased her risk for Austrian syndrome.[4][3]

Diagnosis

Early diagnosis of Austrian syndrome is recommended. The disease is usually diagnosed later in an individual’s life, because it mostly affects older Caucasian men. To determine if an individual has Austrian Syndrome, a series of tests are performed. Bacterial cultivation was the main method in determining the diagnosis of Streptococcus pneumoniae.

Rapid Diagnostic Test is when a liquid sample of ear or nasal discharge is collected. In terms of streptococcus pneumoniae, it also used to confirm the causative bacterium.[5]

X-Ray Imaging of the chest is performed to determine lung inflammation and aortic regurgitation.

Electrocardiogram is used to measure the sound waves of the heart. A physical exam is performed on lung and heart cavities and a spinal tap is also performed to collect cerebrospinal fluid.

Prevention/Treatment

Less consumption of alcohol or no consumption at all are effective ways to decrease the chances of getting Austrian Syndrome. 14% of patients don't have risk factors.[4] Since Austrian Syndrome consists of meningitis, pneumonia and endocarditis, there are separate treatments for each. Streptococcus pneumoniae and endocarditis are usually treated with penicillin, which has said to be the most effective but sources have said that some strains are resistant to penicillin. High doses of penicillin have no effect on pneumonia. Before penicillin was used for treatment, pneumococcus was a cause of a number of endocarditis cases.[3] Also, for endocarditis, a valve replacement would be performed to avoid cardiogenic shock. For meningitis, intravenous antibiotics are used. Earlier studies suggest that dexamethasone improved the outcome of adults with pneumococcal meningitis.[3] In a specific case study, a patient who had symptoms of a fever and headache was treated with cetotaxime, ampicllin, and dexamethasone and had to undergo an emergency valve surgery since the EKG showed mitral vegatation.[6]

Researchers say a balanced diet and a healthy diet is beneficial for many aspects in an individual’s life.

Prognosis

In the 19th century, the mortality rate of Austrian Syndrome was about 75% and has decreased to approximately 32%.[4] The mortality percentage is increased in immunocompromised individuals.The long term effects are eventually death. The older you are, Austrian syndrome consists of pneumonia, endocarditis, and meningitis which all have high mortality rates.

Epidemiology

Austrian syndrome is a rare disease and it affects mostly males in their 50-60’s with a long history of alcohol abuse.

Research

Since Austrian Syndrome is extremely rare with less than 60 cases reported, there hasn't been lots of research done on the disease. But there have been multiple case studies that discuss certain treatments, preventions, diagnosis, depending on the individual. In a case study, a patient who had absolutely no history of alcohol abuse presented symptoms of the triad, such as low fever, myalgia, cough, breathlessness. He had abnormal pupils which indicated injury to the brain. A CT scan was performed and CSF analysis results showed 78 cells mm/3^3, a low glucose concentration and positive latex agglutination. The patient was treated with Cenftriaxone which caused the aortic valve to swell up so Vancomycin and Carbapenam was used next in the treatment process and the patient responded well and was able to recover at home with intensive therapy.[7] So antibiotics are used as tools of treatment.

There have been no further reports on the Austrian syndrome, because there is a lack of knowledge on the subject. Some researchers refer to it as a 'disease of the past'.

References

  1. Chest. 2009 Nov;136(5 Suppl):e30.
  2. Atkinson, Kate; Augustine, Daniel Xavier; Easaw, Jacob (2009-09-15). "Austrian syndrome: a case report and review of the literature". BMJ Case Reports. 2009: bcr0320091724. doi:10.1136/bcr.03.2009.1724. ISSN 1757-790X. PMC 3028387. PMID 21918664.
  3. Rakočević, Rastko; Shapouran, Sara; Pergament, Kathleen M (2019). "Austrian Syndrome – A Devastating Osler's Triad: Case Report and Literature Review". Cureus. 11 (4): e4486. doi:10.7759/cureus.4486. ISSN 2168-8184. PMC 6581326. PMID 31259104.
  4. Rodríguez Nogué, M.; Gómez Arraiz, I.; Ara Martín, G.; Fraj Valle, M. M.; Gómez Peligros, A. (2019). "[Austrian syndrome: A rare manifestation of invasive pneumococcal disease. A case report and bibliographic review]". Revista Espanola de Quimioterapia: Publicacion Oficial de la Sociedad Espanola de Quimioterapia. 32 (2): 98–113. ISSN 1988-9518. PMC 6441982. PMID 30880376.
  5. "Streptococcus pneumoniae: diagnosis and treatment". Otsuka Pharmaceutical Co., Ltd. Retrieved 2020-12-11.
  6. Munoz, P (1999). "Austrian Syndrome Caused by Highly Penicillin-Resistant Streptococcus pneumoniae". Clinical Infectious Diseases. 29 (6): 1591–2. doi:10.1086/313542. PMID 10585831.
  7. Midon, Márcio Estevão; Goldoni, Fernando; Souza, Sylvian Greicy Rocha; Miyasato, Jan Norimitsu Schiemann (2011). "Austrian Syndrome: case report". Arquivos Brasileiros de Cardiologia. 97 (3): e50–52. ISSN 1678-4170. PMID 22030703.
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