Austrian syndrome
Austrian syndrome, also known as Osler's triad, is a medical condition that was named after Robert Austrian in 1957. The presentation of the condition consists of pneumonia, endocarditis, and meningitis, all caused by Streptococcus pneumoniae. It is associated with alcoholism due to hyposplenism (reduced splenic functioning) and can be seen in males between the ages of 40–60 years old.[1] Robert Austrian was not the first one to describe the condition, but Richard Heschl (around 1860s) or William Osler were not able to link the signs to the bacteria because microbiology was not yet developed.
Epidemiology
Signs of Austrian syndrome usually begin in Caucasian[2] males over 40. A study shows that middle-aged men with alcohol issues make up most patients with Austrian syndrome.[3]
Causes
The leading cause of Osler's triad (Austrian syndrome) is Streptococcus pneumoniae, which is usually associated with heavy alcohol use. The condition was named Osler's triad because of its associations with pneumonia,[4] meningitis, and endocarditis. Excessive consumption of alcohol would put the user at risk. It was determined that alcoholism completed the tetrad of associated conditions.
The oldest recorded patient with Austrian syndrome had a history of health concerns such as hypertension, and diabetes mellitus which increased her risk of Austrian syndrome.[5][6]
Mechanism/pathophysiology
Osler's triad, also known as Austrian syndrome, was first introduced in the 19th century. Streptococcus pneumoniae is the cause of Osler's triad of meningitis,[7] pneumonia, and endocarditis. The portal of entry for this triad is said to be the lungs, followed by meningitis and endocarditis. Significant risk factors are heavy alcohol consumption, old age, splenectomy, immunosuppression, etc. Endocarditis typically involves the aortic valve. The native aortic valve is the most frequent vegetation site for Streptococcus pneumoniae and is considered the most common cardiac lesion.[6] In fact it is a historic misnomer because Richard Heschl described the signs before Osler did, but he described this in german, so it got lost in the literature and the name was Osler's triad.
Signs and symptoms
The presentation of Austrian syndrome includes symptoms from all three of the triad: pneumonia, endocarditis, and meningitis.[3] Cough-producing mucus, shortness of breath, and chest pains during cough are associated with pneumonia. Fever, muscle aches, swollen feet, and shortness of breath are some symptoms of endocarditis,[8] and symptoms of meningitis like headache, confusion, sudden fever, and sensitivity to light.
Diagnosis
Early diagnosis of Austrian syndrome is beneficial. The disease is usually diagnosed later in an individual's life because it mostly affects older Caucasian men. Multiple tests are performed to determine if an individual has Austrian syndrome. Bacterial cultivation is the main method in diagnosing Streptococcus pneumoniae.
Rapid diagnostic test is when a liquid sample of the ear or nasal discharge is collected. In terms of Streptococcus pneumoniae, it is also used to confirm the causative bacterium.[9]
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.
Treatment
Since Austrian syndrome consists of meningitis, pneumonia and endocarditis, there are separate treatments for each. Pneumonia and endocarditis are usually treated with beta-lactam therapy penicillin, which has been said to be the most effective but sources have said that some bacterial strains are resistant to penicillin. High doses of penicillin do not affect pneumonia. Before penicillin was used for treatment, pneumococcus was a cause of several endocarditis cases.[6] Also, for endocarditis, a valve replacement would be performed to avoid cardiogenic shock. For meningitis, intravenous antibiotics are used. Earlier studies suggest that dexamethasone[10] improved the outcome of adults with pneumococcal meningitis.[6] In a specific case study, a patient who had symptoms of a fever and headache was treated with cefotaxime, ampicillin,[11] and dexamethasone and had to undergo an emergency valve surgery since the EKG showed mitral vegetation.[12]
Prognosis
In the 19th century, the mortality rate of Austrian syndrome was about 75%; it has since decreased to approximately 32%.[5] The mortality percentage is higher in immunocompromised individuals. Austrian syndrome's symptoms, including pneumonia, endocarditis, and meningitis, all have high mortality rates.[13]
Prevention
Decreased alcohol consumption is an effective way to lower the chances of developing Austrian syndrome. The incidence rate has reduced since the introduction of beta-lactam therapy in the early 1940s. Additionally, the introduction of the pneumococcal vaccination in 1977 further decreased the incidence of infection. However, 14% of patients don't have risk factors.[5]
Research
Due to the rarity of the syndrome, with fewer than 60 cases reported, there has not been much research on the disease. But there have been multiple case studies that discuss certain treatments, preventions, diagnoses, depending on the individual. In a case study, an individual 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 showed 78 cells/mm3 a low glucose concentration, and positive latex agglutination.[14] The individual was treated with Ceftriaxone which caused the aortic valve to swell up so Vancomycin and Carbapenam were used next in the treatment process and the individual responded well and was able to recover at home with intensive therapy.[15] So antibiotics are used as tools of treatment. In another case of Austrian syndrome, a 76-year old woman with diabetes mellitus,[16] hypertension[17] and chronic cervical and lumbar degenerative disease presented with the disease.[6]
References
- Georgiadou, Sarah P.; Manoulakas, Efstratios; Makaritsis, Konstantinos P.; Dalekos, George N. (30 May 2018). "A Chronic Alcoholic Man with High Fever, Neck Rigidity and Loss of Consciousness: Remember the Austrian Syndrome a Commonly Unrecognised Invasive Pneumococcus Triad". BMJ Case Reports. 2018: bcr–2018–225010. doi:10.1136/bcr-2018-225010. ISSN 1757-790X. PMC 5976089. PMID 29848535.
- "Definition of CAUCASIAN".
- Atkinson, Kate; Augustine, Daniel Xavier; Easaw, Jacob (15 September 2009). "Austrian syndrome: a case report and review of the literature". BMJ Case Reports. 2009: bcr0320091724. doi:10.1136/bcr.03.2009.1724. PMC 3028387. PMID 21918664.
- "Everything You Need to Know About Pneumonia". 20 October 2015.
- Nogué, Marta Rodríguez; Arraiz, Ignacio Gómez; Martín, Gema Ara; Valle, Mª Mar Fraj; Peligros, Antonio Gómez (April 2019). "Síndrome de Austrian: Una rara manifestación de la enfermedad neumocócica invasiva. Presentación de un caso y revisión bibliográfica" [Austrian syndrome: A rare manifestation of invasive pneumococcal disease. A case report and bibliographic review]. Revista Española de Quimioterapia (in Spanish). 32 (2): 98–113. PMC 6441982. PMID 30880376.
- Rakočević, Rastko; Shapouran, Sara; Pergament, Kathleen M (17 April 2019). "Austrian Syndrome – A Devastating Osler's Triad: Case Report and Literature Review". Cureus. 11 (4): e4486. doi:10.7759/cureus.4486. PMC 6581326. PMID 31259104.
- "Meningitis: Symptoms, Causes, Types, Treatment, Risks, & More". 30 May 2018.
- "Endocarditis: Risk Factors, Symptoms, and Diagnosis". 7 August 2012.
- "Streptococcus pneumoniae: diagnosis and treatment". Otsuka Pharmaceutical Co., Ltd. Retrieved 11 December 2020.
- "Dexamethasone Uses, Dosage & Side Effects".
- "Ampicillin Uses, Side Effects & Warnings".
- Munoz, P.; Sainz, J.; Rodriguez-Creixems, M.; Santos, J.; Alcala, L.; Bouza, E. (1 December 1999). "Austrian Syndrome Caused by Highly Penicillin-Resistant Streptococcus pneumoniae". Clinical Infectious Diseases. 29 (6): 1591–1592. doi:10.1086/313542. PMID 10585831.
- Rodríguez Nogué, M.; Gómez Arraiz, I.; Ara Martín, G.; Fraj Valle, M. M.; Gómez Peligros, A. (April 2019). [Austrian "syndrome: A rare manifestation of invasive pneumococcal disease. A case report and bibliographic review"]. Revista Espanola de Quimioterapia. 32 (2): 98–113. ISSN 1988-9518. PMC 6441982. PMID 30880376.
{{cite journal}}
: Check|url=
value (help) - "Agglutination - definition, reactions and applications". 16 April 2021.
- Midon, Márcio Estevão; Goldoni, Fernando; Souza, Sylvian Greicy Rocha; Miyasato, Jan Norimitsu Schiemann (September 2011). "Austrian Syndrome: case report". Arquivos Brasileiros de Cardiologia. 97 (3): e50–e52. doi:10.1590/S0066-782X2011001200017 (inactive 31 July 2022). PMID 22030703.
{{cite journal}}
: CS1 maint: DOI inactive as of July 2022 (link) - "Diabetes mellitus | Definition, Types, Symptoms, & Treatment".
- "Facts About Hypertension". cdc.gov. Retrieved 2 August 2022.
Further reading
- Schandl, Christian (March 2020). "Austrian syndrome". Clinical Medicine. 20 (2): e1.1–e1. doi:10.7861/clinmed.Let.20.2.1. PMC 7081815. PMID 32188672.
- Austrian, Robert (1957). "The Syndrome of Pneumococcal Endocarditis, Meningitis and Rupture of the Aortic Valve". Transactions of the American Clinical and Climatological Association. 68: 40–50. PMC 2248949. PMID 13486606.