Austrian syndrome: Report of two new cases and literature review

doi:10.1016/j.ijcard.2005.02.045

International Journal of Cardiology

Volume 108, Issue 2, 4 April 2006, Pages 273-275

https://doi.org/10.1016/j.ijcard.2005.02.045 Get rights and content

Abstract

In April 2004, Rammeloo et al. reported a child-aortic-valve endocarditis following pneumococcal meningitis. Classically, the association of pneumococcal pneumonia along with meningitis and infectious endocarditis (IE) is known as Austrian syndrome. We assessed the incidence, clinical manifestations and follow-up of patients diagnosed with Austrian syndrome in Lugo (Northwest Spain) between 1987 and 2002. A computerized MEDLINE search was also performed for literature review. During the study period 165 Lugo patients met criteria for definite IE. Two of the 3 patients with definite streptococcus pneumoniae IE also met definitions for Austrian syndrome. The incidence of this syndrome in Lugo patients with definite IE was 1.2%. In the literature review 48 cases, including our two patients, were found. Complete clinical information was only available on 16 patients. Alcoholism constituted the most common predisposing factor. Aortic valve involvement and high grade of valve regurgitation leading to cardiac failure and the need of cardiac surgery during the admission were common in these patients. This review confirms that Austrian syndrome is a rare but severe disease. Early recognition of these patients is required to avoid ominous complications.

Introduction

In April 2004, Rammeloo et al. reported a child-aortic-valve endocarditis following pneumococcal meningitis [1]. Classically, the association of pneumococcal pneumonia along with meningitis and infectious endocarditis (IE) is known as Austrian syndrome [2]. We assessed the incidence, clinical manifestations, and follow-up of patients diagnosed with Austrian syndrome in Lugo (Northwest Spain) between 1987 and 2002. A computerized MEDLINE search was performed for literature review.

Section snippets

Case report

During the study period 165 patients met criteria for definite IE. Two of the 3 patients with streptococcus pneumoniae IE met definitions for Austrian syndrome. The incidence of this syndrome in Lugo patients with definite IE was 1.2%.

Discussion

In the review we found 48 cases of Austrian syndrome with complete information on 16 [3], [4] (Table 1). Most were alcoholic men with acute clinical presentation. All patients had native valve IE. Twelve (75%) suffered aortic valve involvement with high grade of valve regurgitation in 8. Nine (56%) experienced cardiac complications including congestive heart failure. Ten (63%) required valve replacement (8 aortic and 2 mitral) during the active phase of the endocarditis. Nine had non-cardiac

References (6)

L. Rammeloo et al.
Austrian syndrome in a child-aortic valve endocarditis following pneumococcal meningitis
Int J Cardiol
(2004)
R. Austrian
Pneumoccocal endocarditis, meningitis and rupture of the aortic valve
Arch Intern Med
(1957)
J. Lindberg et al.
Pneumococcal endocarditis is not just a disease of the past: an analysis of 16 cases diagnosed in Denmark 1986–1997
Scand J Infect Dis
(1998)

There are more references available in the full text version of this article.

Cited by (22)

Acromioclavicular joint arthritis as the first manifestation of Austrian syndrome
2020, Journal of Infection and Chemotherapy
Citation Excerpt :
Risk factors for invasive pneumococcal infections include extremes of age, chronic alcoholism, chronic glucocorticoid therapy, diabetes mellitus, chronic obstructive pulmonary disease, current smoking, congestive heart failure, chronic renal insufficiency, chronic liver disease, and immunosuppressive conditions such as human immunodeficiency virus [2]. In a review, Austrian syndrome developed more frequently in middle-aged men with chronic alcoholism, debilitation, or immune dysfunction [11]. The patient had some of these risk factors for invasive pneumococcal infections and Austrian syndrome.
Austrian syndrome is a rare condition caused by invasive Streptococcus pneumoniae, comprising a triad of pneumococcal meningitis, endocarditis, and pneumonia. Herein, we report a 59-year-old male patient who presented with fever and tenderness of the right shoulder. Although the initial diagnosis was acromioclavicular joint septic arthritis, the present case showed a reduced level of consciousness, pulmonary infiltrates, cerebral infarcts, and destruction of the mitral valve. This case suggests that acromioclavicular joint arthritis could be an initial presentation of pneumococcal infection inclusive of Austrian syndrome, especially in patients with some risk factors of invasive pneumococcal infections, such as chronic alcoholism.
Austrian Syndrome
2010, Medicina Intensiva
Austrian's triad complicated by suppurative pericarditis and cardiac tamponade: a case report and review of the literature
2009, International Journal of Infectious Diseases
Citation Excerpt :
Aronin et al. mentioned that 116 or 59% of the 197 reviewed cases of pneumococcal endocarditis had concurrent meningitis and that 29 of those had Osler's triad.4 A recent report and review by Gonzalez-Juanatey et al. identified two cases of Austrian's syndrome over a 15-year period at their institution in Lugo, Spain and reviewed an additional 16 well-defined cases from the literature.8 No cases of associated pericarditis or cardiac tamponade were identified.
Austrian syndrome (pneumococcal pneumonia, meningitis, and endocarditis): A case report
2008, American Journal of the Medical Sciences
Citation Excerpt :
Although seen infrequently today, unusual manifestations of pneumococcal infection such as those Austrian reported still occur. In the literature review, we found 54 cases of Austrian syndrome with complete information on 20.1,4,8–13 All patients had native valve infective endocarditis.
This report describes a case of Austrian syndrome (pneumonia, meningitis, and endocarditis, as a result of Streptococcus pneumoniae infection). A computerized medline search was performed for review of literature. In the review of literature, 54 cases including our case were found. Complete clinical and microbiological information was available only for 20 cases. Most invasive pneumococcal infections occurred in debilitated middle aged men with chronic alcoholism. Native aortic valve insufficiency was the commonest cause of cardiac failure among these patients, requiring valve replacement. Austrian syndrome is seen infrequently in this antibiotic era but is still associated with a poor outcome. Hence early recognition and appropriate medical or combined medical–surgical treatment needs to be considered promptly.
Austrian syndrome: A clinical rarity
2008, International Journal of Cardiology
Citation Excerpt :
We consider this data are extensible to Austrian syndrome. In a recent review, 48 cases of classic Austrian syndrome are referred with complete information on 16 [6]. Most were alcoholic men with acute clinical presentation in forth of fifth decade.
We present a case of classical Austrian syndrome in a 55-year-old man with a history of alcoholism. The rapid destruction in aortic valve cusps caused perforations with severe aortic insufficiency, acute pulmonary edema, and determined an urgent aortic valve substitution with good postoperative evolution. The review of the literature suggests cardiac surgery in this syndrome to improve the poor survival with conservative treatment. We recommend early surgery in these cases because of the fast impairment of the patient and valvular tissue destruction detected in intraoperatory examination.
Austrian's syndrome: The first described case of pneumococcal meningitis pneumonia and endocarditis in an injecting drug user
2007, European Journal of Internal Medicine
Citation Excerpt :
In the immunocompetent, smoking remains the most significant risk factor for IPD. The triad of meningitis, pneumonia and endocarditis and its association with S. pneumoniae has only been described in a small numbers of patients, with a reported incidence of 1.2% amongst infective endocarditis patients [1]. This is the first reported case in an IDU.
We describe the first reported case of Austrian's syndrome in an injecting drug user (IDU). The triad of endocarditis, meningitis and pneumonia caused by invasive pneumococcal disease (IPD) is most commonly associated with excess alcohol. Injecting drug use is a recognised risk factor for IPD, whose prevalence and resistance continue to rise. We propose that injecting drug use is associated with Austrian's syndrome and that it should at least be considered in ‘at risk’ groups presenting with IPD. Furthermore, IDU presenting with IPD, meningitis and pneumonia should be considered for echocardiography.

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Letter to the EditorAustrian syndrome: Report of two new cases and literature review

Abstract

Introduction

Section snippets

Case report

Discussion

Int J Cardiol

Pneumoccocal endocarditis, meningitis and rupture of the aortic valve

Arch Intern Med

Pneumococcal endocarditis is not just a disease of the past: an analysis of 16 cases diagnosed in Denmark 1986–1997

Scand J Infect Dis

Letter to the Editor
Austrian syndrome: Report of two new cases and literature review