Mitry, Mira, MD

Smith, Stacey, MD, FACP

Templer, Suzanne J, DO

York, Eugene P, MD, FACP

English

Lehigh Valley Health Network: LVHN Scholarly Works

Lehigh Valley Health NetworkLVHN Scholarly WorksDepartment of MedicineAustrian Syndrome: Triad of the Past or Harbingerof the Future?Mira Mitry MDLehigh Valley Health Network, Mira.Mitry@lvhn.orgSuzanne J. Templer DOLehigh Valley Health Network, Suzanne_J.Templer@lvhn.orgStacey Smith MD, FACPLehigh Valley Health Network, Stacey_J.Smith@lvhn.orgEugene P. York MD, FACPLehigh Valley Health Network, Eugene_P.York@lvhn.orgFollow this and additional works at: http://scholarlyworks.lvhn.org/medicinePart of the Diseases Commons, Infectious Disease Commons, Medical Sciences Commons, andthe Other Medical Specialties CommonsThis Poster is brought to you for free and open access by LVHN Scholarly Works. It has been accepted for inclusion in LVHN Scholarly Works by anauthorized administrator. For more information, please contact LibraryServices@lvhn.org.Published In/Presented AtMitry, M., Templer, S., Smith, S., & York, E. (2013). Austrian syndrome: Triad of the past or harbinger of the future? Poster presentation.Lehigh Valley Health Network, Allentown, PennsylvaniaMira Mitry, MD, Suzanne Templer, DO, Stacey Smith, MD, and Eugene York, MD, FACPIntroductionAustrian Syndrome is a rare complication of disseminated Streptococcus pneumoniae infection, consisting of pneumonia, meningitis, and endocarditis. Case DescriptionA 64 year old male with a history of alcohol and tobacco abuse presented with a fever of 101.5° F and acute change in mental status. According to his wife, he had been coughing for 3 weeks. Physical examination noted left ptosis and mydriasis, decreased RUL breath sounds, and an irregular pulse. Chest x-ray confirmed RUL pneumonia and EKG showed atrial flutter. CT brain was negative for mass or herniation. Lumbar puncture was performed and blood cultures were obtained. Vancomycin and ceftriaxone were then initiated. He was intubated and transferred to the intensive care unit. The cerebrospinal fluid and urine antigen tests were positive for Streptococcus pneumonia, so dexamethasone was started as pneumococcal meningitis treatment. Subsequently, the blood cultures were noted to be positive for gram positive cocci. Transesophageal echocardiogram was ordered to rule out endocarditis, and it showed probable mitral valve vegetation. Later, multiple cranial nerve palsies and a brain empyema developed, with thoracic and lumbar emboli resulting in quadriplegia. The patient expired on hospital day 25. DiscussionThe lungs are the usual portal of entry for pneumococcus leading to pneumonia. There are many predisposing risk factors for invasive pneumococcal disease. Alcoholism is one of the strongest risk factors for pneumococcal endocarditis. The complications of Austrian syndrome include systemic embolization, valve perforation and abscess formation. Transesophageal echocardiography is preferred to transthoracic echocardiography in detecting vegetations. Treatment begins empirically with vancomycin and cefotaxime or ceftriaxone until penicillin sensitivity is resulted. Surgical treatment of the valvular lesions is required in the majority of cases. Austrian syndrome is associated with a 24-63% mortality rate, which makes early diagnosis and appropriate treatment critical. The significance of pneumococcal antimicrobial resistance and the role of vaccination will also be discussed. AbstractAustrian Syndrome: Triad of the Past or Harbinger of the Future?References1	 	Velazquez	C,	Araji	O,	Barquero	M.	Austrian	syndrome:	A	clinical	rarity.	Int	J	Cardio	2008:127:	e36-8.	2	 Aronin	SI,	Mukherjee	SK,	West	JC,	et	al.	Review	of	Pneumococcal	Endocarditis	in	Adults	in	the	Penicillin	Era.	Clin	Infect	Dis	1998;26:165-71.3	 	Du	Cheyron	D,	Lesage	A,	Le	page	O,	et	al.	Corticosteroids	as	adjunctive	treatment	in	Austrian’s	syndrome	(pneumococcal	endocarditis,	meningitis,	and	pneumonia):	report	of	two	cases	and	review	of	the	literature.	J	Clin	Pathol	2003;	56:	879-81.4	 	Blumberg	E,	Karalis	D,	et	al.	Endocarditis	Associated	Paravalvular	Abscesses:	Do	Clinical	Parameters	Predict	the	Presence	of	Abscess?	Chest	1995;	107:898-903.5	 	Auburtin	M,	Porcher	R,	et	al.	Pneumococcal	Meningitis	in	the	Intensive	Care	Unit.	American	Journal	of	Respiratory	and	Critical	Care	Medicine	2002;	165;	713-717.6	 	Trang	M,	Perera	S,	Lonn	E,	and	Dokainish	Hisham.	Pneumococcal	Endocarditis	Causing	Valve	Destruction	in	the	Absence	of	Vegetations	on	Transesophageal	Echocardiography:	A	Series	of	Three	Consecutive	Cases.	Canadian	Journal	of	Cardiology	2012.7	 Baddour	L,	et	al.	Infective	Endocarditis;	Diagnosis	and	Management.	Circulation	2005;111;e394-e434.8	 Bayer	A,	et	al.	Diagnosis	and	Management	of	Infective	Endocarditis	and	Its	Complications.	Circulation	1998;	98:	2936-2948.9	 Lynch	J,	et	al.	Streptococcus	pneumoniae:	Does	Antimicrobial	Resistance	Matter?	Semin	Respir	Crit	Care	Med	2009;30;210-238.	10	 	Song	J,	et	al.	The	relationship	between	pneumococcal	serotypes	and	antibiotic	resistance.	Vaccine	30	(2012)	2728-2737.11	 Gruber	W,	et	al.	Development	and	clinical	evaluation	of	Prevnar	13,	a	13-valent	pneumocococcal	CRM197	conjugate	vaccine.	Ann.	N.	Y.	Acad.	Sci	1263	(2012)	15-26.12	 	Reinert	R.	The	antimicrobial	resistance	profile	of	Streptococcus	pneumoniae.	Clin	Microbial	Infect	2009;	15	(Suppl.	3):	7-11.13	 Richter	S,	et	al.	Pneumococcal	Serotypes	before	and	after	Introduction	of	Conjugate	Vaccines,	United	States,	1999–2011.	Emerging	Infectious	Diseases	2013;	19;7.14	 Song	J,	et	al.	Clinical	Implications	of	Pneumococcal	Serotypes:	Invasive	Disease	Potential,	Clinical	Presentations,	and	Antibotics	Resistance.	J	Korean	Med	Sci	2013;	28:	4-15.15	 Jefferies	J,	et	al.	13-	valent	pneumococcal	conjugate	vaccine	(PCV13).	Human	Vaccines	2011;	7:10,	1012-1018.© 2013 Lehigh Valley Health NetworkAustrian triad was named after Dr. Robert Austrian in 1957, who presented 8 cases that were associated with aortic valve rupture and 6 of which died. The first to relate this triad was an Austrian pathologist, Dr. Heschl in 1862, and subsequently by Sir William Osler in 1881 (the year that pneumococcus was discovered).Lung is the usual portal of entry for Pneumococcus (82%). Once encountered, both humoral and cellular responses are initiated to fight the infection. Invasive pneumococcal disease (IPD) is defined by the isolation of pneumococci from normally sterile sites (pleural fluid, blood and CSF). There are risk factors (local and systemic) that have been identified for IPD. Once bacteremia is sustained, pneumococcus can seed various organs. Meningitis is a common complication, occurring in 59.5% of the cases in one study.  In the preantibiotic era, pneumococcus was responsible for 15-20% of endocarditis. Since the advent of penicillin in the early 1940s, pneumococcal endocarditis has been seen in 3% of patients with infectious endocarditis (IE). Many reports have linked pneumococcal endocarditis with alcoholism, transforming Osler’s triad into a tetrad.Pneumococcal endocarditis is associated with a high mortality rate (24-63%). Several causes have been identified:	 	 A)		The	presentation	is	usually	acute.	As	a	result,	the	patient	usually	lacks	the	peripheral	stigmata	of	endocarditis.		 	 B)		The	destruction	of	endothelial	tissue	generally	occurs	rapidly	followed	by	acute	valvular	insufficiency,	congestive	heart	failure	and	sometimes,	early	death.		 	 C)		Cardiac	and	extracardiac	sequelae	are	common,	specifically	hemodynamic	instability	secondary	to	valve	perforation,	abscess	formation	and	systemic	embolization.	Streptococcus pneumonia has a predilection for the aortic valve (74%). However, a pre-existing valvulopathy is not the rule in pneumococcal endocarditis. Echocardiography plays an important role in the diagnosis of IE: 	 	 1)	 	TTE	is	a	noninvasive,	rapid	procedure	with	excellent	specificity	for	vegetations	(98%),	but	has	poor	sensitivity	(<60%).		Besides,	TTE	alone	cannot	exclude	important	aspects	of	IE,	including	fistulae,	leaflet	perforation,	infection	on	prosthetic	valves,	and	periannular	abscess.	 	 2)	 	TEE,	however,	has	significantly	higher	sensitivity	(76%	to	100%)	and	specificity	(94%)	than	TTE	for	perivalvular	extension	of	infection.	Moreover,	it	enhances	visualization	of	prosthetic	valves,	with	86%	to	94%	sensitivity	and	88%	to	100%	specificity	for	IE.SummaryTreatmentIn acute bacterial meningitis or endocarditis, recommendations are to start patient empirically on cefotaxime/ceftriaxone and vancomycin, until penicillin sensitivity is obtained. If the pneumococcus is resistant to cefotaxime (MIC >2 µg/ml), then a vancomycin and rifampin combination should be used. Corticosteroid is beneficial in treating pneumococcal meningitis patients. It reduces the inflammatory response, increases cerebral perfusion, and improves the adults’ outcome.The clinical course of pneumococcal endocarditis is usually acute and aggressive. Local (peri-valvular abscesses, or perforated valves), and systemic (uncontrolled infection, and multiple emboli) complications frequently occur despite adequate antibiotic usage. As a result, surgical treatment of the valvular lesions is required in the majority of cases. Indications for surgery are as follow:	 	 A)		Vegetation	involvement	(persistent	vegetation	after	systemic	embolization,	anterior	mitral	leaflet	vegetation,	particularly	with	size	>10	mm,	1	or	more	embolic	events	during	2	weeks	of	antimicrobial	therapy,	increase	in	vegetation	size	despite	appropriate	antimicrobial	therapy).7		 	 B)		Valvular	dysfunction	(acute	aortic	or	mitral	insufficiency	with	signs	of	ventricular	failure,	heart	failure	unresponsive	to	medical	therapy,	valve	perforation	or	rupture).7		 	 C)		Peri-valvular	extension	(valvular	dehiscence,	rupture,	or	fistula,	new	heart	block,	large	abscess	or	extension	to	the	abscess	despite	appropriate	antimicrobial	therapy).7		Significance of Pneumococcal Antimicrobial ResistanceCurrently, 15 to 30% of S. pneumoniae worldwide are multidrug-resistant (MDR). Despite increase in antimicrobial resistance worldwide over the past few decades, mortality rates for IPD have not increased. Clinical failures often include factors independent of the pneumococcal antimicrobial susceptibility. These include:	 	 A)		Host	factors	(e.g.,	comorbidities;	extremes	of	age;	or	underlying	immunosuppression).	 	 B)		Pneumococcal	virulence	(e.g.,	capsular	subtype).		 	 C)		Mortality	rates	are	also	higher	in	the	presence	of:	multilobar	involvement,	hypoxemia,	renal	insufficiency,	and	the	need	for	ICU	care.	Given the above confounding factors, dissecting out the impact of antimicrobial resistance on clinical outcomes is difficult, if not impossible. Vaccination and its ImportanceIn the United States, about 39,750 cases of IPD and 4,000 deaths occur annually. The clinical spectrum from colonization to IPD depends on the pneumococcal capsular serotype. Currently, 94 capsular serotypes have been identified. Six serotypes (i.e., 4, 6B, 9V, 14, 19F, and 23F) account for >80% of IPD in children and >50% of IPD in adults in the United States. They also account for the majority of IPD in Europe.Since the introduction of the PCV7 (4, 6B, 9V, 14, 18C, 19F, 23F), the rate of IPD due to PCV7 serotypes has declined significantly in many countries. In the US, it decreased from 64% of invasive and 50% of noninvasive isolates in 1999–2000 to 3.8% and 4.2%, respectively, in 2010–2011. The PCV7 also has indirect (herd) effects that have led to decreased incidence of vaccine serotypes disease in unvaccinated children and adults.  However, there have been reports of an increase in non-PCV7 serotypes, especially 19A. This phenomenon is termed ‘replacement’.PCV13 adds pneumococcal serotypes 1, 3, 5, 6A, 7F, and 19A to PCV7’s serotypes to provide coverage for over 85% of epidemiologically significant pneumococcal serotypes in the United States and throughout the world.Austrian Triad and VaccinationReports of pneumococcal endocarditis often failed to provide adequate data on either the infecting pneumococcal serotype or patients’ vaccination histories. Among the limited data we have, Aronin et el showed that the most common capsular serotypes that were identified causing Austrian triad were 12, 1, and 8. Hence, PCV 13 could potentially prevent a significant number of Austrian syndrome cases.Future ProspectsThe development of effective conserved pneumococcal protein vaccines (CPPV) that would not target the polysaccharide capsule are currently in trials. These may prevent pneumococcal disease and carriage without resulting in the selective pressure that is thought to drive serotype replacement.

Austrian Syndrome: Triad of the Past or Harbinger of the Future?

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Austrian Syndrome: Triad of the Past or Harbinger of the Future?

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