EUH Krakow Conference: Review of Lemierre’s Syndrome

Bottom Line: Vijay and Fattah1 state that Lemierre’s syndrome typically presents in young adults who were previously healthy. In a case series of 222 patients who fit the “Lemierre’s syndrome case presentation, the median age was 19 years and 89% of patients were aged 10 to 35 years.”2 Its four key elements are “primary oropharyngeal infection within 4 weeks, suppurative thrombophlebitis of the internal jugular (IJ) vein, metastatic septic emboli, and [a] causal association with F necrophorum.” It should be “suspected in those with a recent history of oropharyngeal infection presenting with fever and rigors, with or without evidence of metastatic lesions, particularly respiratory symptoms (pleuritic chest pain, dyspnoea, haemoptysis).” Penicillin/beta-lactamase inhibitor, penicillin plus metronidazole, and carbapenem” are “appropriate empirical antibiotic regimens….There are no controlled trials to guide management, but most sources recommend between 2–6 weeks of antibiotics in total.” Review article provides four computed tomography pulmonary angiogram (CTPA) images with red arrows that indicate locations of multiple peripheral nodular lesions.

References:

1. Vijay V, Fattah Z. Lesson of the month 1: Lemierre‘s syndrome: a reminder of the ‘forgotten disease.’ Clin Med (Lond). 2018 Feb;18(1):100-102. doi:10.7861/clinmedicine.18-1-100.

2. Riordan T. Human infection with Fusobacterium necrophorum (Necrobacillosis), with a focuson Lemierre’s syndrome. Clin Microbiol Rev. 2007 Oct;20(4):622-59. doi:10.1128/CMR.00011-07.

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EUH Morning Report: Review of microscopic colitis

Bottom Line: “Microscopic colitis is a common cause of chronic watery diarrhea, particularly in the elderly. The accompanying symptoms, which include abdominal pain and fatigue, can markedly impair patients’ quality of life. Diagnosis is based upon characteristic histologic findings of the colonic mucosa….Two recent randomized studies have confirmed the effectiveness of oral budesonide for both induction and maintenance treatment of microscopic colitis and is now endorsed by the American Gastroenterological Association as first-line treatment.”

Reference: Cotter TG, Pardi DS. Current approach to the evaluation and management of microscopic colitis. Curr Gastroenterol Rep. 2017 Feb;19(2):8.

EUH Dressler Case Conference: What is the definition of heart failure with mid-range ejection fraction (HFmrEF), which is also called intermediate ejection fraction and borderline heart failure with preserved left ventricular (LV) ejection fraction (HFpEF)? Do patients benefit from typical HFrEF therapies?

Bottom Line: In a meta-analysis, Zheng, Chan, Nabeebaccus, et al. state, “The latest European Society of Cardiology guidelines introduced the term heart failure with mid-range ejection fraction (HFmrEF), categorising an intermediate group of patients with an LV ejection fraction of between 40% and 49%, with HFpEF defined as an LV ejection fraction ≥50% with the same echocardiographic criteria. The American College of Cardiology defines HFpEF as an LV ejection fraction >40%, with anything from 41% to 49% as borderline HFpEF.” Dressler reviewed the aforementioned article and states, “In 6 randomized trials (≈1300 patients), β-blockers, compared with placebo, significantly lowered early mortality (18% vs. 20%). In 15 randomized trials (>13,000 patients), HFpEF patients who received any drug class that improves [heart failure with reduced ejection fraction (HFrEF) outcomes were significantly less likely to be hospitalized (14% vs. 16% with placebo; number needed to treat, 64), but no individual medication class yielded significant improvements.” Drug classes that are typically used to treat HFrEf and were evaluated in the meta-analysis include β-blockers, angiotensin-converting–enzyme inhibitors, angiotensin-receptor blockers, and mineralocorticoid-receptor antagonists.
References
Zheng SL, Chan FT, Nabeebaccus AA, et al. Drug treatment effects on outcomes in heart failure with preserved ejection fraction: a systematic review and meta-analysis. Heart. 2017 Aug 5.
Dressler DD. Do any meds improve outcomes in heart failure with preserved ejection fraction. N Engl J Med J Watch. 2018 Jan 16.

EUH Dressler Case Conference: In chest x-rays, what is the water bottle sign?

Bottom Line: In patients with pericardial effusion, the water bottle sign appears when the chest x-ray’s cardiac silhouette has a globular shape that looks like a water bottle as the result of slowly accumulating fluid. In the screenshot from YouTube below, the four images show a normal chest x-ray (top left corner), a patient with cardiomegaly (lower right corner), and a chest x-ray with a water bottle sign (lower right corner). The water bottle shape appears in the upper right corner.
water bottle sign

Reference for Image: Hammadshams. Chest X-Ray – Cardiomegaly or No Cardiomegaly – Water Bottle Sign. https://www.youtube.com/watch?v=oG_bE2x5LG8. Accessed January 23, 2018.

EUH Hunt Conference: What is the sensitivity of cytology tests of pleural effusion and ascites?

Bottom Line: Sensitivity of ascitic cytology is approximately 60%, and the sensitivity of pleural effusion cytology is approximately 50%.

References:

Karoo RO, Lloyd TD, Garcea G, Redway HD, Robertson GS. How valuable is ascitic cytology in the detection and managent of malignancyPostgrad Med. 2003 May;79(931):292-294. Study included 276 samples.

Motherby H, Nadjari B, Friegel P, Kohaus J, Ramp U, Bocking A. Diagnostic accuracy of effusion cytology. Diagn Cytopathol. 1999 Jun;20(6):350-357. Study included 300 pleural effusions and 300 ascitic effusions and provides sensitivity for both types of effusions.

 

EUH Hunt Conference: What is the diagnostic workup of cardiac sarcoidosis?

The Bottom Line: Cardiac sarcoidosis has varied presentations depending on the extent and location of cardiac granulomas. Patients may have AV block, heart failure, or sudden cardiac death. The disease should be considered especially in young or middle-aged women who present with heart failure and/or AV block or ventricular arrhythmias.

Echocardiography may show septal thinning, wall motion abnormalities in a noncoronary distribution, or LV aneurysm. Diagnosis can be made by EMB; however, given the patchy distribution of disease within the myocardium, the sensitivity is only 20–26%. Positron emission tomography and magnetic resonance imaging are both useful modalities for identifying cardiac sarcoidosis. Diagnosis requires a combination of clinical/imaging evidence of cardiac involvement along with tissue diagnosis of extracardiac sarcoid.

References: DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 306338, Cardiac sarcoidosis; [updated 2017 Mar 31, cited 2017 December 19]; [about 11 screens]. Emory login required.

Selby, Van N.. “Myocarditis, Toxic Cardiomyopathy, and Stress Cardiomyopathy.” CURRENT Diagnosis & Treatment: Cardiology, 5e   Ed. Michael H. Crawford. New York, NY: McGraw-Hill.

Summary: According to DynaMed Plus (2017), to make the diagnosis:

  • suspect cardiac sarcoidosis in patients with extracardiac sarcoidosis plus
    • cardiac symptoms, such as palpitations, presyncope or syncope, or symptoms of heart failure (such as dyspnea)
    • cardiac manifestations, such as atrioventricular (AV) block, ventricular tachycardia, atrial arrhythmia, or supraventricular tachycardia
  • isolated cardiac sarcoidosis may be suspected in patients without extracardiac sarcoidosis but with cardiac manifestations of either
    • unexplained high-degree (Mobitz type II or third-degree) AV block
    • symptomatic left atrial tachycardia without history of ablation or surgery for atrial fibrillation
  • no formal diagnostic criteria for cardiac sarcoidosis have been established but 3 guidelines have proposed criteria, which generally include
    • histological diagnosis if noncaseating granulomas composed of epithelioid cells in myocardial tissue on endomyocardial biopsy
    • clinical diagnosis if all of
      • extracardiac sarcoidosis confirmed on histology in another organ
      • presence of ≥ 1 criteria for cardiac sarcoidosis
      • other causes of cardiac manifestation have been reasonably excluded

EUH Hunt Conference: What is the sensitivity/specificity of ACE levels in cardiac sarcoidosis?

The Bottom Line:  Serum levels of angiotensin-converting enzyme (ACE) can be helpful in the diagnosis of sarcoidosis. However, the test has somewhat low sensitivity and specificity. Elevated levels of ACE are reported in 60% of patients with acute disease and only 20% of patients with chronic disease. Although there are several causes for mild elevation of ACE, including diabetes, elevations of >50% of the upper limit of normal are seen in only a few conditions including sarcoidosis, leprosy, Gaucher’s disease, hyperthyroidism, and disseminated granulomatous infections such as miliary tuberculosis. Because the ACE level is determined by a biologic assay, the concurrent use of an ACE inhibitor such as lisinopril will lead to a very low ACE level.

Reference: Baughman RP, Lower EE. Sarcoidosis. In: Kasper D, Fauci A, Hauser S, Longo D, Jameson J, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 19e New York, NY: McGraw-Hill; 2014.

EUH Krakow Conference: What is the diagnosis and treatment for complement-mediated thrombotic microangiopathy?

The Bottom Line: The clinical presentation of thrombotic microangtiopathy (TMA) reflects hemolysis and ischemic organ dysfunction, and depends on the underlying disease etiology. AKI is a common manifestation of TMAs although it is rarely a severe feature of TTP.

The initial priority should be the consideration of TTP, because urgent management is imperative given the high mortality if untreated, and therefore in adults, PE should be instituted (after obtaining a sample for ADAMTS13 activity testing) on the presumption that it is TTP unless other evidence is available that strongly suggests an alternative cause. If the ADAMTS13 result excludes TTP, then complement-mediated aHUS is presumed and treatment with eculizumab is recommended, pending the complete evaluation.

Reference: Brocklebank V, Wood KM, Kavanagh D. Thrombotic microangiopathy and the kidney. Clin J Am Soc Nephrol. 2017 Oct 17. pii:CJN.00620117. doi:10.2215/CJN.00620117.

Summary:  Complement-mediated aHUS is prototypical of disease occurring as a consequence of complement dysregulation. The complement system is a regulated cascade network of signaling and amplification that incorporates >30 plasma and cell surface–bound proteins.

In complement-mediated aHUS, dysregulated complement activation primarily occurs on endothelial cell surfaces, and although abnormal serum levels of complement components such as C3 may be observed, normal levels do not exclude complement-mediated disease. There is evidence of complement activation (plasma levels and tissue staining) in many other TMAs, but whether this is a primary event, a disease modifier, or an inconsequential bystander phenomenon has not yet been definitively established.

EUH Dressler Conference: What is the epidemiology of legionella pneumonia?

The Bottom Line: Legionella pneumonia accounts for 2-7% of community-acquired pneumonia (CAP) worldwide. In the U.S., legionella infections accounted for 1.3 per 100,000 cases between 2011-2013.

Of the 1,426 cases reported in the U.S between 2011-2013:

  • 79% in patients > 50 years old
  • 65% in male patients
  • 72% in white patients

(DynaMed Plus, 2017)

Reference: DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 904756, Legionella infections; [updated 2016 May 20, cited 2017 December 7]; [about 12 screens]. Emory login required.

Edelstein PH, Roy CR. Legionnaires’ Disease and Pontiac Fever. In Mandell, Douglas, and Bennett’s Principles and Practice of Infectious Diseases, Updated Edition (8th ed). 2014.

Summary: The incubation period during most outbreaks of legionnaires disease is between 2 and 10 days, with median values of 4 to 6 days, and some outliers from 1 up to 28 days. Its mortality rates are highly variable, ranging from less than 1% to as high as 80%, depending on the underlying health of the patient, promptness of specific therapy, and whether the disease is sporadic, nosocomial, or part of a large outbreak.

Legionnaires Disease is transmitted from the environment to humans by inhalation of an infectious aerosol. Multiple examples of exclusive aerosol transmission exist, especially in cases having a cooling tower, water spa, water fountain, or water mister as the source of disease. Proximity to the aerosol generator, duration of exposure, and presence in an area downstream of the contaminated device have all been found to be risk factors for disease acquisition (Edelstein and Roy, 2015).

EUH Dressler Conference: What is the sensitivity/specificity of the legionella urine antigen test?

The Bottom Line: The sensitivity and specificity of the Legionella urine antigen test are as high as 90% and 99%, respectively. The test for Legionella pneumophila detects only serogroup 1, but this serogroup accounts for most community-acquired cases of Legionnaires’ disease in the United States (Mandell and Wunderink, 2014).

References: DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Legionella urine antigen; [updated 2017 July 07; cited 2017 Dec 12]; [about 24 screens]. Emory login required.

Mandell LA, Wunderink RG. Pneumonia. In: Kasper D, Fauci A, Hauser S, Longo D, Jameson J, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 19e New York, NY: McGraw-Hill; 2014.

Summary: Urinary antigen detection provides an early diagnosis (within 15 minutes with an immunochromatographic assay) and is particularly valuable in areas where L pneumophila serogroup 1 is the most common cause of the disease. The test also is a useful screening tool for investigating outbreaks of legionnaires’ disease (DynaMed Plus, 2017).