Angioedema Publications (7095)


Angioedema Publications

To estimate health-related quality-of-life changes in patients with hereditary angioedema due to C1-inhibitor (C1-INH) deficiency who received subcutaneous C1-INH with recombinant hyaluronidase (rHuPH20) for attack prophylaxis in a randomized, double-blind, dose-ranging, cross-over study.
Patients with type I/II hereditary angioedema received 1000 U of C1-INH with 24,000 U of rHuPH20 or 2000 U of C1-INH with 48,000 U of rHuPH20 every 3-4 days for 8 weeks and then crossed over for another 8-week period. The study was terminated early as a precaution related to non-neutralizing antibodies to rHuPH20. Read More

The Angioedema Quality of Life questionnaire (AE-QoL) was administered at weeks 1 and 5 of both periods, and at 1 week after the second treatment period. Changes in AE-QoL scores were calculated over both treatment periods and within each treatment period for patients with greater then or equal to 4 weeks of treatment.
Forty-one patients had evaluable AE-QoL data, and 22 patients completed treatment. At screening, 43% of the patients were receiving intravenous C1-INH. A significant average AE-QoL total score decline (improvement) of -8.1 (95% confidence interval, -13.7 to -2.5) was observed from baseline to the end of the study, and significant AE-QoL score declines were observed in the Functioning, Fear/Shame, and Nutrition domains. Patients on 2000 U reported higher mean AE-QoL score declines in Functioning and Nutrition domains relative to the 1000 U dose. Overall, 43.9% of all the patients, 45.5% of the study completers, and 46.7% of the nonprophylaxis users at baseline on high treatment doses achieved a reduction in the AE-QoL total score of six points.
Despite early termination and prestudy prophylactic intravenous C1-INH use by 43% of the patients, improved AE-QoL scores were observed after less than or equal to 16 weeks of subcutaneous C1-INH-rHuPH20 prophylaxis.

Expert Rev Neurother
Expert Rev Neurother 2017 Jan 17. Epub 2017 Jan 17.
d Consultant neurologist , Gloucestershire Hospitals NHS Foundation Trust Ringgold standard institution , Great Western Road, Gloucester , United Kingdom of Great Britain and Northern Ireland.

Autoimmunity is an important cause of disease both in the central and peripheral nervous systems. Aetiologies and clinical manifestations are complex and heterogeneous. Inappropriate control of complement activation at inappropriate sites has been recognized as a major determinant in several neurological conditions, including Guillain-Barré syndrome and neuromyelitis optica. Read More

In each case pathogenesis is thought to be associated with generation of autoantibodies which upon binding guide activation of the complement system to self-tissue. Areas covered: Modulation of the complement system activation at such sites may represent a novel therapeutic approach for treatment of immune-mediated inflammatory conditions. In this review we focus on the therapeutic effects of complement inhibitors in Guillain-Barré syndrome and neuromyelitis optica and highlight recent developments within the field. Expert Commentary: Conventional first line treatment strategies in GBS and NMO have the potential disadvantage of causing widespread immunosuppressive effects. A more targeted approach may therefore be more effective and less disruptive to the immune system, especially in the case of NMO, which requires long term immunosuppression. Modulation of the complement system may hold the key and has already been shown to be of clinical benefit in other non-neurological conditions, including paroxysmal nocturnal hemoglobinuria and hereditary angioedema.

Am J Case Rep
Am J Case Rep 2017 Jan 16;18:52-59. Epub 2017 Jan 16.
Department of Medicine, Riverside University Health System, Moreno Valley, CA, USA.
Expert Opin Pharmacother
Expert Opin Pharmacother 2017 Jan 12. Epub 2017 Jan 12.
a Department of Internal Medicine, Division of Immunology, Rheumatology, and Allergy , University of Cincinnati College of Medicine.

Introduction Angioedema, a sudden, self-limited swelling of localized areas of any part of the body that may or may not be associated with urticaria, is thought to be the result of a mast-cell mediated process versus a bradykinin etiology. Understanding the mechanism is key in determining the proper treatment. Areas Covered Clinical presentation of varying angioedema types may be similar; however, the appropriate treatment algorithm is dependent upon clinicians' knowledge of the underlying pathophysiology and classification of angioedema. Read More

Literature review of recent guidelines, available medications, and alternative therapies was completed to provide an overview of options. Conclusion There are no formal guidelines for treatment of acute or chronic histamine-mediated angioedema, and therefore, algorithms for the treatment of acute and chronic urticaria should be followed until such information becomes available. Differentiating histamine-mediated versus bradykinin mediated angioedema is essential, as treatments and treatment responses are quite different. Further research is needed to better understand idiopathic angioedema that is unresponsive to H1/H2 antagonists, LTMAs, or medications designed to treat bradykinin-mediated angioedema.

BMJ Case Rep
BMJ Case Rep 2017 Jan 10;2017. Epub 2017 Jan 10.
Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, Copenhagen, Denmark.

Angioedema (AE) of the upper airways is a severe and potentially life-threatening condition. The incidence has been increasing in the past two decades, primarily due to increased use of medications inhibiting the degradation of vasoactive peptides. Acquired angioedema related to angiotensin-converting enzyme inhibitors (ACEI-AAE) is well known, but other pharmaceutical agents also affect the degradation of bradykinin and substance P. Read More

We present a middle-aged man with recurrent episodes of severe AE of the oral cavity, hypopharynx and larynx due to pharmacological inhibition of dipeptidyl peptidase IV.

Orphanet J Rare Dis
Orphanet J Rare Dis 2017 Jan 10;12(1). Epub 2017 Jan 10.
Hungarian Angioedema Center, 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi street 4, H-1125, Budapest, Hungary.

Hereditary angioedema caused by C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. C1-INH-HAE is characterized by edema-formation, which may occur in response to stress. The individual's response to stress stimuli is partly genetically determined. Read More

Activation of the hypothalamic-pituitary-adrenal axis results in the release of cortisol. In turn, the secreted gluco- and mineralocorticoids affect the metabolism, as well as the cardiovascular and immune systems. We hypothesized that changes in serum cortisol level and polymorphisms of the glucocorticoid receptor (GR) modify the individual sensitivity to stressor stimuli of C1-INH-HAE patients.
We compared the response to stress with Rahe's Brief Stress and Coping Inventory of 43 C1-INH-HAE patients, 18 angioedema patients and 13 healthy controls. 139 C1-INH-HAE patients and 160 healthy controls were genotyped for glucocorticoid receptor polymorphisms BclI, N363S and A3669G. Serum cortisol levels were determined during attacks and during symptom-free periods in 36 C1-INH-HAE patients. The relationships between clinical, laboratory data and GR SNPs (Single Nucleotide Polymorphisms) were assessed using ANOVA. C1-INH-HAE patients have decreased coping capabilities compared to healthy controls. Cortisol levels were significantly higher during attacks than in symptom-free periods (p = 0.004). The magnitude of the elevation of cortisol levels did not show a significant correlation with any clinical or laboratory data. Among the C1-INH-HAE patients, the carriers of the A3669G allele had significantly lower cortisol levels, and increased body mass index compared with non-carriers.
The higher cortisol level observed during attacks may reflect the effect of a stressful situation (such as of the attack itself), on the patients' neuroendocrine system. In A3669G carriers, the lower cortisol levels might reflect altered feedback to the hypothalamic-pituitary-adrenal axis, due to decreased sensitivity to glucocorticoids.

Expert Opin Drug Saf
Expert Opin Drug Saf 2017 Jan 6. Epub 2017 Jan 6.
a School of Medicine , University of California San Francisco , San Francisco , CA , USA.

Sacubitril-valsartan is a combination drug that contains the neprilysin inhibitor sacubitril and angiotensin II receptor blocker valsartan. In 2015, the US Food and Drug Administration approved sacubitril-valsartan for treatment of heart failure patients with reduced ejection fraction and New York Heart Association class II-IV symptoms following a large, Phase III clinical trial (PARADIGM-HF) that demonstrated a 20% reduction in the combined primary end-point of death from cardiovascular cause or hospitalization for heart failure compared to enalapril. Areas covered: This review discusses the clinical efficacy and safety of angiotensin receptor neprilysin inhibitor sacubitril-valsartan in heart failure with reduced ejection fraction. Read More

Expert opinion: Based on the PARADIGM-HF trial, sacubitril-valsartan offers compelling reductions in meaningful clinical endpoints, independent of age or severity of disease. The rate of adverse events was comparable between the enalapril and sacubitril-valsartan groups, although the absolute rates are likely underestimated due to the entry criteria and run-in period. Future trials and post-market surveillance are critical to better understand the risk of angioedema in high risk populations, particularly African-Americans, as well as long-term theoretical risks including the potential for increased cerebral amyloid plaque deposition with possible development of neurocognitive disease. Current trials are underway to evaluate potential benefit in patients with heart failure with preserved ejection fraction.

J Neurosci Nurs
J Neurosci Nurs 2017 Feb;49(1):31-36
Questions or comments about this article may be directed to Hannah Fernandez-Gotico, MS RN-BC CPHQ at She is a Quality Director of Clinical Services, Kaiser Permanente, South San Francisco, CA. Tiffany Lightfoot, MS RN CPPS, is Healthcare Consultant, The Rosenberger Group, Los Angeles, CA. Melissa Meighan, MS RN CNRN, is Regional Stroke Coordinator, Kaiser Northern California, Oakland, CA.

The approved treatment by the Food and Drug Administration for acute ischemic stroke is intravenous tissue-type plasminogen activator (IV tPA). After IV tPA administration, patients are monitored for adverse events using an American Heart Association/American Stroke Association guideline instituted in 1996. There is limited evidence describing the safest and most efficient method to monitor patients during the first 24 hours after tPA administration. Read More

Although the overall rates of adverse events have been reported, the time when patients may be at most risk for an event has not been studied. The purpose of this study was to identify the time of adverse event occurrences in the first 24 hours after IV tPA administration.
This was a descriptive, retrospective chart review study of patients admitted to an integrated health system and treated with IV tPA for acute stroke between July 2010 and July 2012. Charts were reviewed for adverse events using the Institute for Healthcare Improvement's Global Trigger Tool for Measuring Adverse Events. Possible chart indicators of adverse events or "triggers" included neurological decline, vital signs elevated above specified parameters, and emergent imaging. Adverse events included episodes of neurological decline, angioedema, allergic reactions, bleeding, and intracerebral hemorrhage (ICH). The timing of each detected event was determined, and descriptive statistics were used for data analysis.
Fourteen adverse events (2.8%) were detected in a population of 498 patients. Reactions consisted of allergic reaction (n = 1), angioedema (n = 1), neurological decline without ICH (n = 1), gastrointestinal bleeding (n = 1), bleeding gums (n = 1), and high-risk ICH (n = 9). Thirteen of the 14 adverse events (92.9%) occurred within the first 12 hours after IV tPA administration.
Close monitoring during the first 12 hours after IV tPA treatment may be essential. However, close monitoring after 12 hours may not contribute significantly to improved patient outcomes. Larger studies may provide evidence for the safest and most efficient monitoring protocol for patients treated with IV tPA for ischemic stroke.

Eur J Emerg Med
Eur J Emerg Med 2017 Jan 4. Epub 2017 Jan 4.
aDepartment of Anaesthesiology and Intensive care, Edouard Herriot University Hospital bDepartment of Clinical Research and Innovation, Hospices Civils de Lyon, Lyon cEmergency Department, Louis Mourier University Hospital, Paris 7 University dDepartment of Internal Medicine, Saint Antoine University Hospital, Paris 6 University, Assistance Publique-Hôpitaux de Paris, Paris eDepartment of Internal Medicine, Grenoble University Hospital, Grenoble-Alpes University, Grenoble fDepartment of Dermatology, Gabriel-Montpied University Hospital, Clermont-Ferrand gDepartment of Internal Medicine, Niort Hospital, Niort hDepartment of Internal Medicine, Archet 1 University Hospital, Nice Sophia-Antipolis University, Nice iDepartment of Medicine, Saint Louis University Hospital, Saint Pierre, Réunion jDepartment of Internal Medicine, Caen University Hospital, Caen kDepartment of Dermatology and Allergology, Grenoble University Hospital, Grenoble lDepartment of Internal Medicine, Timone University Hospital, Assistance Publique-Hôpitaux de Marseille, Marseille mDepartment of Dermatology, Saint Eloi University Hospital, Montpellier nDepartment of Dermatology, Besançon University Hospital, Franche-Comté University, INSERM UMR 1098, Besançon oDepartment of Internal Medicine Toulouse University Hospital, Toulouse University, Toulouse pDepartment of Internal Medicine, Lille University Hospital, Lille University, INSERM U995 Lille, Lille, France.

Angio-oedema is a transitory, localized, noninflammatory oedema of subcutaneous tissue or mucous. When the oedema affects the mouth, lips, tongue or larynx, it can result in fatal asphyxiation in the absence of specific treatment. Oedema secondary to plasma extravasation is usually mediated by either histamine or bradykinin. Read More

As laboratory tests are not available in an emergency setting, the implicated mediator cannot be readily determined. The challenge for the emergency physician is to determine the aetiological type, evaluate severity and initiate adapted treatment by means of a structured approach. A team of experts from the French Reference Centre for Angio-oedema reached a consensus for recommendations for the diagnostic and therapeutic strategy to be adopted by emergency departments faced with angio-oedema of the upper airways in adults. The experts defined 11 important questions. Responses were rated using a two-round Delphi methodology. The 11 recommendations were related to triage on admission, a step-by-step diagnostic protocol, definition of attack severity, discouragement of instrumental examination, prioritization of treatment for severe attacks according to clinical signs and anticipation of access to specific treatments by the hospital. Angio-oedema of the upper airways can be fatal and requires anticipation by the emergency department. A search for the aetiology, an evaluation of clinical symptoms and the availability of the treatments are challenges justifying these recommendations.

Germs 2016 Dec 2;6(4):151-154. Epub 2016 Dec 2.
MD, Université Catholique de Bukavu, Bukvau, Democratic Republic of Congo, Université de Kinshasa, Kinshasa, Democratic Republic of Congo.

Mansonella perstans is a human filarial parasite transmitted by biting midges (Diptera: Ceratopogonidae) belonging to the genus Culicoides and it is widely spread in sub-Saharan Africa. While most cases are asymptomatic, mansonelliasis can be associated with angioedema, arthralgia, swellings, pain in the scrotum or in serous cavities such as the pleura, the peritoneum, the pericardium, etc. Mansonelliasis can be really hard to treat, but it has been shown that an intensive treatment using albendazole can clear the parasite. Read More

Here we describe a case of a 16 months-old malnourished child with pneumonia due to M. perstans in the east of the Democratic Republic of Congo.
Although our investigations confirmed M. perstans infection, this case shows that it is very difficult to come to a conclusive diagnosis.