A score for the differential diagnosis of bradykinin- and histamine-induced head and neck swellings

Lenschow, M; M, Bas; Johnson, F. R.; Wirth, Markus; Straßen, Ulrich

doi:10.1007/s00405-018-4989-1

Cited by 7 publications

(8 citation statements)

References 38 publications

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“…Differentiating histamine and bradykinin-mediated angioedema can be difficult. One retrospective study evaluated 188 patients, with one point assigned to age > 65 years, dyspnea, no itching or erythema, laryngeal involvement, and intake of ACEi/AT-II antagonist, and two points assigned if there was no response to steroid therapy 97. If the score was ≥ 3 points, the patient was treated with C1-INH or B2 receptor antagonist for suspicion of bradykinin-mediated angioedema.…”

Section: Discussionmentioning

confidence: 99%

“…If the score was ≥ 3 points, the patient was treated with C1-INH or B2 receptor antagonist for suspicion of bradykinin-mediated angioedema. This resulted in a sensitivity of 96% and specificity of 84% for the diagnosis of bradykinin-mediated angioedema 97. While this tool can help to differentiate the underlying etiology, it requires further validation before routine use.…”

Section: Discussionmentioning

confidence: 99%

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Evaluation and Management of Angioedema in the Emergency Department

Long

Koyfman

Gottlieb

2019

WestJEM

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Angioedema is defined by non-dependent, non-pitting edema that affects several different sites and is potentially life-threatening due to laryngeal edema. This narrative review provides emergency physicians with a focused overview of the evaluation and management of angioedema. Two primary forms include histamine-mediated and bradykinin-mediated angioedema. Histamine-mediated forms present similarly to anaphylaxis, while bradykinin-mediated angioedema presents with greater face and oropharyngeal involvement and higher risk of progression. Initial evaluation and management should focus on evaluation of the airway, followed by obtaining relevant historical features, including family history, medications, and prior episodes. Histamine-mediated angioedema should be treated with epinephrine intramuscularly, antihistaminergic medications, and steroids. These medications are not effective for bradykinin-mediated forms. Other medications include C1-INH protein replacement, kallikrein inhibitor, and bradykinin receptor antagonists. Evidence is controversial concerning the efficacy of these medications in an acute episode, and airway management is the most important intervention when indicated. Airway intervention may require fiberoptic or video laryngoscopy, with preparation for cricothyrotomy. Disposition is dependent on patient’s airway and respiratory status, as well as the sites involved.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Evaluation and Management of Angioedema in the Emergency Department

Long

Koyfman

Gottlieb

2019

WestJEM

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“…So far, all our patients have been successfully treated during laryngeal attacks thanks to a properly conducted therapy and appropriate training. In case of recurrent, refractory to glucocorticosteroids and antihistamines laryngeal swellings, especially with an accompanying positive family history, hereditary angioedema due to C1-inhibitor deficiency should always be taken into account in the differential diagnosis and management (see additional materials) [12,13,14].…”

Section: Discussionmentioning

confidence: 99%

Life-threatening laryngeal attacks in hereditary angioedema patients

Piotrowicz-Wójcik

Porębski

2020

Otolaryngol Pol

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Background: Hereditary angioedema due to C1-inhibitor deficiency (HAE-C1INH) is a rare genetic disease that runs in the family. As a result of the disease, acute swellings of the subcutaneous tissue and mucous membranes of the digestive and respiratory systems, including the larynx, occur. Any attack of the disease involving the throat and larynx is particularly dangerous and requires knowledge of clinical determinants of the disease and its proper management. Materials and methods: The study included adult consecutive HAE-C1INH patients having follow-up visits in our centre. The group was examined with a structured clinical questionnaire, concerning the last 6 months and focusing particularly on laryngeal swelling attacks. Results: 55 subjects (F/M – 35/20, age range – 18–76) were included in the study. Laryngeal attacks occurred in 19 individuals (34.5%): 1–3, 4–6, and ≥7 attacks in 9, 8 and 2 patients, respectively, two of whom required intubation. In comparison to other patients, subjects with laryngeal attacks were characterised by significantly more frequent: (1) facial attacks, (2) severe disease activity, (3) the occurrence of female patients, (4) mental stress as a trigger of attacks. All patients with laryngeal attacks had a rescue medication at home and 15/19 (78%) patients could use it at home. Most of them used plasma-derived C1-inhibitor 17/19 (89.5%) and icatibant, 8/19 (42.1%). Discussion: HAE-C1INH patients with laryngeal attacks require particular attention. Proper training regarding the identification of these patients, adequate management, access to emergency services and emergency drugs are essential to ensure the safety of subjects with this localization of HAE-C1INH attacks.

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“…HAE is a relatively rare form of non-allergic angioedema, and it is associated with major morbidity and potential mortality [10,11]. While HAE may have similar manifestations as other types of angioedema, there is a notable absence of urticaria [12,13].…”

Section: Introductionmentioning

confidence: 99%

“…Historically, misunderstanding of symptoms and low awareness of HAE has contributed to misdiagnosis and delayed diagnoses [12]. Given the unique nature of HAE in relation to other types of angioedema, the potential for adverse consequences with incorrect or delayed diagnosis, and the availability of highly effective treatment options for HAE, it is imperative to consider HAE in the differential diagnosis of angioedema [10,12]. This article presents an illustrative case study of HAE, provides an overview of epidemiology and pathophysiology, reviews guidelines for diagnosis and management, and describes current and emerging treatment options.…”

Section: Introductionmentioning

confidence: 99%

The importance of recognizing and managing a rare form of angioedema: hereditary angioedema due to C1-inhibitor deficiency

Jacobs

Neeno

2021

Postgraduate Medicine

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The majority of angioedema cases encountered in clinical practice are histamine-mediated (allergic); however, some cases are bradykinin-related (non-allergic) and do not respond to standard anti-allergy medications. Among bradykinin-related angioedema, hereditary angioedema (HAE) is a rare, but chronic and debilitating condition. The majority of HAE is caused by deficiency (type 1) or abnormal function (type 2) of the naturally occurring protein, C1-inhibitor (C1-INH)-a major inhibitor of proteases in the contact (kallikrein-bradykinin cascade), fibrinolytic pathway, and complement systems. Failure to recognize HAE and initiate appropriate intervention can lead to years of pain, disability, impaired quality of life (QoL) and, in cases of laryngeal involvement, it can be life-threatening. HAE must be considered in the differential diagnosis of non-urticarial angioedema, particularly for patients with a history of recurrent angioedema attacks, family history of HAE, symptom onset in childhood/adolescence, prodromal signs/symptoms before swellings, recurrent/painful abdominal symptoms, and upper airway edema. Management strategies for HAE include on-demand treatment for acute attacks, shortterm prophylaxis prior to attack-triggering events/procedures, and long-term or routine prophylaxis for attack prevention. Patients should be evaluated at least annually to assess need for routine prophylaxis. HAE specific medications like plasma-derived and recombinant C1-INH products, kallikrein inhibitors, and bradykinin B2 receptor antagonists, have improved management of HAE. While the introduction of intravenous C1-INH represented a major breakthrough in routine HAE prophylaxis, some patients fail to achieve adequate control and others have psychological barriers or experience complications related to intravenous administration. Subcutaneous (SC) C1-INH, SC monoclonal antibody (mAb)-based therapies, and an oral kallikrein inhibitor offer effective alternatives for HAE attack prevention and may facilitate self-administration. HAE management should be individualized, with QoL improvement being a key goal. This can be achieved with broader availability of existing options for routine prophylaxis, including greater global availability of C1-INH(SC), mAb-based therapy, oral treatments, and multiple on-demand therapies.

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A score for the differential diagnosis of bradykinin- and histamine-induced head and neck swellings

Abstract: Utilization of the proposed score allows quick and reliable assignment of patients to the correct subgroup and thereby reduces time for treatment.

Cited by 7 publications

References 38 publications

Evaluation and Management of Angioedema in the Emergency Department

Evaluation and Management of Angioedema in the Emergency Department

Life-threatening laryngeal attacks in hereditary angioedema patients

The importance of recognizing and managing a rare form of angioedema: hereditary angioedema due to C1-inhibitor deficiency

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