Abstract
IntroductionLaryngeal oedema caused by acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a life-threatening condition. The swelling is bradykinin mediated and will not respond to the usual treatment with antihistamines, corticosteroids, or epinephrine. Instead, kallikrein-bradykinin-targeted therapies should be used promptly to prevent asphyxiation.Case presentationA 43 years old female presented at the Hereditary Angioedema Centre reporting a one-year history of peripheral, facial, and neck oedema. Treatment with antihistamines and corticosteroids had been ineffective. Laboratory results showed complement level deficiencies and monoclonal gammopathy characterised as immunoglobulin M. An abdominal ultrasound revealed splenomegaly. A bone marrow biopsy was normal. Based on these data, the diagnosis of C1-INH-AAE associated with monoclonal gammopathy of uncertain significance (MGUS) was made. As C1-INH-AAE can present with life-threatening, standard treatment-resistant laryngeal oedema, an emergency care treatment plan was proposed, and the patient was advised to present to the emergency department (ED) with this medical letter. Based on these recommendations, three laryngeal attacks were successfully treated in the ED with recombinant human C1-inhibitor (two attacks) and fresh frozen plasma (one attack). After these episodes, the patient was prescribed prophylactic treatment with antifibrinolytics. No further angioedema attacks were reported by the patient at the 18 months follow-up visit.ConclusionsBecause angioedema of the upper airways is a life-threatening condition, recognising the specific type of swelling by the emergency physician is critical in providing immediate and effective treatment to reduce the associated risk of asphyxiation. C1-INH-AAE being a rare disorder, patients should have available an emergency care treatment plan with recommendations of acute treatment possibilities.
Highlights
Laryngeal oedema caused by acquired angioedema due to C1-inhibitor deficiency (C1-INH-Acquired angioedema (AAE)) is a life-threatening condition
Identifying the triggering mediator of the swelling may be difficult especially in the emergency department (ED), where failure to discriminate between different types of angioedema may prevent the effective treatment of the less common conditions [2, 3]
When seeing recurrent episodes of non-itching, nonpitting cutaneous or mucous membranes swelling in patients forty years of age or older, having no known relatives with angioedema, clinicians should contemplate a diagnosis of an acquired form of C1-INH deficiency
Summary
Angioedema manifests as a swelling of the skin or mucosa. It is considered a consequence of a sudden local increase in vascular permeability with histamine, bradykinin, and other mediators. Oedema usually lasted one to two days She reported having a facial and external-neck swelling episode and colicky abdominal pain episodes with nausea and vomiting, after tooth extraction. The patient was given neither angiotensinconverting enzyme inhibitor nor angiotensin receptor blocking medication She had no family history of angioedema and denied having any drug or food allergies. For this attack, two vials of recombinant human C1INH replacement therapy, rhC1-INH (Conestat alfa, Pharming Group NV – Leiden, Netherlands), 2100 IU/ vial, were administered by intravenous injection on 18th January 2019, based on the accompanying medical letter’s recommendation. As prophylaxis against further angioedema attacks, the patient was prescribed prophylactic treatment with antifibrinolytics, Exacyl (Sanofi-Aventis Sp, Rzeszow, Poland), 500mg, in a dose of 3g per day (3x2 tablets per day), administered orally from 2nd April. No further attacks were reported at the eighteenmonth follow-up visit
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