Plasma biomarkers of acute attacks in patients with angioedema due to C1-inhibitor deficiency

M. Cugno, A. Zanichelli, A. G. Bellatorre, S. Griffini, M. Cicardi

Research output: Contribution to journalArticle

Abstract

Background: Cl-inhibitor (C1-INH) deficiency leads to recurrent attacks of mucocutaneous edema and may be inherited (hereditary angioedema [HAE]) or acquired (acquired angioedema [AAE]), which have the same clinical picture characterized by angioedema involving the skin, gastrointestinal tract, and larynx. Although cutaneous swelling is evident, abdominal angioedema is still a diagnostic challenge and attacks can mimic surgical emergencies. There is currently no laboratory marker for identifying angioedema attacks. Objective: As coagulation and fibrinolysis are activated during angioedema attacks, we assessed if plasma measurements of prothrombin fragment F1 + 2 (marker of thrombin generation) and D-dimer (marker of fibrin degradation) can be useful for the diagnosis of angioedema because of C1-INH deficiency, especially in case of hidden locations as abdominal attacks. Methods: In addition to complement, we measured plasma levels of F1 + 2 and D-dimer in 28 patients with C1-INH deficiency during acute attacks and remission, 35 patients without C1-INH deficiency during abdominal colics, and 20 healthy subjects. Results: Plasma F1 + 2 levels were higher in patients with C1-INH deficiency during remission than in healthy controls (P = 0.001), and further increased during cutaneous and abdominal attacks (P = 0.0001); patients without C1-INH deficiency had normal F1 + 2 levels during abdominal colics. Plasma D-dimer levels were higher in patients with C1-INH deficiency during remission than in controls (P = 0.012) and increased during angioedema attacks, reaching higher levels than in patients without C1-INH deficiency during colics (P = 0.002). Conclusions: During acute angioedema attacks, patients with C1-INH deficiency have high prothrombin fragment F1 + 2 and D-dimer levels, the measurement of which may have an important diagnostic value.

Original languageEnglish
Pages (from-to)254-257
Number of pages4
JournalAllergy: European Journal of Allergy and Clinical Immunology
Volume64
Issue number2
DOIs
Publication statusPublished - Feb 2009

Keywords

  • Angioedema
  • C1-inhibitor
  • Complement
  • D-dimer
  • Prothrombin fragment F1 + 2

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

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