Plasma biomarkers of acute attacks in patients with angioedema due to C1‐inhibitor deficiency
- 21 January 2009
- Vol. 64 (2), 254-257
- https://doi.org/10.1111/j.1398-9995.2008.01859.x
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.Keywords
This publication has 13 references indexed in Scilit:
- Diagnostic Strategies for Excluding Pulmonary Embolism in Clinical Outcome StudiesAnnals of Internal Medicine, 2003
- Increased vascular permeability in C1 inhibitor–deficient mice mediated by the bradykinin type 2 receptorJCI Insight, 2002
- Plasma bradykinin in angio-oedemaThe Lancet, 1998
- Activation of the contact system and fibrinolysis in autoimmune acquired angioedema: A rationale for prophylactic use of tranexamic acidJournal of Allergy and Clinical Immunology, 1994
- Hereditary and Acquired C1-Inhibitor DeficiencyMedicine, 1992
- Plasma levels of C1- inhibitor complexes and cleaved C1- inhibitor in patients with hereditary angioneurotic edema.JCI Insight, 1990
- The metabolism of C1 inhibitor and C1q in patients with acquired C1-inhibitor deficiencyJournal of Allergy and Clinical Immunology, 1986
- Prekallikrein Activation and High-Molecular-Weight Kininogen Consumption in Hereditary AngioedemaNew England Journal of Medicine, 1983
- Detection of active kallikrein in induced blister fluids of hereditary angioedema patients.The Journal of Experimental Medicine, 1980
- Hereditary angioneurotic edema: II. Deficiency of inhibitor for serum globulin permeability factor and/or plasma kallikreinJournal of Allergy, 1962