ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases

Manfred Rieger, Pier Mannuccio Mannucci, Johanna A. Kremer Hovinga, Andrea Herzog, Gabi Gerstenbauer, Christian Konetschny, Klaus Zimmermann, Inge Scharrer, Flora Peyvandi, Miriam Galbusera, Giuseppe Remuzzi, Martina Böhm, Barbara Plaimauer, Bernhard Lämmle, Friedrich Scheiflinger

Research output: Contribution to journalArticlepeer-review


Autoantibodies neutralizing human ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif), the metalloprotease that physiologically cleaves von Willebrand factor, are a major cause of severe deficiency of the protease and of acquired thrombotic thrombocytopenic purpura (TTP). We evaluated prevalence of anti-ADAMTS13 antibodies in 59 patients with thrombotic microangiopathies (TMAs) and in 160 patients with immunologic or thrombocytopenic diseases different from TTP, using an enzyme-linked immunosorbent assay (ELISA). Immunoglobulin G (IgG) antibodies directed against ADAMTS13 were found in 97% of untreated patients with acute acquired TMA who had plasma levels of ADAMTS13 activity below 10%. The corresponding prevalence of IgM antibodies was 11%. In contrast, anti-ADAMTS13 antibodies of G or M isotypes were detected in 20% of patients with TMA with ADAMTS13 activity above 10%. The ELISA was more sensitive than the standard functional inhibitor assay for detecting antibodies against ADAMTS13. Patients with thrombocytopenia from various causes (n = 50), systemic lupus erythematosus (SLE; n = 40), and the antiphospholipid antibody syndrome (APS; n = 55) had prevalences of IgG antibodies of 8%, 13%, and 5% respectively, only slightly higher than the prevalence in 111 healthy donors (4%). A rather high prevalence of anti-ADAMTS13 IgM antibodies was found in patients with SLE and APS (18% each). The clinical significance of IgM antibodies in these groups is unclear. In conclusion, the ELISA method detected anti-ADAMTS13 IgG antibodies in a very large proportion of patients with acquired TMA associated with severe ADAMTS13 deficiency, and was more sensitive than the inhibitor assay.

Original languageEnglish
Pages (from-to)1262-1267
Number of pages6
Issue number4
Publication statusPublished - Aug 15 2005

ASJC Scopus subject areas

  • Hematology


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