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Blood, 15 August 2005, Vol. 106, No. 4, pp. 1262-1267.
Prepublished online as a Blood First Edition Paper on May 17, 2005May 12, 2005; DOI 10.1182/blood-2004-11-4490.
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Submitted November 30, 2004
Accepted April 11, 2005
ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases
Manfred Rieger, Pier M Mannucci, Johanna A Hovinga, Andrea Herzog, Gabi Gerstenbauer, Christian Konetschny, Klaus Zimmermann, Inge Scharrer, Flora Peyvandi, Miriam Galbusera, Giuseppe Remuzzi, Martina Bohm, Barbara Plaimauer, Bernhard Lammle, and Friedrich Scheiflinger*
Baxter BioScience, Biomedical Research Center, Orth, Austria
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Department of Internal Medicine and Dermatology, Maggiore Hospital, Mangiagalli and Regina Elena IRCCS Foundation and University of Milan, Milan, Italy
Department of Hematology and Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland
Department of Hemostaseology, University Hospital, Frankfurt, Germany
Mario Negri Institute for Pharmacological Research, Bergamo, Italy
Mario Negri Institute for Pharmacological Research, Bergamo, Italy; Division of Nephrology and Dialysis, Ospedali Riuniti di Bergamo, Bergamo, Italy
* Corresponding author; email: scheiff{at}baxter.com.
Autoantibodies neutralizing human ADAMTS13, 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 surveyed the prevalence of anti-ADAMTS13 antibodies in thrombotic microangiopathies (TMAs) and in other diseases using a recently developed enzyme-linked immunosorbent assay (ELISA). We found IgG antibodies directed against ADAMTS13 associated with ADAMTS13 activities below 10% in 97% of patients with acquired TMAs receiving no treatment and in 81% of patients with TMA receiving plasma treatment. The corresponding prevalences of IgM antibodies were 11% and 0%. In addition, anti-ADAMTS13 antibodies of G or M isotypes were detected in only 20% of patients with TMAs associated with ADAMTS13 activities above 10%. The prevalence of IgG antibodies in patients with systemic lupus erythematosus (SLE, n=40), with antiphospholipid antibody syndrome (APS, n=55) and with thrombocytopenia from various causes (n=50), was 12.5%, 5.5% and 8%, respectively. These are only slightly higher than the prevalence in 111 healthy donors (4%) but we found a relatively high prevalence of anti-ADAMTS13 IgM antibodies in patients with SLE and APS (30 to 33%). However, the clinical significance of IgM antibodies in these patient groups is unclear. In conclusion, the ELISA method detected anti-ADAMTS13 IgG antibodies in a large proportion of patients with acquired TMAs associated with severe ADAMTS13 deficiency, and was more sensitive than the inhibitor assay.
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