U Specks

Summary

Affiliation: Mayo Clinic
Country: USA

Publications

  1. doi request reprint Epitope-specific anti-neutrophil cytoplasmic antibodies: do they matter? Can they be detected?
    Ulrich Specks
    Thoracic Disease Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    APMIS Suppl . 2009
  2. doi request reprint Controversies in ANCA testing
    Ulrich Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 45905, USA
    Cleve Clin J Med 79:S7-11. 2012
  3. doi request reprint Biologic agents in the treatment of granulomatosis with polyangiitis
    Ulrich Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 45905, USA
    Cleve Clin J Med 79:S50-3. 2012
  4. ncbi request reprint Recombinant human proteinase 3, the Wegener's autoantigen, expressed in HMC-1 cells is enzymatically active and recognized by c-ANCA
    U Specks
    Thoracic Diseases Research Unit, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    FEBS Lett 390:265-70. 1996
  5. ncbi request reprint Functional significance of Asn-linked glycosylation of proteinase 3 for enzymatic activity, processing, targeting, and recognition by anti-neutrophil cytoplasmic antibodies
    Ulrich Specks
    Thoracic Disease Research Unit, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    J Biochem 141:101-12. 2007
  6. ncbi request reprint ANCA subsets: influence on disease phenotype
    Ulrich Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    Cleve Clin J Med 69:SII56-9. 2002
  7. ncbi request reprint Response of Wegener's granulomatosis to anti-CD20 chimeric monoclonal antibody therapy
    U Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    Arthritis Rheum 44:2836-40. 2001
  8. ncbi request reprint Diffuse alveolar hemorrhage syndromes
    U Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA
    Curr Opin Rheumatol 13:12-7. 2001
  9. pmc What you should know about PR3-ANCA. Conformational requirements of proteinase 3 (PR3) for enzymatic activity and recognition by PR3-ANCA
    U Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA
    Arthritis Res 2:263-7. 2000
  10. ncbi request reprint Are animal models of vasculitis suitable tools?
    U Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA
    Curr Opin Rheumatol 12:11-9. 2000

Research Grants

  1. PR3-ANCA Subsets and Disease Phenotype
    Ulrich Specks; Fiscal Year: 2005

Collaborators

Detail Information

Publications46

  1. doi request reprint Epitope-specific anti-neutrophil cytoplasmic antibodies: do they matter? Can they be detected?
    Ulrich Specks
    Thoracic Disease Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    APMIS Suppl . 2009
    ..The epitope specificity of ANCA may therefore influence the functional effects of ANCA and/or may reflect the mechanisms behind different disease manifestations or disease courses...
  2. doi request reprint Controversies in ANCA testing
    Ulrich Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 45905, USA
    Cleve Clin J Med 79:S7-11. 2012
    ..Overall, ANCA is an important diagnostic and prognostic marker for small-vessel vasculitis and warrants further investigation...
  3. doi request reprint Biologic agents in the treatment of granulomatosis with polyangiitis
    Ulrich Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 45905, USA
    Cleve Clin J Med 79:S50-3. 2012
    ..The timing of re-treatment can be individualized based on patients' B-cell counts and ANCA levels in patients with chronically relapsing GPA...
  4. ncbi request reprint Recombinant human proteinase 3, the Wegener's autoantigen, expressed in HMC-1 cells is enzymatically active and recognized by c-ANCA
    U Specks
    Thoracic Diseases Research Unit, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    FEBS Lett 390:265-70. 1996
    ..This expression system for rPR3 represents a versatile tool for the analysis of its intracellular processing, structure-function relationships and interaction with autoantibodies...
  5. ncbi request reprint Functional significance of Asn-linked glycosylation of proteinase 3 for enzymatic activity, processing, targeting, and recognition by anti-neutrophil cytoplasmic antibodies
    Ulrich Specks
    Thoracic Disease Research Unit, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    J Biochem 141:101-12. 2007
    ..Finally, a capture ELISA for ANCA detection, using rPR3 glycosylation variants as target antigens, reveals that in about 20% of patients, epitope recognition by ANCA is affected by the glycosylation status of PR3...
  6. ncbi request reprint ANCA subsets: influence on disease phenotype
    Ulrich Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    Cleve Clin J Med 69:SII56-9. 2002
  7. ncbi request reprint Response of Wegener's granulomatosis to anti-CD20 chimeric monoclonal antibody therapy
    U Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    Arthritis Rheum 44:2836-40. 2001
    ..This modality warrants closer examination in a carefully conducted clinical trial...
  8. ncbi request reprint Diffuse alveolar hemorrhage syndromes
    U Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA
    Curr Opin Rheumatol 13:12-7. 2001
    ..To provide the practicing specialist with a rational diagnostic and management approach to the patient with DAH, this review summarizes the most recent publications and salient information derived from older publications...
  9. pmc What you should know about PR3-ANCA. Conformational requirements of proteinase 3 (PR3) for enzymatic activity and recognition by PR3-ANCA
    U Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA
    Arthritis Res 2:263-7. 2000
    ..The expression of recombinant PR3 has provided a better understanding of the significance of the various intracellular processing steps for enzymatic activity and recognition by PR3-ANCA...
  10. ncbi request reprint Are animal models of vasculitis suitable tools?
    U Specks
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA
    Curr Opin Rheumatol 12:11-9. 2000
    ..Even though the existing models may not exactly mirror any specific human disease, they offer reproducible, highly controlled conditions to answer specific questions about pathogenesis and novel therapeutic approaches...
  11. ncbi request reprint Differences between human proteinase 3 and neutrophil elastase and their murine homologues are relevant for murine model experiments
    Olaf Wiesner
    Thoracic Diseases Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic Foundation, Rochester, MN 55905, USA
    FEBS Lett 579:5305-12. 2005
    ..In conclusion, appropriate interpretation of experiments in murine models requires individual species-specific assessment of neutrophil protease function and inhibition...
  12. ncbi request reprint Capture-ELISA based on recombinant PR3 is sensitive for PR3-ANCA testing and allows detection of PR3 and PR3-ANCA/PR3 immunecomplexes
    J Sun
    Thoracic Diseases Research Unit, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    J Immunol Methods 211:111-23. 1998
    ..The capture ELISA can also be used to detect of PR3-ANCA immunecomplexes and, in combination with the rabbit antiserum, for the quantitative measurement of PR3 in biological fluids...
  13. ncbi request reprint Rituximab treatment of idiopathic membranous nephropathy
    F C Fervenza
    Division of Nephrology and Hypertension, Mayo Clinic, 200 First Street SW, Rochester, MN 55901, USA
    Kidney Int 73:117-25. 2008
    ..Rituximab appears effective in reducing proteinuria in some patients with idiopathic membranous nephropathy but prospective identification of responsive patients was not possible...
  14. ncbi request reprint Antineutrophil cytoplasmic antibodies reacting with the pro form of proteinase 3 and disease activity in patients with Wegener's granulomatosis and microscopic polyangiitis
    K A Russell
    Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA
    Arthritis Rheum 44:463-8. 2001
    ..It raises new questions about the primary target of the PR3 ANCA immune response in patients with small vessel vasculitis...
  15. pmc Agreement of anti-neutrophil cytoplasmic antibody measurements obtained from serum and plasma
    A S Lee
    Thoracic Disease Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    Clin Exp Immunol 146:15-20. 2006
    ..95 (P < 0.0001). Our study shows that serum and plasma samples can be used interchangeably for measuring ANCA...
  16. ncbi request reprint Part 2: Synopsis of B-lymphocyte targeted therapy of ANCA-associated vasculitis
    J M Golbin
    Division of Pulmonary and Critical Care, Mayo Clinic, Rochester, MN 55905, USA
    Clin Exp Rheumatol 25:S74-6. 2007
  17. pmc Glycosylation of proteinase 3 (PR3) is not required for its reactivity with antineutrophil cytoplasmic antibodies (ANCA) in Wegener's granulomatosis
    J D Finkielman
    Thoracic Disease Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA
    Clin Exp Rheumatol 27:S45-52. 2009
    ..The present study explores the influence of the glycosylation status of PR3 on the PR3 recognition by ANCA in a well characterized population of patients with WG...
  18. ncbi request reprint Recurrence of ANCA-associated vasculitis following renal transplantation in the modern era of immunosupression
    M Gera
    Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA
    Kidney Int 71:1296-301. 2007
    ..Potent antirejection regimes are well tolerated in these patients, are associated with a low risk of recurrence and an absence of AAV-related graft dysfunction...
  19. ncbi request reprint Are antineutrophil cytoplasmic antibodies pathogenic? Experimental approaches to understand the antineutrophil cytoplasmic antibody phenomenon
    K A Russell
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USA
    Rheum Dis Clin North Am 27:815-32, vii. 2001
    ..Taken in conjunction with clinical observations and data from animal models, a concept of the pathogenicity of ANCA emerges...
  20. ncbi request reprint Diabetes insipidus and anterior pituitary insufficiency as presenting features of Wegener's granulomatosis
    V D Garovic
    Divisions of Hypertension, Nephrology, Endocrinology, and Pulmonary Medicine and Critical Care and the Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA
    Am J Kidney Dis 37:E5. 2001
    ..Early diagnosis and treatment may be crucial in preventing pituitary gland destruction and long-term endocrine sequelae. We suggest screening for anterior pituitary failure in the presence of the WG-associated DI...
  21. ncbi request reprint Evaluation of capture ELISA for detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 in Wegener's granulomatosis: first results from a multicentre study
    Elena Csernok
    Department of Rheumatology, University of Schleswig Holstein Campus Lübeck and Rheumaklinik Bad Bramstedt, Germany
    Rheumatology (Oxford) 43:174-80. 2004
    ..To evaluate the performance characteristics of direct and capture ELISA for the detection of PR3-ANCA in Wegener's granulomatosis (WG) in international ANCA reference laboratories...
  22. doi request reprint Idiopathic membranous nephropathy: diagnosis and treatment
    Fernando C Fervenza
    Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, 200 First Street, SW, Rochester, MN 55905, USA
    Clin J Am Soc Nephrol 3:905-19. 2008
    ....
  23. ncbi request reprint Churg-Strauss syndrome: update on clinical, laboratory and therapeutic aspects
    Karina A Keogh
    Division of Pulmonary and Critical Care Medicine, Department of Medicine, Mayo Clinic College of Medicine, Rochester, MN 55905, USA
    Sarcoidosis Vasc Diffuse Lung Dis 23:3-12. 2006
    ..This review provides an update on the clinical diagnosis of Churg-Strauss syndrome in light of changing disease definitions and classifications, and focuses on evolving therapeutic approaches for this challenging systemic disorder...
  24. ncbi request reprint Melanoma antigen A4 is expressed in non-small cell lung cancers and promotes apoptosis
    Tobias Peikert
    Thoracic Diseases Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA
    Cancer Res 66:4693-700. 2006
    ..028) in 293/MAGE-A4 cells. These findings suggest that MAGE-A4 expression may promote tumor cell death, sensitize malignancies to apoptotic stimuli, such as chemotherapeutic agents, and therefore may represent a tumor suppressor protein...
  25. ncbi request reprint A novel capture-ELISA for detection of anti-neutrophil cytoplasmic antibodies (ANCA) based on c-myc peptide recognition in carboxy-terminally tagged recombinant neutrophil serine proteases
    Augustine S Lee
    Thoracic Disease Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, MN 55905, USA
    J Immunol Methods 307:62-72. 2005
    ....
  26. ncbi request reprint Sinonasal osteocartilaginous necrosis in cocaine abusers: experience in 25 patients
    Matteo Trimarchi
    Department of Otorhinolaryngology, University of Brescia, Italy
    Am J Rhinol 17:33-43. 2003
    ..Cocaine-induced lesions may cause extensive destruction of the osteocartilaginous structures of the nose, sinuses, and palate that mimics the clinical picture of other diseases...
  27. ncbi request reprint Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists
    Karina A Keogh
    Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA
    Am J Med 115:284-90. 2003
    ..To determine the association of antineutrophil cytoplasmic antibodies (ANCA) and leukotriene receptor antagonists with disease activity in a large series of patients with Churg-Strauss syndrome...
  28. ncbi request reprint A 70-year-old man with pulmonary infiltrates and a positive antineutrophil cytoplasmic autoantibody test result
    Augustine S Lee
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St, SW, Rochester, MN 55905, USA
    Chest 127:1045-50. 2005
  29. ncbi request reprint Subtle modifications of a PR3-ANCA ELISA affect assay performance
    Jason Lewis
    Clin Immunol 118:124-5. 2006
  30. ncbi request reprint Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis
    Javier D Finkielman
    Mayo Clinic, Rochester, Minnesota 55905, USA
    Ann Intern Med 147:611-9. 2007
    ..The utility of antineutrophil cytoplasmic antibody (ANCA) levels to guide the management of patients with Wegener granulomatosis remains controversial...
  31. ncbi request reprint Targeting B lymphocytes as therapy for ANCA-associated vasculitis
    Jason M Golbin
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic Foundation, 200 First Street SW, Rochester, MN 55905, USA
    Rheum Dis Clin North Am 33:741-54, v. 2007
    ..This novel approach has sparked hope for patients and physicians in their search for effective, well-tolerated therapy for AAV. B-cell depletion is now undergoing rigorous investigation in randomized clinical trials...
  32. ncbi request reprint Damage caused by Wegener's granulomatosis and its treatment: prospective data from the Wegener's Granulomatosis Etanercept Trial (WGET)
    Philip Seo
    Johns Hopkins University, Baltimore, Maryland
    Arthritis Rheum 52:2168-78. 2005
    ..To analyze damage occurring in patients with Wegener's granulomatosis (WG) enrolled in the WG Etanercept Trial (WGET) and to correlate that damage with disease activity, adverse events, and quality of life...
  33. doi request reprint Functional characterization of antineutrophil cytoplasmic antibodies in patients with cocaine-induced midline destructive lesions
    Tobias Peikert
    Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA
    Arthritis Rheum 58:1546-51. 2008
    ..We also compared the binding of PR3 ANCA in patients with CIMDL with that in patients with Wegener's granulomatosis (WG)...
  34. ncbi request reprint Antineutrophil cytoplasmic autoantibodies against the murine homolog of proteinase 3 (Wegener autoantigen) are pathogenic in vivo
    Heiko Pfister
    Department of Neuroimmunology, Max Planck Institute of Neurobiology, Am Klopferspitz 18a, D 82152 Martinsried, Germany
    Blood 104:1411-8. 2004
    ..In conclusion, our in vivo study provides the first evidence for a pathogenic effect of proteinase 3-specific ANCAs at local sites of inflammation...
  35. ncbi request reprint Different gender-associated genotype risks of Wegener's granulomatosis and microscopic polyangiitis
    Zoltán Bártfai
    Medical Hospital, Research Center Borstel, D 23845 Borstel, Germany
    Clin Immunol 109:330-7. 2003
    ..In contrast, our findings suggest a role of the enhanced IL-10 (-1082) PM in WG and MPA with a significant gender difference in MPA...
  36. ncbi request reprint Clinical utility of ANCA tests for the dermatologist
    Lawrence E Gibson
    Division of Laboratory Dermatology, Department of Laboratory Medicine and Pathology, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA
    Int J Dermatol 42:859-69. 2003
  37. ncbi request reprint Wegener's granulomatosis: survey of 701 patients in North America. Changes in outcome in the 1990s
    Nabih I Abdou
    Wegener s Granulomatosis Support Group Inc, International, Center for Rheumatic Disease, Kansas City, Missouri 64188 8660, USA
    J Rheumatol 29:309-16. 2002
    ..To study the medical and socioeconomic impact of Wegener's granulomatosis (WG) in a large cohort (n = 701) of patients who are members of the international WG Support Group (WGSG)...
  38. ncbi request reprint ANCA are detectable in nearly all patients with active severe Wegener's granulomatosis
    Javier D Finkielman
    Thoracic Disease Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, Minn 55905, USA
    Am J Med 120:643.e9-14. 2007
    ..The objective of this study was to determine the frequency of ANCA in patients with active Wegener's granulomatosis and to assess the influence of disease severity on test results...
  39. ncbi request reprint Detection of anti-neutrophil cytoplasmic antibodies under actual clinical testing conditions
    Kimberly A Russell
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA
    Clin Immunol 103:196-203. 2002
    ..1%), and C-ANCA determination using IIF, the highest specificity (99.6%). While maintaining maximal diagnostic accuracy, significant labor savings are achieved by screening for WG/MPA by ELISA followed by confirmatory IIF...
  40. ncbi request reprint Outcome of patients with small-vessel vasculitis admitted to a medical ICU
    S Anjum Khan
    Division of Pulmonary and Critical Care Medicine, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA
    Chest 131:972-6. 2007
    ..This study aims to describe the clinical course and prognostic factors of patients with small-vessel vasculitis admitted to a medical ICU...
  41. ncbi request reprint Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis
    Olaf Wiesner
    Mayo Clinic, Rochester, MN 55905, USA
    Arthritis Rheum 50:2954-65. 2004
    ..We tested the hypothesis that discrepancies in the reported frequencies of HNE ANCAs in patients with vasculitis may be related to differences in detection methods, and that HNE ANCA may be a marker for CIMDL...
  42. ncbi request reprint Effects of carboxy-terminal modifications of proteinase 3 (PR3) on the recognition by PR3-ANCA
    Stephen A Capizzi
    Thoracic Diseases Research Unit and Hematology Research Unit, Mayo Graduate School of Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA
    Kidney Int 63:756-60. 2003
    ..Carboxy-terminally modified or tagged recombinant PR3 (rPR3) molecules may be useful for the refinement of diagnostic assays and for the study of biological processes...
  43. ncbi request reprint Induction of remission by B lymphocyte depletion in eleven patients with refractory antineutrophil cytoplasmic antibody-associated vasculitis
    Karina A Keogh
    Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Arthritis Rheum 52:262-8. 2005
    ..To assess the clinical effects of rituximab therapy in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)...
  44. ncbi request reprint Churg-Strauss syndrome
    Karina A Keogh
    Division of Pulmonary and Critical Care Medicine, Department of Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA
    Semin Respir Crit Care Med 27:148-57. 2006
    ..However, the exact role of glucocorticoid-sparing immunosuppressive agents and treatment options for refractory disease remain poorly studied...
  45. ncbi request reprint Herpes zoster in immunocompromised patients: incidence, timing, and risk factors
    Peter K Wung
    Johns Hopkins University School of Medicine, Johns Hopkins University, Baltimore, MD, USA
    Am J Med 118:1416. 2005
    ..To evaluate the risk factors for herpes zoster as well as the incidence and timing of this complication in patients who were treated with immunosuppression because of active Wegener's granulomatosis...
  46. ncbi request reprint Does infection play a role in the pathogenesis of pulmonary vasculitis?
    Stephen A Capizzi
    Thoracic Diseases Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, USA
    Semin Respir Infect 18:17-22. 2003
    ..Despite significant advances, the precise connection between infections and pulmonary vasculitis remains poorly understood, and further studies into the pathogenesis of these diseases are needed...

Research Grants1

  1. PR3-ANCA Subsets and Disease Phenotype
    Ulrich Specks; Fiscal Year: 2005
    ..This study will provide new insights into the potential pathogenic role of PR3-ANCA that are not obtainable in any other way, and will constitute the most definitive study of these antibodies to date. ..