Studies in Scleroderma and Cystic Fibrosis Osteoporosis

Summary

Principal Investigator: Peter Merkel
Abstract: To provide support for research projects in the field of rheumatology that will form the basis of a structured mentoring program for young investigators pursuing careers in patient-oriented clinical research. This application has two major specific research aims: 1) Develop new outcome measures for skin assessment in scleroderma for use in clinical trials and 2) Determine the prevalence and progression of osteoporosis in patients with cystic fibrosis (CF). Both projects will have direct relevance to furthering the health of the populations under study. Clinical research in scleroderma, including therapeutic trials, is greatly hampered by a lack of reliable and precise outcome measurements of disease activity. Skin thickening and fibrosis are major causes of morbidity and dysfunction for patients with scleroderma. The great success in extending the life expectancy of patients with CF gained in the last 20 years has resulted in patients now experiencing diseases as adults not formerly encountered in this population. Among these diseases is osteoporosis. Patients with CF appear to be at high risk for osteoporosis due to nutritional, pharmacologic, and genetic factors but the pathophysiology and extent of the problem is not known. Patients with scleroderma will be followed prospectively and evaluated for skin disease activity by skin scoring, durometer readings (thickness), light-based technologies, skin biopsies, self-assessments, and functional status instruments. These data will be analyzed to determine a core set of outcome measures for scleroderma. and validated by an expert panel of national researchers in this disease. An observational cohort of patients with CF will be studied. Baseline and 2-year measurements of bone density, nutritional status, and biochemical markers of bone turnover will performed. A comprehensive program for training new clinical investigators by the principal investigator is proposed. This program includes trainees taking an active and integral role in the research studies described. Additionally, trainees will be enrolled in formal coursework in biostatistics, epidemiology, and clinical research techniques leading to a master degree. A unique seminar and a series of support services at the host institution will further complement the training program.
Funding Period: 2001-07-18 - 2007-06-30
more information: NIH RePORT

Top Publications

  1. pmc Recombinant human relaxin in the treatment of systemic sclerosis with diffuse cutaneous involvement: a randomized, double-blind, placebo-controlled trial
    Dinesh Khanna
    David Geffen School of Medicine, and School of Public Health, University of California, Los Angeles, USA
    Arthritis Rheum 60:1102-11. 2009
  2. pmc Alpha₁-antitrypsin deficiency-related alleles Z and S and the risk of Wegener's granulomatosis
    Alfred D Mahr
    Boston University School of Medicine, Boston, Massachusetts, USA
    Arthritis Rheum 62:3760-7. 2010
  3. pmc Patient-reported outcome assessment in vasculitis may provide important data and a unique perspective
    Karen Herlyn
    University Hospital Schleswig Holstein, Lubeck, Germany
    Arthritis Care Res (Hoboken) 62:1639-45. 2010
  4. ncbi Brief report: The value of a patient global assessment of disease activity in granulomatosis with polyangiitis (Wegener's)
    Gunnar Tomasson
    University of Iceland, Reykjavik, Iceland
    Arthritis Rheumatol 66:428-32. 2014
  5. ncbi Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part I
    Charles G Helmick
    CDC, Atlanta, Georgia 30341 3717, USA
    Arthritis Rheum 58:15-25. 2008
  6. ncbi Effects of glucocorticoids on weight change during the treatment of Wegener's granulomatosis
    Peter K Wung
    Johns Hopkins University School of Medicine, Baltimore, MD, USA
    Arthritis Rheum 59:746-53. 2008
  7. pmc Patterns and predictors of change in outcome measures in clinical trials in scleroderma: an individual patient meta-analysis of 629 subjects with diffuse cutaneous systemic sclerosis
    P A Merkel
    Boston University, Boston, Massachusetts, USA
    Arthritis Rheum 64:3420-9. 2012
  8. pmc Meta-analysis of genetic polymorphisms in granulomatosis with polyangiitis (Wegener's) reveals shared susceptibility loci with rheumatoid arthritis
    Sharon A Chung
    University of California, San Francisco, CA, USA
    Arthritis Rheum 64:3463-71. 2012
  9. ncbi Serum proteins reflecting inflammation, injury and repair as biomarkers of disease activity in ANCA-associated vasculitis
    Paul A Monach
    Vasculitis Center, Section of Rheumatology, and the Clinical Epidemiology Unit, Boston University School of Medicine, Boston, MA, USA
    Ann Rheum Dis 72:1342-50. 2013
  10. ncbi Association of granulomatosis with polyangiitis (Wegener's) with HLA-DPB1*04 and SEMA6A gene variants: evidence from genome-wide analysis
    Gang Xie
    Mount Sinai Hospital and University of Toronto, Toronto, Ontario, Canada
    Arthritis Rheum 65:2457-68. 2013

Scientific Experts

  • Peter Merkel
  • Ravi Suppiah
  • Paul A Monach
  • D Khanna
  • Eugene Y Kissin
  • C M Gordon
  • DANIEL ERIC FURST
  • John Stone
  • M Walsh
  • A D Mahr
  • Christopher P Denton
  • Gary S Hoffman
  • Ulrich Specks
  • E William St Clair
  • Robert Spiera
  • John C Davis
  • Philip Seo
  • W Joseph McCune
  • Steven R Ytterberg
  • Gunnar Tomasson
  • Nancy B Allen
  • Carol A Langford
  • Jodi K Sebastian
  • Javier D Finkielman
  • Simon Carette
  • Gang Xie
  • Sharon Farkas
  • Jobert Barin
  • David Blumenthal
  • Aileen Schiller
  • Sonya L Crook
  • Billie Jo Coomer
  • Kimberly Strom
  • Michael J Regan
  • Susan Fisher
  • David B Hellmann
  • Kristin Bradt
  • Ana Morrel-Samuels
  • Nancy Min
  • Steve Lund
  • Amanda M Moore
  • Kathy Mieras
  • Curtis L Meinert
  • Mark Van Natta
  • Janet T Holbrook
  • Noel R Rose
  • Maureen Fitzpatrick
  • Ken Fye
  • Paul L Canner
  • Rosanne Berman
  • Jessica Donithan
  • Kimberly Carlson
  • Doyt L Conn
  • Hilary Haftel
  • Barbara Gilson
  • Boleyn Hammel
  • Tiffany Clark
  • Laurel Murrow
  • Lourdes Pinachos
  • Sudhakar Sridharan
  • Karen Rodin
  • Monica Talor
  • Melynn Nuite
  • Sandra Neckel
  • Leonard H Calabrese
  • John Dodge
  • Steven Ytterberg
  • Jacki Smith
  • Rondi Gelbard
  • J Richard Landis
  • William Wilke
  • Edna Scarlett
  • Debora Bork
  • Misty L Uhlfelder
  • Sandy Enuha
  • C Lynne Burek
  • Karen Herlyn
  • Tobias Peikert
  • Andrea K Tibbs
  • Peter C Grayson
  • E M Miloslavsky
  • David Cuthbertson
  • Delnaz Roshandel
  • Robert F Spiera
  • Nader A Khalidi
  • Jeffrey C Edberg
  • Paul F Dellaripa
  • Richard Sherva
  • Katherine A Siminovitch
  • Sharon A Chung

Detail Information

Publications46

  1. pmc Recombinant human relaxin in the treatment of systemic sclerosis with diffuse cutaneous involvement: a randomized, double-blind, placebo-controlled trial
    Dinesh Khanna
    David Geffen School of Medicine, and School of Public Health, University of California, Los Angeles, USA
    Arthritis Rheum 60:1102-11. 2009
    ....
  2. pmc Alpha₁-antitrypsin deficiency-related alleles Z and S and the risk of Wegener's granulomatosis
    Alfred D Mahr
    Boston University School of Medicine, Boston, Massachusetts, USA
    Arthritis Rheum 62:3760-7. 2010
    ..This study investigated the relationship of the α(1) AT deficiency Z and S alleles with the risk of developing WG in a large cohort...
  3. pmc Patient-reported outcome assessment in vasculitis may provide important data and a unique perspective
    Karen Herlyn
    University Hospital Schleswig Holstein, Lubeck, Germany
    Arthritis Care Res (Hoboken) 62:1639-45. 2010
    ..Outcome assessment in vasculitis primarily focuses on physician evaluations of disease activity and damage. Little is known about the patients' perspectives regarding the burden of disease...
  4. ncbi Brief report: The value of a patient global assessment of disease activity in granulomatosis with polyangiitis (Wegener's)
    Gunnar Tomasson
    University of Iceland, Reykjavik, Iceland
    Arthritis Rheumatol 66:428-32. 2014
    ....
  5. ncbi Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part I
    Charles G Helmick
    CDC, Atlanta, Georgia 30341 3717, USA
    Arthritis Rheum 58:15-25. 2008
    ..A companion article (part II) addresses additional conditions...
  6. ncbi Effects of glucocorticoids on weight change during the treatment of Wegener's granulomatosis
    Peter K Wung
    Johns Hopkins University School of Medicine, Baltimore, MD, USA
    Arthritis Rheum 59:746-53. 2008
    ..Weight gain is a side effect of glucocorticoid (GC) use, but the natural history and health implications of changes in weight that occur during the treatment of inflammatory disease are not understood...
  7. pmc Patterns and predictors of change in outcome measures in clinical trials in scleroderma: an individual patient meta-analysis of 629 subjects with diffuse cutaneous systemic sclerosis
    P A Merkel
    Boston University, Boston, Massachusetts, USA
    Arthritis Rheum 64:3420-9. 2012
    ..To examine the range and responsiveness to change of clinical outcome measures and study predictors of clinical response in patients with diffuse cutaneous systemic sclerosis (dcSSc) in the context of clinical trials...
  8. pmc Meta-analysis of genetic polymorphisms in granulomatosis with polyangiitis (Wegener's) reveals shared susceptibility loci with rheumatoid arthritis
    Sharon A Chung
    University of California, San Francisco, CA, USA
    Arthritis Rheum 64:3463-71. 2012
    ....
  9. ncbi Serum proteins reflecting inflammation, injury and repair as biomarkers of disease activity in ANCA-associated vasculitis
    Paul A Monach
    Vasculitis Center, Section of Rheumatology, and the Clinical Epidemiology Unit, Boston University School of Medicine, Boston, MA, USA
    Ann Rheum Dis 72:1342-50. 2013
    ..To identify circulating proteins that distinguish between active anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and remission in a manner complementary to markers of systemic inflammation...
  10. ncbi Association of granulomatosis with polyangiitis (Wegener's) with HLA-DPB1*04 and SEMA6A gene variants: evidence from genome-wide analysis
    Gang Xie
    Mount Sinai Hospital and University of Toronto, Toronto, Ontario, Canada
    Arthritis Rheum 65:2457-68. 2013
    ..To identify genetic determinants of granulomatosis with polyangiitis (Wegener's) (GPA)...
  11. ncbi Clinical outcomes of remission induction therapy for severe antineutrophil cytoplasmic antibody-associated vasculitis
    E M Miloslavsky
    Massachusetts General Hospital, Boston
    Arthritis Rheum 65:2441-9. 2013
    ....
  12. ncbi Efficacy of remission-induction regimens for ANCA-associated vasculitis
    Ulrich Specks
    Mayo Clinic Foundation, Rochester, MN, USA
    N Engl J Med 369:417-27. 2013
    ....
  13. ncbi New features of disease after diagnosis in 6 forms of systemic vasculitis
    Peter C Grayson
    From The Vasculitis Center, Section of Rheumatology, and the Clinical Epidemiology Unit, Boston University School of Medicine, Boston, Massachusetts Department of Biostatistics, University of South Florida, Tampa, Florida Division of Rheumatology, Mount Sinai Hospital, Toronto, Ontario and the Center for Vasculitis Care and Research, Cleveland Clinic, Cleveland, Ohio Division of Rheumatology, McMaster University, Hamilton, Ontario, Canada Division of Rheumatology, University of Utah, Salt Lake City, Utah Division of Rheumatology, University of Pittsburgh Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester Division of Rheumatology, Mayo Clinic College of Medicine, Rochester, Minnesota and Division of Rheumatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA
    J Rheumatol 40:1905-12. 2013
    ..To quantify the occurrence of features of vasculitis that initially present after diagnosis in 6 types of primary vasculitis...
  14. pmc Effects of duration of glucocorticoid therapy on relapse rate in antineutrophil cytoplasmic antibody-associated vasculitis: A meta-analysis
    Michael Walsh
    Addenbrooke s Hospital, Cambridge, UK
    Arthritis Care Res (Hoboken) 62:1166-73. 2010
    ..The role of low-dose glucocorticoids (GC) in relapse prevention is controversial. We undertook a systematic review and meta-analysis to determine if GC target doses influence relapses of AAV...
  15. pmc Rituximab versus cyclophosphamide for ANCA-associated vasculitis
    John H Stone
    Massachusetts General Hospital, Boston, USA
    N Engl J Med 363:221-32. 2010
    ..Uncontrolled studies suggest that rituximab is effective and may be safer than a cyclophosphamide-based regimen...
  16. pmc Relationship between markers of platelet activation and inflammation with disease activity in Wegener's granulomatosis
    Gunnar Tomasson
    Boston University BU School of Medicine, and Massachusetts General Hospital, Boston, Massachusetts, USA
    J Rheumatol 38:1048-54. 2011
    ..Our objective was to determine whether measures of platelet activation or inflammation are associated with disease activity in Wegener's granulomatosis (WG)...
  17. ncbi A model to predict cardiovascular events in patients with newly diagnosed Wegener's granulomatosis and microscopic polyangiitis
    Ravi Suppiah
    Nuffield Orthopaedic Centre, Oxford, UK
    Arthritis Care Res (Hoboken) 63:588-96. 2011
    ..To create a prognostic tool to quantify the 5-year cardiovascular (CV) risk in patients with newly diagnosed Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) without premorbid CV disease...
  18. pmc Solid malignancies among etanercept-treated patients with granulomatosis with polyangiitis (Wegener's): long-term followup of a multicenter longitudinal cohort
    Francisco Silva
    Mayo Clinic, Rochester, Minnesota, USA
    Arthritis Rheum 63:2495-503. 2011
    ..The present study was conducted to determine the malignancy risk beyond the time of exposure to study therapy...
  19. pmc Assessment of health-related quality of life as an outcome measure in granulomatosis with polyangiitis (Wegener's)
    Gunnar Tomasson
    Boston University, Boston, Massachusetts, USA
    Arthritis Care Res (Hoboken) 64:273-9. 2012
    ..To assess a generic measure of health-related quality of life (HRQOL) as an outcome measure in granulomatosis with polyangiitis (Wegener's) (GPA)...
  20. pmc Circulating angiopoietin-2 as a biomarker in ANCA-associated vasculitis
    Paul A Monach
    Section of Rheumatology, Department of Medicine, School of Medicine, Vasculitis Center, Boston University, Boston, Massachusetts, United States of America
    PLoS ONE 7:e30197. 2012
    ..Thus, Ang-2 appears to have limited practical value in AAV as a biomarker of disease activity at time of measurement or for predicting future activity...
  21. pmc Circulating markers of vascular injury and angiogenesis in antineutrophil cytoplasmic antibody-associated vasculitis
    Paul A Monach
    Boston University School of Medicine, Boston, Massachusetts 02118, USA
    Arthritis Rheum 63:3988-97. 2011
    ..To identify biomarkers that distinguish between active antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and remission in a manner superior or complementary to established markers of systemic inflammation...
  22. pmc Nutrient status of adults with cystic fibrosis
    Catherine M Gordon
    Division of Endocrinology, Children s Hospital Boston, Boston, MA 22015, USA
    J Am Diet Assoc 107:2114-9. 2007
    ..These findings call into question the applicability of established nutrient thresholds for patients with cystic fibrosis...
  23. ncbi Myofibroblasts and hyalinized collagen as markers of skin disease in systemic sclerosis
    Eugene Y Kissin
    Boston University Medical Center, Boston, Massachusetts, USA
    Arthritis Rheum 54:3655-60. 2006
    ..To investigate the correlation between the degree of dermal fibrosis and myofibroblast infiltration using clinical assessments of skin thickness and hardness in systemic sclerosis (SSc)...
  24. 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...
  25. pmc Comparison of disease activity measures for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis
    P A Merkel
    Vasculitis Center, E5, Boston University School of Medicine, 72 East Concord Street, Boston, Massachussets, 02118, USA
    Ann Rheum Dis 68:103-6. 2009
    ..An exercise comparing six different vasculitis instruments was performed...
  26. ncbi Infliximab for maintenance of glucocorticosteroid-induced remission of giant cell arteritis: a randomized trial
    Gary S Hoffman
    Center for Vasculitis Care and Research, The Cleveland Clinic Foundation, Lerner College of Medicine, Cleveland, Ohio 44195, USA
    Ann Intern Med 146:621-30. 2007
    ..Tumor necrosis factor-alpha is present in arteries in giant cell arteritis...
  27. ncbi Antiendothelial cell antibodies in patients with Wegener's granulomatosis: prevalence and correlation with disease activity and manifestations
    Jodi K Sebastian
    Boston University, Boston, Massachusetts 02118, USA
    J Rheumatol 34:1027-31. 2007
    ..We examined a large, well-characterized cohort of patients with WG and active disease for the presence of AECA...
  28. ncbi Part 1: The need for novel treatment regimens for ANCA-associated vasculitis
    P A Merkel
    Boston University School of Medicine, Boston, MA 02118, USA
    Clin Exp Rheumatol 25:S72-3. 2007
  29. ncbi Recombinant human anti-transforming growth factor beta1 antibody therapy in systemic sclerosis: a multicenter, randomized, placebo-controlled phase I/II trial of CAT-192
    Christopher P Denton
    Royal Free Hospital, London, UK
    Arthritis Rheum 56:323-33. 2007
    ..To evaluate CAT-192, a recombinant human antibody that neutralizes transforming growth factor beta1 (TGFbeta1), in the treatment of early-stage diffuse cutaneous systemic sclerosis (dcSSc)...
  30. ncbi Durometry for the assessment of skin disease in systemic sclerosis
    Eugene Y Kissin
    Boston University School of Medicine, Boston, Massachusetts 02118, USA
    Arthritis Rheum 55:603-9. 2006
    ..To examine the validity of a durometer to objectively measure skin hardness in systemic sclerosis (SSc), and to compare digital durometry with the modified Rodnan skin score (MRSS) and ultrasonography...
  31. ncbi Epidemiology of Wegener's granulomatosis: Lessons from descriptive studies and analyses of genetic and environmental risk determinants
    A D Mahr
    Section of Rheumatology and the Clinical Epidemiology Unit, Boston University School of Medicine, Boston, MA 02118, USA
    Clin Exp Rheumatol 24:S82-91. 2006
    ..This and many other questions remain to be answered by future structured epidemiological research. This review focuses on the current knowledge of descriptive epidemiology and genetic and environmental factors associated with WG...
  32. ncbi Solid malignancies among patients in the Wegener's Granulomatosis Etanercept Trial
    John H Stone
    Johns Hopkins University, Baltimore, Maryland, USA
    Arthritis Rheum 54:1608-18. 2006
    ..This study was undertaken to further explore the potential association between anti-TNF therapy and the development of malignancy in these patients...
  33. ncbi Responsiveness of the SF-36 and the Health Assessment Questionnaire Disability Index in a systemic sclerosis clinical trial
    Dinesh Khanna
    Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, Los Angeles, California, USA
    J Rheumatol 32:832-40. 2005
    ....
  34. ncbi Systemic sclerosis - continuing progress in developing clinical measures of response
    Daniel Furst
    University of California at Los Angeles, Los Angeles, California 90025, USA
    J Rheumatol 34:1194-200. 2007
    ..Another Delphi exercise is designed to develop consensus regarding a combined SSc response index to be validated in future RCT...
  35. ncbi Outcome measures in systemic sclerosis: an update on instruments and current research
    Dinesh Khanna
    Division of Immunology, Department of Internal Medicine, University of Cincinnati, PO Box 670563, Cincinnati, OH 45267 0563, USA
    Curr Rheumatol Rep 9:151-7. 2007
    ..This review provides an update on currently used outcome measures in SSc with specific attention on newer tools and also describes methodology under development...
  36. ncbi The future of damage assessment in vasculitis
    Philip Seo
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    J Rheumatol 34:1357-71. 2007
    ..This initiative is part of a larger international effort to create a unified approach to disease assessment for the primary systemic vasculitides...
  37. ncbi A multicenter, randomized, double-blind, placebo-controlled trial of oral type I collagen treatment in patients with diffuse cutaneous systemic sclerosis: I. oral type I collagen does not improve skin in all patients, but may improve skin in late-phase di
    Arnold E Postlethwaite
    University of Tennessee, Memphis, and VAMC, Memphis
    Arthritis Rheum 58:1810-22. 2008
    ..To investigate the safety and efficacy of oral bovine type I collagen (CI) treatment in patients who have had diffuse cutaneous systemic sclerosis (dcSSc; scleroderma) for <or=10 years...
  38. pmc Assessment of the item selection and weighting in the Birmingham vasculitis activity score for Wegener's granulomatosis
    Alfred D Mahr
    Boston University, Boston, Massachusetts 02118, USA
    Arthritis Rheum 59:884-91. 2008
    ..To assess the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) with respect to its selection and weighting of items...
  39. pmc Validity, reliability, and feasibility of durometer measurements of scleroderma skin disease in a multicenter treatment trial
    Peter A Merkel
    Boston University School of Medicine, Boston, MA 02118, USA
    Arthritis Rheum 59:699-705. 2008
    ..To determine the validity, reliability, and feasibility of durometer measurements of skin hardness as an outcome measure in clinical trials of scleroderma...
  40. ncbi 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...
  41. ncbi The frequency of anticardiolipin antibodies and genetic mutations associated with hypercoagulability among patients with Wegener's granulomatosis with and without history of a thrombotic event
    Jodi K Sebastian
    Boston University School of Medicine, Boston, Massachusetts 02118, USA
    J Rheumatol 34:2446-50. 2007
    ..The frequency of anticardiolipin antibodies (aCL), anti-beta2-glycoprotein antibodies (anti-beta2-GP), and several genetic hypercoagulable factors were examined in a large cohort of patients with WG...
  42. pmc Development of a provisional core set of response measures for clinical trials of systemic sclerosis
    D Khanna
    Division of Rheumatology, Department of Medicine, David Geffen School of Medicine, University of California at Los Angeles, California 90095, USA
    Ann Rheum Dis 67:703-9. 2008
    ..To develop a provisional core set of response measures for clinical trials of systemic sclerosis (SSc)...
  43. ncbi Adjunctive methotrexate for treatment of giant cell arteritis: an individual patient data meta-analysis
    Alfred D Mahr
    Vasculitis Center, Boston University School of Medicine, 75 Albany Street, Boston, MA 02118, USA
    Arthritis Rheum 56:2789-97. 2007
    ..To reevaluate the efficacy and safety of adjunctive low-dose methotrexate (MTX) in giant cell arteritis (GCA)...
  44. ncbi 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...
  45. ncbi Brief communication: high incidence of venous thrombotic events among patients with Wegener granulomatosis: the Wegener's Clinical Occurrence of Thrombosis (WeCLOT) Study
    Peter A Merkel
    Boston University School of Medicine, Vasculitis Center, E 5, 715 Albany Street, Boston, MA 02118, USA
    Ann Intern Med 142:620-6. 2005
    ..Venous thrombotic events (VTEs) have been observed in Wegener granulomatosis, but the incidence rate is not known...