Anca-associated Vasculitides: Immunological And Clinical Aspects (advances In Experimental Medicine And Biology)

Preface: Pathophysiological Athophysiological Aspects and Antigen Specificity: cANCA-Targets. Proteinase 3 etc. pANCA-Targets: a. Myeloperoxidase. pANCA-Targets: b. Other Lysosomal Antigens. ANCA-Targets: Endothelial Cell Elements. Animal Studies and Animal Models. Clinical aspects of Wegener's Granulomatosis and other ANCA Associated Diseases: Wegener's and ANCA Associated Vasculitis. Therapeutic Strategies. ANCA and Inflamatory Bowel and Liver Diseases: Gastroenterologic Aspects. 100 articles. Index. Front Matter....Pages i-xix Friedrich Wegener and the Nature of Fame....Pages 1-4 Front Matter....Pages 5-5 Pathogenic Potential of Anti-Neutrophil Cytoplasmic Autoantibodies....Pages 7-15 Neutrophilic Leukocyte Granules: From Structure to Function....Pages 17-33 Pathogenetic Aspects of Autoantibodies to Endothelial Cells in Systemic Vasculitis....Pages 35-44 Membrane Surface Proteinase 3 Expression and Intracytoplasmic Immunoglobulin on Neutrophils from Patients with ANCA-Associated Vasculitides....Pages 45-50 A New Approach to the Molecular Characterization of the C-ANCA Antigen in Wegener’s Granulomatosis....Pages 51-54 Proteinase 3: Substrate Specificity and Possible Pathogenetic Effect of Wegener’s Granulomatosis Autoantibodies (C-ANCA) by Dysregulation of the Enzyme....Pages 55-60 Inhibition of Proteinase 3 Activity by Peptides Derived from Human Epidermis....Pages 61-64 Implications of c-ANCA Testing for the Classification of Wegener’s Granulomatosis: Performance of Different Detection Systems....Pages 65-70 ANCA and IgG Subclasses....Pages 71-75 Anti-MPO Antibody (Id) and Autoantibody to Purified Anti-MPO F(Ab)2 (Anti-Id)....Pages 77-80 Cross-Reactivity between Antibodies to Thyroid Microsomal Antigens and Myeloperoxidase....Pages 81-85 Antineutrophil Cytoplasmic Autoantibodies (ANCA) Recognizing a Recombinant Myeloperoxidase Subunit....Pages 87-92 Pooled Human IgG (PHIG) Inhibits the Binding of Anti-Myeloperoxidase Antibodies to Myeloperoxidase....Pages 93-96 Studies to Demonstrate Inhibition of Functional Activity of Neutrophil Lysosomal Enzymes with ANCA....Pages 97-100 Lactoferrin Co-Purifies with Myeloperoxidase and is Recognised by Anti-Neutrophil Cytoplasm Antibodies....Pages 101-104 ANCA Specificities for HL-60 and Neutrophil Constituents....Pages 105-108 In Vitro Interactions of C-ANCA (Antibodies to Proteinase 3) with Human Endothelial Cells....Pages 109-113 Anti-Neutrophil Cytoplasm Antibodies (ANCA) Increase Neutrophil Adhesion to Cultured Human Endothelium....Pages 115-119 Myeloperoxidase Binds to Vascular Endothelial Cells, is Recognized by ANCA and can Enhance Complement Dependent Cytotoxicity....Pages 121-123 Front Matter....Pages 5-5 Vascular Permeability Changes Induced by Antibodies to Myeloperoxidase....Pages 125-127 Endothelium, Myeloperoxidase, Anti-Myeloperoxidase Interaction in Vasculitis....Pages 129-132 Further Characterization of an Animal Model of Systemic Vasculitis....Pages 133-137 Induction of an Humoral and Cellular (Auto) Immune Response to Human and Rat Myeloperoxidase(MPO) in Brown-Norway(BN), Lewis and Wistar Kyoto(WKY) Rat Strains....Pages 139-142 Front Matter....Pages 143-143 Disease Associations with Anti-Neutrophil Cytoplasmic Antibodies....Pages 145-155 Classification of Vasculitis....Pages 157-163 Classification of Systemic Vasculitis — A Pathologist’s View....Pages 165-172 Polyarteritis Nodosa and Churg-Strauss Syndrome an Internist’s View....Pages 173-184 Vasculitis from the Rheumatologist’s Viewpoint....Pages 185-195 A Nephrological View of the Classification of Vasculitis....Pages 197-208 The Nosology of Wegener’s Granulomatosis Utilizing the Elk Format Augmented by C-ANCA....Pages 209-215 Vasculitis — The Neurologist’s View....Pages 217-226 Treatment of Chronic Idiopathic Systemic Vasculitides....Pages 227-234 Monoclonal Antibody Therapy for Vasculitis....Pages 235-238 Heterogeneity of ANCA Sera Showing Atypical, Peripheral and Classical Cytoplasmic Immunofluorescence Patterns....Pages 239-243 ANCA in Systemic Vasculitides, Collagen Vascular Diseases, Rheumatic Disorders and Inflammatory Bowel Diseases....Pages 245-251 Perinuclear Antineutrophil Cytoplasmic Antibodies (P-ANCA): Clinical Significance and Relation to Antibodies Against Myeloid Lysosomal Enzymes....Pages 253-256 Methods of Detection of Anticathepsin G Autoantibodies in Human....Pages 257-261 Anti-Myeloperoxidase Antibodies and Associated Diseases....Pages 263-266 Autoantibodies Directed Against Lysozyme: A New Target Antigen for Anti-Neutrophil Cytoplasmic Antibodies (ANCA)....Pages 267-272 Front Matter....Pages 143-143 Clinical Significance of ANCA in 98 Patients....Pages 273-279 Anti-Neutrophil Cytoplasmic Autoantibodies (ANCA): Antigenic Specificities and Clinical Associations....Pages 281-286 Significance of Autoantibodies to Purified Proteinase 3 in Systemic Vasculitis....Pages 287-289 Characterization of Anti-Myeloperoxidase Antibodies in Vasculitis....Pages 291-294 Low Predictive Value of C-ANCA and Anti-α -Granule Antibody for Vasculitis in Hong Kong....Pages 295-298 Detection and Clinical Associations of Autoantibodies to Myeloid Granular Proteins....Pages 299-302 Relationship Between Disease Activity and ANCA Level by Elisa in the Long-Term Management of Vasculitis....Pages 303-304 ANCA in Ocular Inflammatory Disorders....Pages 305-307 Head and Neck Involvement in Wegener’s Granulomatosis (WG)....Pages 309-313 Orbital Involvement in Wegener’s Granulomatosis....Pages 315-317 MRI of the Head in Wegener’s Granulomatosis....Pages 319-321 The Clinical, Serologic, and Immunopathologic Heterogeneity of Cutaneous Leukocytoclastic Angiitis....Pages 323-326 No ANCA in Thromboangiitis Obliterans (Bürger’s Disease)....Pages 327-330 Alpha-1-Proteinase Inhibitor and Pulmonary Haemorrhage in Systemic Vasculitis....Pages 331-335 Alpha-1-Antitrypsin, CRP and Interleukin-6 in ANCA-positive Vasculitis....Pages 337-340 Pro- and Anti-Inflammatory Cytokines in Primary Systemic Vasculitis....Pages 341-344 Problems of Classification in Necrotizing Vasculitis....Pages 345-348 Anti-Neutrophil Cytoplasm Antibodies in HIV Infection....Pages 349-352 Anti-Neutrophil Cytoplasmic Antibodies (ANCA) and Infection....Pages 353-356 Anti-Lactoferrin Antibodies in Patients with Rheumatoid Arthritis with Vasculitis....Pages 357-362 Front Matter....Pages 143-143 Anti-Neutrophil Cytoplasmic Antibodies in Giant Cell Arteritis and Polymyalgia Rheumatica....Pages 363-366 Antibodies to Neutrophil Cytoplasmic Antigens in Rheumatoid Arthritis....Pages 367-370 Incidence and Specificity of p-ANCA in Rheumatic Diseases....Pages 371-373 Anti-Granulocyte Perinuclear Antibodies but Not Anti-Neutrophil Cytoplasmic Antibodies (ANCA) in Rheumatoid Arthritis....Pages 375-380 Anti-Neutrophil Cytoplasmic Antibodies in Patients with Systemic Lupus Erythematosus....Pages 381-384 ANCA with Specificity for Lactoferrin in Systemic Lupus Erythematosus (SLE)....Pages 385-387 Serum Markers of T-Cell Activation in Relapses of Wegener’s Granulomatosis....Pages 389-392 Presence of Anticardiolipin Antibodies Discriminates Between Wegener’s Granulomatosis and Microscopic Polyarteritis....Pages 393-396 Increased Expression of CD 25 and Adhesion Molecules on Peripheral Blood Lymphocytes of Patients with Wegener’s Granulomatosis (WG) and ANCA Positive Vasculitides....Pages 397-404 Wegener’s Granulomatosis with Severe Peripheral and Central Neuropathy — Case Report....Pages 405-409 Limited Prognostic Value of Changes in Antineutrophil Cytoplasmic Antibody Titers in Patients with Wegener’s Granulomatosis....Pages 411-414 Wegener’s Granulomatosis and Henoch-Schönlein Purpura in a Family with Hereditary C4 Deficiency....Pages 415-418 Little Evidence for Anti-Endothelial-Cell Antibodies in Microscopic Polyarteritis and Wegener’s Granulomatosis....Pages 419-422 Prevalence of ANCAs in Patients on Maintenance Haemodialysis....Pages 423-425 Rhabdomyolysis in Vasculitis....Pages 427-430 Rapidly Progressive Glomerulonephritis (RPGN): Is there Still an “Idiopathic” Subgroup?....Pages 431-434 Association between Silica Exposure and Necrotizing Crescentic Glomerulonephritis with p-ANCA and anti-MPO Antibodies: A Hospital-Based Case-Control Study....Pages 435-440 ANCA and Anti-GBM Antibodies in RPGN....Pages 441-444 P-ANCA with Myeloperoxidase Antibodies and C-ANCA with Proteinase 3 Antibodies Define a Different Vasculitis Entity in Patients with Renal Involvement....Pages 445-447 Antineutrophil-Cytoplasmic-Autoantibodies in Poststreptococcal Nephritis....Pages 449-453 Front Matter....Pages 143-143 Indentification of Apparent Dual ANCA Specificities in a Subset of Patients with Systemic Vasculitis and Crescentic Glomerulonephritis....Pages 455-459 C-ANCA and p-ANCA Positive Vasculitis with Crescentic Glomerulonephritis: The Same Clinical Feature but Different Immunological Pathways ?....Pages 461-464 Clinical Manifestations in Patients on Chronic Dialysis with High Titres of ANCA....Pages 465-468 Pooled Intravenous Immunoglobulin in the Management of Systemic Vasculitis....Pages 469-472 Cyclosporin a Therapy for Wegener’s Granulomatosis....Pages 473-476 Necrotizing Vasculitis — Relapse Despite Cytotoxic Therapy....Pages 477-481 Effectiveness of Cyclophosphamide Pulse Treatment in Wegener’s Granulomatosis....Pages 483-486 Treatment of Wegener’s Granulomatosis with Intravenous Immunoglobulin....Pages 487-489 Treatment of Wegener’s Granulomatosis....Pages 491-495 Treatment Modalities and ANCA in Takayasu’s Arteritis....Pages 497-501 Late Recurrence of Systemic Vasculitis after Kidney Transplantation Involving the Kidney Allograft....Pages 503-506 P-ANCA of Undefined Specificity in Ulcerative Colitis: Correlation to Disease Activity and Therapy....Pages 507-513 Anti-Neutrophil Cytoplasmic Auto-Antibodies (ANCA) in Patients with Ulcerative Colitis (UC): Influence of Disease Activity and Familial Study....Pages 515-518 Antineutrophil Antibodies in Inlammatory Bowel Disease Recognize Different Antigens....Pages 519-522 Antineutrophil Cytoplasmic Auto-Antibodies in Sera from Patients with Ulcerative Colitis after Proctocolectomy with Ileo-Anal Anastomosis....Pages 523-525 P-ANCA as a Diagnostic Marker in Ulcerative Colitis....Pages 527-531 Lactoferrin, Anti-Lactoferrin Antibodies and Inflammatory Disease....Pages 533-538 Detection of Antineutrophil Cytoplasmatic Antibodies (ANCA) and their Association with other Autoantibodies in Patients with Hepatobiliary Disorders....Pages 539-544 Anti-Neutrophil Cytoplasmic Antibodies (ANCA) in Autoimmune Liver Disease....Pages 545-549 Back Matter....Pages 551-552 WEGENER'S GRANULOMATOSIS & ANCA-ASSOCIATED DISEASES: THE STORY CONTINUES The disease now designated as Wegener's granulomatosis (WG) was first described in 1931 by Heinz Klinger, who considered it to be a special form of polyarteritis nodosa. Klinger's friend, Friedrich Wegener, expanded on the first observations and interpreted the pathological and clinical fmdings to represent a distinct disease entity (Wegener, 1939). He described this entity as a "peculiar rhinogenous granulomatosis with a unique participation of the arterial system and the kidneys". Later, Godman and Churg (1954) established the classical diagnostic criteria (the "WG triad"): granuloma, vasculitis, and glomerulonephritis. In 1958 Walton pointed out the poor prognosis of WG based on a small number of published cases (mean survival time: 5 months). In 1966 Carrington and Liebow reported "limited forms" of WG with a defmitely more favorable prognosis. Since then positive results have been reported with cyclophosphamide therapy. In addition, a retrospective study of combined low-dose cyclophosphamide and prednisolone in 85 WG patients over a period of 21 years found a similarly encouraging outcome. The·latter experience led to the current "standard" treatment protocol (FAUCI et al., 1973 and 1983). More recently, strong evidence has emerged that some of the morbidity and mortality ofWG - and other types of systemic vasculitis - may be a consequence of this treatment (Hoffman et al., 1992) Proceedings of the Fourth International Workshop on ANCA and the Second International Colloquium on Wegener's Granulomatosis and Vasculitic Disorders held in Lubeck, Germany, May 28-30, 1992