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ANCA Vasculitis Or ANCA-Associated Vasculitis

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ANCA Vasculitis Or ANCA-Associated Vasculitis One Hundred and Eleventh Series – May 9-10, 2019 Portland, Oregon Why Do Patients Develop ANCA Disease? Ronald J. Falk, MD Nan and Hugh Cullman Eminent Professor of Medicine Chair, Department of Medicine University of North Carolina, Chapel Hill NC USA 1 Common Clinical Questions 1. “What is the name of my disease?” “How did I get it?” What is the cause of disease? 2. “What will make it go away?” What factors are involved in disease pathogenesis? 3. “What will prevent it from coming back?” What causes a relapse, and what sustains a remission? 2 Our First Case JP is a 15-year-old female with the sudden onset of coughing up blood in a previously healthy child. There was a 2-week history of flu-like illness. Physical examination was marked by hypertension and pulmonary findings of diffuse crackles in both lung fields. The serum creatinine was 10 mg/dL. Treatment was with pulses of methylprednisolone. 3 Antigen Specificity Cytoplasmic ANCA Perinuclear ANCA Proteinase 3 Myeloperoxidase (PR3-ANCA), BPI, (MPO-ANCA), 4 others elastase, others The Early ANCA Vasculitis Story • Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener’s granulomatosis o van der Woude FJ, Rasmussen N, Lobatto S et al. Lancet 1985; 325(8426):425-9 • Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis o Falk RJ, Jennette JC. N Engl J Med 1988; 318(25):1651-7 • Wegener’s granulomatosis autoantibodies identify a novel diisopropylfluorophosphate-binding protein in the lysosomes of normal human neutrophils o Goldschmeding R et al. J Clin Invest 1989; 84(5):1577-87 • Specificity of anti-neutrophil cytoplasmic autoantibodies for proteinase 3 o Jennette JC, Hoidal JR, Falk RJ. Blood 1990 74(11):2263-4 5 ANCA testing led to: • A new classification system and a unified, overall name: ANCA vasculitis or ANCA-associated vasculitis • Recognition that disparate clinical symptoms are part of the same disease process • Simplification of induction therapy strategies • Organization of several randomized clinical trials 6 Immune Complex Small Vessel Vasculitis 2012 Chapel Hill Cryoglobulinemic Vasculitis Consensus Conference IgA Vasculitis (Henoch-Schönlein) Vasculitis Nomenclature Hypocomplementemic Urticarial Vasculitis (Anti-C1q Vasculitis) Medium Vessel Vasculitis Polyarteritis Nodosa Anti-GBM Disease Kawasaki Disease ANCA-Associated Small Vessel Vasculitis Microscopic Polyangiitis Granulomatosis with Polyangiitis (Wegener’s) Eosinophilic Granulomatosis with Polyangiitis Large Vessel Vasculitis (Churg-Strauss) Takayasu Arteritis Giant Cell Arteritis Jennette JC, Falk RJ et al. Arthritis Rheum 2013;65:1-11 ANCA-associated vasculitis can affect virtually any tissue and organ in the body: Pulmonary Glomerular alveolar capillaries capillaries (glomerulonephritis) (capillaritis) Necrosis Crescent Venules in Small arteries in many organs multiple organs (venulitis) (arteritis) Capillaries: Venules: Arteries: • Glomerulonephritis • Purpura • Mononeuritis multiplex • Pulmonary capillaritis • Medullary angiitis • Skin nodules and ulcers Concept of Pauci-Immune Glomerulonephritis Crescentic Glomerulonephritis Immune Pauci- Anti-GBMComplex Immune >90% ANCA+ 9 Signs and Symptoms of Necrotizing Small Vessel Vasculitis • Cutaneous purpura, nodules and ulcerations • Peripheral neuropathy (mononeuritis multiplex) • Abdominal pain and blood in stool • Hematuria, proteinuria and renal insufficiency • Hemoptysis and pulmonary infiltrates or nodules • Necrotizing (hemorrhagic) sinusitis • Myalgias and arthralgias • Muscle and pancreatic enzymes in blood • Propensity for venous thrombosis 10 Light Microscopic Morphology FOCAL SCLEROSING GN CHRONIC GN FOCAL NECROSIS AND CRESCENTIC END STAGE NECROSIS FEW CRESCENTS GN KIDNEY HEMATURIA and/or ACUTE RAPIDLY SLOWLY PROTEINURIA NEPHRITIS PROGRESSIVE PROGRESSIVE NEPHRITIS NEPHRITIS 11 12 Antigen Specificity Cytoplasmic ANCA Perinuclear ANCA Proteinase 3 Myeloperoxidase (PR3-ANCA), BPI, (MPO-ANCA), 14 others elastase, others ANCA Disease PR3-ANCA Disease ANCA- MPO-ANCA Negative Disease Disease FOR EXAMPLE: MPO-ANCA glomerulonephritis MPO-ANCA microscopic polyangiitis PR3-ANCA granulomatosis disease MPO-ANCA Churg-Strauss Syndrome 15 Falk RJ and Jennette JC. J Am Soc Nephrol 2010; 21(5):745-52 CCX168 (Avacopan) azathioprine plasmapheresis methotrexate rituximab mycophenolate glucocorticoids Cyclophosphamide ©cjb Current Therapy Plasmapheresis or IV methylprednisolone then IV cyclophosphamide and/or rituximab then azathioprine or rituximab and/or Stop In general, remission rates are on the order of 70% to 93%. Relapse rates vary from 0% to 50%. Worrying about Over-Immunosuppression 19 Take Home Message #1 ANCA, which was an early biomarker of disease, is now the name of the disease, resulting in earlier diagnosis and more prompt therapy. 20 Common Clinical Questions 1. “What is the name of my disease?” “How did I get it?” What is the cause of disease? 2. “What will make it go away?” What factors are involved in disease pathogenesis? 3. “What will prevent it from coming back?” What causes a relapse, and what sustains a remission? 21 Pathogenesis of ANCA Necrotizing Vasculitis To cause vasculitis, ANCA must induce this event sequence: . Leukocyte margination, adherence, and diapedesis . Leukocyte activation with degranulation and generation of toxic oxygen metabolites . Vascular necrosis with karyorrhexis and fibrinous insudation 22 JCJ Hypothetical Pathogenesis of ANCA Vasculitis by ANCA Primed neutrophils are activated by ANCA with involvement of FcR and complement activation amplification. Modified from: Falk RJ, Jennette JC. J Am Soc Nephrol 2010; 21:745-52 23 MPO-ANCA Mediated Necrotizing and Crescentic Glomerulonephritis and Vasculitis in Mice Immunize with MPO MPO-KO 1. Immunize MPO-knockout mice with murine MPO. Anti-MPO antibodies 2. Transfer anti-MPO IgG into Rag2-/- or WT B6 mice. 3. Causes MPO-ANCA-induced pauci-immune necrotizing and crescentic glomerulonephritis and small vessel vasculitis. Xiao H, Heeringa P, Hu P, Liu Z, Zhao M, Aratani Y, Maeda N, Falk RJ, Jennette JC. J Clin Invest 2002; 110:955-963. Comparative Pathology of Human AAV and Anti-MPO Mouse Model of AAV Mouse Human Glomerulonephritis Fibrinoid necrosis Crescent Vasculitis Fibrinoid necrosis Inflammation 25 In this anti-MPO IgG induced mouse model of ANCA glomerulonephritis: • Anti-MPO IgG alone can cause disease o Jennette JC et al. J Am Soc Nephrol 2006; 17:1235-1242 • Neutrophils are required o Xiao H et al. Am J Pathol 2005; 167:39-45 • Neutrophil priming exacerbates disease o Jennette JC et al. J Am Soc Nephrol 2006; 17:1235-1242 • T cells do not cause acute disease o Xiao H et al. J Clin Invest 2002; 110:955-963 • Abrogation of the alternative complement pathway prevents disease o Xiao H et al. Am J Pathol 2007; 170:52-64 • C5a receptor antagonists abrogate disease o Schreiber A et al. J Am Soc Nephrol 2009; 20:289-298 26 An orally active antagonist of human C5a receptor given to mice with knocked in human C5a receptor and knocked out mouse C5a receptor suppresses induction of glomerulonephritis by anti-MPO IgG Xiao H, et al. J Am Soc Nephrol 2014; 25(2):225-231 Glomerulonephritis Clinical Study Group (UK) CLEAR trial (NCT01363388): Phase 2 trial of CCX168 (complement inhibitor) in ANCA vasculitis 27 Take Home Message #2 ANCA are pathogenic and target circulating cells rather than directly attacking the target organ (vessels). 28 Major Phases of Autoimmune Disease Genetic Predisposition ANCA Production + T Cell and Macrophage Defective T and B Cell Mediation of Acute Injury (by neutrophils and monocytes) Response to Injury Regulation Environmental Resolution Scarring Factors Relapse/Recurrence 29 Patient Y ANCA Titer ANCA Patient X Active Disease Disease Remission Active Disease 30 Cryptic Epitopes in MPO 6/4/2019 31 PR3 Transcripts are Detected in Mature Neutrophils and Monocytes But Not in Lymphocytes of ANCA 32 Yang JJ et al. J Am Soc Nephrol 2004; 15:2103-14 PRTN3 and MPO mRNA levels in leukocytes from ANCA patients are significantly increased compared to levels from healthy controls PRTN3mRNA p<0.0001 MPOmRNA p<0.0001 8000 12000 7000 10000 6000 8000 5000 4000 6000 mRNA Expression mRNA 3000 4000 2000 (Relativeto a standard curve) 2000 1000 0 0 HC (n=169) ANCA (n=969) HC (n-169) ANCA (n=969) 33 PRTN3 and MPO mRNA Levels are Closely Correlated with Disease Activity During Disease Course MPOmRNA 1000 4000 750 3000 500 2000 mRNA Expression mRNA 250 1000 (Relative (Relative to a standard curve) 0 0 A R A A R A A A R A A R A A 34 PR3 and MPO Gene Transcription are Closely Correlated 1600 R² = 0.70 1400 P<0.0001 1200 1000 800 600 400 MPOmRNA (Spearman Rank) MPOmRNA 200 0 0 200 400 600 800 1000 1200 1400 1600 PR3mRNA (Spearman Rank) 35 MPO and PR3 expression results in new protein production in neutrophils isolated from patients with ANCA vasculitis. McInnis E et al. J Am Soc Nephrol 2015; 26(2): 390-9 36 Transcriptional Dysregulation Linked to Alternative Transcripts PRTN3 PRTN3-002 About 25% of patients are positive for the alternative transcript, mostly during active disease 37 McInnis E et al. J Am Soc Nephrol 2015; 26(2): 390-9 Epigenetic Silencing of Neutrophil Granule Genes Ciavatta DJ et al. J Clin Invest 2010 ; 120(9):3209-19 38 DNA Methylation at CpG Islands Leads to Transcriptional Silencing of Gene Promoters M Methylated Unmethylated Gene Expression CpG Island Gene M M M Gene
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