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Vasculitis anca positivo 2015

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Vasculitis anca positivo 2015

  1. 1. Vasculitis ANCA positivo 2015 Dr. Cristhian Mauricio Bueno Lara Especialista en medicina interna – Universidad Autónoma de Bucaramanga Fellow en Nefrología – Universidad del Valle
  2. 2. Conceptos Comprenhensive Clinical Nephrology 4th Edition 11Para el nefrólogo 1 2 3 4 5 6 7 8 9 10 11 91 caso por cada 1000 a 10000 habitantes/año 1 3 4 7 865 9 2 3Vasculitis ANCA positivo 1 32
  3. 3. Conceptos Síndrome riñón - Pulmón Glomerulonefritis rápidamente progresiva Glomerulonefritis pauciinmune Vasculitis ANCA positivo Glomerulonefritis rápidamente progresiva – Hemorragia alveolar difusa Disfunción renal en días/semanas Proteinuria – Hematuria – Semilunas celulares Presión arterial normal Escasos depósitos de inmunoglobulinas o complemento en glomérulo vasos renales Poliangeítis granulomatosa Poliangeítis microscópica Poliangeítis granulomatosa eosinofílica Stephen C West, Nishkantha Arulkumaran, Philip W Ind, Charles D Pusey. Pulmonary-renal syndrome: a life threatening but treatable condition. Postgrad Med J 2013
  4. 4. Clasificación American College of Rheumatology 1990
  5. 5. Clasificación Chapel Hill Consensus Conference 1994
  6. 6. Clasificación The European Medicines Agency 2008
  7. 7. Clasificación Eoin F. McKinney & Lisa C. Willcocks & Verena Broecker. The immunopathology of ANCA-associated vasculitis. Semin Immunopathol (2014) 36:461–478 99 Pacientes ANCA positivo
  8. 8. Friedrich Wegener • Nacimiento: 4 abril 1907, Noreste de Alemania. • Médico y asistente en patología • Granulomatosis rinógena singular • Retiro de la medicina en 1970 en medio del reconocimiento. • Muerte: 1990 a los 83 años. • Título: Master Clinician of the American College of Chest Physician Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener’s granulomatosis). Journal of Autoimmunity 48-49 (2014) 94e98 • 50 a 100 autopsias en judíos al mes • Año 2007: Destitución de titulo de Master por ACCP
  9. 9. Jacob Churg – Lotte Strauss • 1951: 13 necropsias en pacientes con asma y vasculitis sistémica. • Chumbley y Lanham han cambiado los criterios. Diagnosis and classication of eosinophilic granulomatosis with polyangiitis (formerly named Churge Strauss syndrome) Journal of Autoimmunity 48-49 (2014) 99e103
  10. 10. Clasificación Chapel Hill Consensus Conference 2012
  11. 11. Clasificación Chapel Hill Consensus Conference 2012 Vasculitis de pequeño vaso 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Vol. 65, No. 1, January 2013, pp 1–11
  12. 12. Clasificación Chapel Hill Consensus Conference 2012 Vasculitis ANCA positivo 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Vol. 65, No. 1, January 2013, pp 1–11 Vasculitis necrotizante Sin o muy poco depósitos inmunes Afectación de pequeño vaso Asociado a ANCA Mieloperoxidasa o Proteinasa 3 Poliangeítis microscópica Glomerulonefritis necrotizante Capilaritis pulmonar es frecuente No inflamación granulomatosa Poliangeítis granulomatosa Inflamación granulomatosa necrotizante Compromiso respiratorio alto o bajo Glomerulonefritis necrotizante Poliangeítis granulomatosa eosinofílica Inflamación granulomatosa necrotizante y rica en eosinófilos Asociada a asma con eosinofilia Afectación del tracto respiratorio
  13. 13. Epidemiología Classification, epidemiology and clinical subgrouping of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Nephrol Dial Transplant (2015) 30: i14–i22 Por millón de habitantes
  14. 14. Epidemiología local 2015 85% Hispanos 48% Caucásicos
  15. 15. Fisiopatología Diagnosis and classication of eosinophilic granulomatosis with polyangiitis (formerly named Churge Strauss syndrome) Journal of Autoimmunity 48-49 (2014) 99e103
  16. 16. Fisiopatología Comprenhensive Clinical Nephrology 4th Edition
  17. 17. Manifestaciones Clínicas Comparison of disease activity measures for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis PA Merkel1, DD Cuthbertson2, B Hellmich3, GS Hoffman4, DRW Jayne5, CGM Kallenberg6, JP Krischer2, R Luqmani7, AD Mahr8, EL Matteson9, U Specks9, and JH Stone10 for the Vasculitis Clinical Research Consortium 1Boston University School of Medicine, Boston, Massachussets, USA 2University of South Florida, Tampa, Florida, USA 3Kreiskrankenhaus Plochingen, Plochingen, Germany 4Cleveland Clinic, Cleveland, Ohio, USA 5Addenbrookes Hospital, Cambridge, UK 6University Medical Center Groningen, University of Groningen, Groningen, The Netherlands 7University of Oxford, Oxford, UK 8Hospital Cochin, Paris, France 9Mayo Clinic, Rochester, Minnesota, USA 10Massachusetts General Hospital, Boston, Massachussets, USA Abstract Aim—Currently, several different instruments are used to measure disease activity and extent in clinical trials of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, leading to division among investigative groups and difficulty comparing study results. An exercise comparing six different vasculitis instruments was performed. Methods—A total of 10 experienced vasculitis investigators from 5 countries scored 20 cases in the literature of Wegener granulomatosis or microscopic polyangiitis using 6 disease assessment NIH Public Access Author Manuscript Ann Rheum Dis. Author manuscript; available in PMC 2011 July 19. Published in final edited form as: Ann Rheum Dis. 2009 January ; 68(1): 103–106. doi:10.1136/ard.2008.097758. Comparison of disease activity measures for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis PA Merkel1, DD Cuthbertson2, B Hellmich3, GS Hoffman4, DRW Jayne5, CGM Kallenberg6, JP Krischer2, R Luqmani7, AD Mahr8, EL Matteson9, U Specks9, and JH Stone10 for the Vasculitis Clinical Research Consortium 1Boston University School of Medicine, Boston, Massachussets, USA 2University of South Florida, Tampa, Florida, USA 3Kreiskrankenhaus Plochingen, Plochingen, Germany 4Cleveland Clinic, Cleveland, Ohio, USA 5Addenbrookes Hospital, Cambridge, UK 6University Medical Center Groningen, University of Groningen, Groningen, The Netherlands 7University of Oxford, Oxford, UK 8Hospital Cochin, Paris, France 9Mayo Clinic, Rochester, Minnesota, USA 10Massachusetts General Hospital, Boston, Massachussets, USA Abstract Aim—Currently, several different instruments are used to measure disease activity and extent in clinical trials of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, leading to division among investigative groups and difficulty comparing study results. An exercise comparing six different vasculitis instruments was performed. Methods—A total of 10 experienced vasculitis investigators from 5 countries scored 20 cases in NIH Public Access Author Manuscript Ann Rheum Dis. Author manuscript; available in PMC 2011 July 19. Published in final edited form as: Ann Rheum Dis. 2009 January ; 68(1): 103–106. doi:10.1136/ard.2008.097758. 2009 Poliangeítis microscópica 82% compromiso glomerular Piel, Articulaciones y pulmonar Poliangeítis granulomatosa 80% compromiso glomerular 90% compromiso pulmonar (Alto o bajo) Poliangeítis granulomatosa eosinofílica 50% frecuente compromiso glomerular 100% Asma 72% Sistema nervioso periférico
  18. 18. Manifestaciones Clínicas Comprenhensive Clinical Nephrology 4th Edition
  19. 19. Diagnóstico Estudio inicial Exámenes iniciales Hemograma tipo IV Creatinina Nitrógeno ureico en sangre Uroanálisis Anticuerpos antinucleares Función hepática Clínica ANCAs Comprenhensive Clinical Nephrology 4th Edition
  20. 20. Diagnóstico ANCA dirigido a auto-antígenos Clinical features and diagnosis of small-vessel vasculitis. doi:10.3949/ccjm.79.s3.01 Mejor prueba para la evaluación de los ANCA Valor pronóstico de los ANCA Implicación clínica del tipo de ANCA toemphasizethat t usedintheselabora whereIFTwithor w A variety of dif tionof ANCAshav includingthird-ge based multiplex a for analysingANC summarizesthem methodologyand testinginvasculitis applyingthesetech Automated ana Despitetheemerge IFTremainstherec ingin vasculitis. H dependsonanum conjugatesandfixa cells,storagecond andwashing.7 Furt consuming,requir performance compared with conventional assays and can be used for PR3-ANCA and MPO-ANCAdetection The new methods for ANCAdetection and evaluation in ANCA-associated vasculitis should be urgentlyevaluated in multicentre studies, in anticipation of updating of standardization processes and a revision of existing strategies a b Figure 1 | ANCAIIFpattern differentiation byautomated signal intensityanalysis. IIFimages, taken automatically byAKLIDES®, can discriminate a | P-ANCA-specific and b | C-ANCA-specific staining of neutrophils byuse of mathematical algorithms for pattern differentiation. Chromatin is stained byDAPI (blue) and specific ANCA
  21. 21. Diagnóstico ANCA dirigido a auto-antígenos Clinical features and diagnosis of small-vessel vasculitis. doi:10.3949/ccjm.79.s3.01 Mejor prueba para la evaluación de los ANCA Inmunofluorescencia Pruebas antígeno- específicas
  22. 22. Diagnóstico ANCA dirigido a auto-antígenos Current and emerging techniques for ANCA detection in vasculitis Nat. Rev. Rheumatol. 10, 494–501 (2014) rapidANCA testing.35 In thisassay,IFT iscombinedwitha dot-blot test for PR3-ANCA and MPO-ANCA. However, theresultsobtained areonly qualitativeand should be confirmed and quantified by other ANCA assays. patientswith suspected vasculitisneedstobeevaluated in prospectivestudies. Clinical usefulness of ANCA testing Table 1 | Comparison of methods for testing for PR3-ANCA and MPO-ANCA in ANCA-associated vasculitis Patient population (n) vs comparison group (n) Method Sensitivity (%) Specificity (%) PPV (%) NPV (%) AUC/ ROC Comments GPA (86) vs non-vasculitic disease (450)28 IFT Direct PR3-ANCA ELISA Capture ELISA Anchor ELISA 92 60 72 96 99 99 99.3 98.5 Nd Nd 0.96 (0.94–0.98) 0.80 (0.76–0.83) 0.86 (0.82–0.89) 0.96 (0.94–0.98) Histological diagnosis Retrospective study GPA (232) vs in ammatory diseases (661)29 IFT Anchor ELISA 77.9 80.4 90.9 97.4 73 88 93 93 Nd Histological diagnosis Prospective study GPA (59* ) vs in ammatory and infectious diseases (585)30 Hn–hr PR3-ANCA ELISA Capture ELISA Direct (hn) PR3-ANCA 94 66 64 99 (prede ned) Nd Nd Nd Histological diagnosis Retrospective study GPA (34) vs SLE (65)31 Direct PR3-ANCA Anchor ELISA 97.1 98.4 Nd Nd 0.999 (0.947–1.00) Clinical diagnosis Retrospective study GPA (40) vs RA or SLE (20)32 IFT Direct PR3-ANCA (n= 5 kits) Capture ELISA (n=2 kits) Anchor ELISA (n= 4 kits) 62.5 45–55 60–62.5 60–62.5 95–100 Nd Nd Nd Histological diagnosis Retrospective study MPA (40) vs RA or SLE (20)32 IFT Direct MPO-ANCA ELISA (n= 8 kits) Capture ELISA (n=2 kits) Anchor ELISA (n= 1 kit) 82.5 62.5–85 80 75 95–100 Nd Nd Nd Histological diagnosis Retrospective study GPA (55) vs suspected vasculitis (175)33 IFT Direct PR3-ANCA ELISA (n= 2 kits) Capture ELISA (n=2 kits) Anchor ELISA (n= 3 kits) Other assays (n=2) 69.1 61.8–72.7 70.9–72.7 61.8–72.7 72.7–74.5 100 95.4–96.4 95.9–99.5 98.5–99.0 95.9–97.9 Nd Nd Nd 0.856–0.879 0.862–0.878 0.833–0.881 0.878–0.902 Clinical diagnosis Retrospective study * 47 of 59 patients in the GPA group had a cytoplasmic ANCA pattern on IFT. Abbreviations: ANCA, antineutrophil cytoplasmic antibody; AUC, area under the curve; GPA, granulomatosis with polyangiitis; hn, human native; hr, human recombinant; IFT, indirect immunofluorescence technique; MPA, microscopic polyangiitis; MPO, myeloperoxidase; Nd, not determined; NPV, negative predictive value; PPV, positive predictive value; PR3, proteinase 3; RA, rheumatoid arthritis; ROC, receiver operating characteristics; SLE, systemic lupus erythematosus. REVIEWS
  23. 23. Diagnóstico ANCA dirigido a auto-antígenos Valor pronóstico de los ANCA Comparison of disease activity measures for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis PA Merkel1, DD Cuthbertson2, B Hellmich3, GS Hoffman4, DRW Jayne5, CGM Kallenberg6, JP Krischer2, R Luqmani7, AD Mahr8, EL Matteson9, U Specks9, and JH Stone10 for the Vasculitis Clinical Research Consortium 1Boston University School of Medicine, Boston, Massachussets, USA 2University of South Florida, Tampa, Florida, USA 3Kreiskrankenhaus Plochingen, Plochingen, Germany 4Cleveland Clinic, Cleveland, Ohio, USA 5Addenbrookes Hospital, Cambridge, UK 6University Medical Center Groningen, University of Groningen, Groningen, The Netherlands 7University of Oxford, Oxford, UK 8Hospital Cochin, Paris, France 9Mayo Clinic, Rochester, Minnesota, USA 10Massachusetts General Hospital, Boston, Massachussets, USA Abstract NIH Public Access Author Manuscript Ann Rheum Dis. Author manuscript; available in PMC 2011 July 19. Published in final edited form as: AnnRheumDis. 2009 January ; 68(1): 103–106. doi:10.1136/ard.2008.097758. Comparison of disease activity measures for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis PA Merkel1, DD Cuthbertson2, B Hellmich3, GS Hoffman4, DRW Jayne5, CGM Kallenberg6, JP Krischer2, R Luqmani7, AD Mahr8, EL Matteson9, U Specks9, and JH Stone10 for the Vasculitis Clinical Research Consortium 1Boston University School of Medicine, Boston, Massachussets, USA 2University of South Florida, Tampa, Florida, USA 3Kreiskrankenhaus Plochingen, Plochingen, Germany 4Cleveland Clinic, Cleveland, Ohio, USA 5Addenbrookes Hospital, Cambridge, UK 6University Medical Center Groningen, University of Groningen, Groningen, The Netherlands 7University of Oxford, Oxford, UK 8Hospital Cochin, Paris, France 9Mayo Clinic, Rochester, Minnesota, USA 10Massachusetts General Hospital, Boston, Massachussets, USA NIH Public Access Author Manuscript Ann Rheum Dis. Author manuscript; available in PMC 2011 July 19. Published in final edited form as: Ann Rheum Dis. 2009 January ; 68(1): 103–106. doi:10.1136/ard.2008.097758. NIH-PAAuthorManuscriptNIH-P 2009 NO
  24. 24. Diagnóstico ANCA dirigido a auto-antígenos Implicación clínica del tipo de ANCA 1995
  25. 25. Diagnóstico Imágenes Pathogenesis of ANCA-Associated Vasculitis: New Possibilities for Intervention. Am J Kidney Dis. 62(6):1176-1187 Nódulos pulmonares múltiples Cavitaciones Patrón en vidrio esmerilado
  26. 26. Diagnóstico Histopatología Focal Creciente Mixto Esclerótica 2010
  27. 27. Diagnóstico Histopatología Histopathological classification of pauci-immune glomerulonephritis and its impact on outcome. Rheumatol Int (2014) 34:1721–1727
  28. 28. Diagnóstico Clinical features and diagnosis of small-vessel vasculitis. doi:10.3949/ccjm.79.s3.01
  29. 29. Diagnóstico 2008
  30. 30. Tratamiento Ciclofosfamida 2009
  31. 31. Tratamiento Treatment of ANCA-Associated Vasculitis: New Therapies and a Look at Old Entities. Advances in Chronic Kidney Disease, Vol 21, No 2 (March), 2014: pp 182-193 1. Ciclofosfamida 15 mg/Kg/dosis cada 2 semanas por 3 dosis. 2. Continuar con Ciclofosfamida 15 mg/Kg/dosis cada 3 semanas hasta la remisión de la enfermedad 3. Continuar hasta 3 meses posterior a remisión de la enfermedad con medicación oral o endovenosa (Mantenimiento) Ciclofosfamida
  32. 32. Tratamiento Rituximab 2015
  33. 33. Tratamiento Plasmaféresis 2013
  34. 34. Tratamiento Inmunoglobulina 2009
  35. 35. Tratamiento Inmunoglobulina Intravenousimmunoglobulin asadjuvant therapy for Wegener’sgranulomatosis(Review) Fortin PM, Tejani AM, Bassett K, Musini VM Figure 1. QUORUM (qualityof reportingmeta-analyses) flowchart of studyselection Included studies For detailsof theincluded study seeCharacteristicsof included studies. Thisstudywasaprospective,randomized,double-blind,placebo- controlled multicenter trial. Thirty four patientswererandomly assignedtoreceiveIVIg(total dose=2g/kg,n=17)or placebo(n patientseither relapsing on steroidsalone(or steroidsin combi- nation with immunosuppressant therapy) or thosethat did not achievefull remission on steroidsin combination with immuno- suppressant therapyfollowinginitial presentation. Patients were excluded from participating in the trial if they had any of the following characteristics: IVIg therapy during the previous three months, history of anaphylaxis to properly matchedbloodproducts,selectiveIgAdeficiency,rapidlyprogres- Main results WeincludedoneRCT with34participantswhowererandomlyassignedtoreceiveIVIgor placebooncedailyinaddition toazathioprine and prednisolone for remission maintenance. Therewere no significant differencesbetween adjuvant IVIg and adjuvant placebo in mortality, seriousadverseevents, timetorelapse, open-label rescuetherapy, and infection rates. Thefall in diseaseactivity score, derived from patient-reported symptoms, was slightly greater in the IVIg group than in the placebo group at one month (MD 2.30; 95% Confidenceinterval (CI) 1.12 to 3.48, P<0.01) and threemonths(MD 1.80; 95% CI 0.35 to 3.25, P=0.01). Therewasasignificant increasein total adverseeventsin theIVIggroup (relativerisk (RR) 3.50; 95% CI 1.44 to 8.48, P< 0.01). Authors’ conclusions Thereisinsufficient evidencefromoneRCT that IVIgadjuvant therapyprovidesatherapeuticadvantagecomparedwiththecombination of steroids and immunosuppressants for patients with WG. Given the high cost of IVIg (one dose at 2 g/kg for a 70 kg patient = $8,400), it should belimited to treat WG in thecontext of awell conducted RCT powered to detect patient-relevant outcomes. P L A I N L A N G U A G E S U M M A R Y Intravenousimmunoglobulin in addition to standard treatmentsfor Wegener’sgranulomatosis Wegener’sgranulomatosis isararedisorder that causesinflammation of theblood vessels. Thisinflammation restrictsblood flow to 2013
  36. 36. Tratamiento Treatment of ANCA-Associated Vasculitis: New Therapies and a Look at Old Entities. Advances in Chronic Kidney Disease, Vol 21, No 2 (March), 2014: pp 182-193
  37. 37. Futuro 2015

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