Annual Rheumatology & Therapeutics Review for Organizations & Societies
|
|
- Percival Willis
- 5 years ago
- Views:
Transcription
1 Annual Rheumatology & Therapeutics Review for Organizations & Societies
2 Update on Granulomatosis with Polyangiitis (Wegener s)
3 Learning Objectives Identify the clinical features of granulomatosis with polyangiitis (Wegener s)(gpa) Describe the diagnostic approach to GPA Outline the treatment options for GPA
4 Vasculitis = Inflammation of the Blood Vessel blood vessel damage compromise of vessel lumen attenuation of vessel wall organ ischemia aneurysm formation / hemorrhage Renal infarction Renal microaneurysms
5 Vasculitis Is Not One Specific Disease Primary Vasculitides Unique disease entities without a currently identified underlying cause where vasculitis forms the pathological basis of tissue injury Secondary Vasculitides Vasculitis occurring secondary to an underlying disease or exposure Giant cell arteritis Takayasu arteritis Kawasaki disease Polyarteritis nodosa Granulomatosis with polyangiitis (Wegener s) Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) IgA vasculitis (Henoch-Schönlein) Medications Infection Malignancy Transplant Cryoglobulinemia Connective tissue disease (RA, SLE, myositis, Sjögren s) Jennette et al. A&R 2013; 65:1
6 Granulomatosis with Polyangiitis (Wegener s) (Formerly Wegener s Granulomatosis) Epidemiology Affects ~3 in 100,000 persons Male = female Mean age of onset years (can occur at any age) Etiology No currently identified: - infectious or environmental trigger - genetic association Strong evidence that disease is immunologically mediated - data gathered within the laboratory - response to immunosuppressive treatment - inflammation seen on tissue histology
7 Granulomatosis with Polyangiitis (Wegener s) Characterized by: Histology: necrotizing granulomatous inflammation vasculitis of small to medium vessels Clinical predilection to involve: upper airways lungs kidneys
8 Granulomatosis with Polyangiitis (Wegener s) Upper Airway Involvement Occurs in ~95% of Patients
9 GPA (Wegener s) Pulmonary Involvement Occurs in ~ 85% of Patients
10 GPA (Wegener s) Glomerulonephritis (GN) 80% will have GN at some point 20% have GN at presentation can be rapidly progressive lacks symptoms proteinuria, active urine sediment Histology focal, segmental, crescentic, necrotizing GN few to no immune complexes (pauci-immune)
11 Granulomatosis with Polyangiitis (Wegener s) Ocular Involvement Occurs in 56 % Can affect any ocular structure and be visually threatening Scleritis / episcleritis Orbital disease
12 Granulomatosis with Polyangiitis (Wegener s) Cutaneous Involvement Occurs in 46% of Patients
13 Granulomatosis with Polyangiitis (Wegener s) Organ System Involvement in 158 Patients ENT LUNG KIDNEY JOINT EYE SKIN PNS CNS Onset Total HEART Hoffman et al. Ann Intern Med Percent of Patients
14 Granulomatosis with Polyangiitis (Wegener s) Differential Diagnosis infection neoplasm / lymphoproliferative disease connective tissue disease granulomatous disease other causes of glomerulonephritis (when present) Differentiation from GPA (Wegener s) is essential as the treatment is different in many instances Diagnosis is usually made by the presence of specific histologic features in a clinically compatible setting
15 QUESTION You are seeing a 33 year old male where GPA is suspected although infection remains a concern. He has sinus mucosal thickening, cavitary pulmonary nodules, nodular skin lesions, and a (+) panca. A biopsy of which of the following would provide the highest diagnostic yield for GPA? A. Sinus biopsy B. Lung biopsy by VATS C. Skin biopsy D. Biopsy is not needed as (+) panca has sufficient predictive value
16 Granulomatosis with Polyangiitis (Wegener s) Diagnosis - Biopsy Not all biopsies are diagnostic Presence of granulomas and/or vasculitis can be patchy Positive yield associated with the amount of tissue obtained ENT 21% sinus>nasal Lung 91% open lung biopsy 7% transbronchial biopsy Kidney Skin focal, segmental, necrotizing glomerulonephritis with few to no immune deposits (pauci-immune) usually insufficient evidence for diagnosis cutaneous vasculitis can be seen in many settings
17 Granulomatosis with Polyangiitis (Wegener s) Evaluation History Physical examination Radiographs Laboratories symptoms - presence and duration nasal membranes, eye, skin, joint, nerve CXR even in the absence of symptoms serum chemistries (renal function) CBC ESR Urinalysis - if (+) blood perform microscopy on fresh urine to look for casts ANCA
18 Antineutrophil Cytoplasmic Antibodies (ANCA) 1982 Davies et al. BMJ Observed serum antibodies that stained neutrophil cytoplasm in 8 patients with segmental necrotizing glomerulonephritis 1985 Van der Woude et al. Lancet Association between cytoplasmic staining antibodies and GPA (Wegener s) Antibodies were more frequently found in active (25 of 27) vs inactive (4 of 32) disease
19 Target antigens in GPA (Wegener s) Cytoplasmic canca = Proteinase-3 Ethanol Fixation = Myeloperoxidase panca Perinuclear
20 Methods of ANCA Testing Indirect Immunofluorescence canca panca Load Antigen Add Serum Antibody Add Antibody Enzyme Conjugate Add Enzyme Substrate ELISA (target antigen-specific) Proteinase 3 Myeloperoxidase Measure Optical Density
21 canca GPA (Wegener s) Microscopic polyangiitis EGPA (Churg-Strauss) Case reports of associations positive canca by IIF positive anti-pr3 ELISA positive canca by IIF negative anti-pr3 ELISA A (+) ANCA done by immunofluorescence should be confirmed by antigen-specific (PR3, MPO) ELISA panca GPA (Wegener s) Microscopic polyangiitis EGPA (Churg-Strauss) Idiopathic crescentic GN Inflammatory bowel disease Other autoimmune diseases Infection Drugs positive panca by IIF positive anti-mpo by ELISA positive panca by IIF negative anti-mpo by ELISA
22 canca panca (-) ANCA GPA (Wegener s) 75-90% 5-20% up to 20% Question: Can (+) ANCA be used to diagnose GPA in place of a tissue biopsy? It depends upon the likelihood of disease based upon the clinical scenario Influenced by GPA (Wegener s) being uncommon and treatment toxicity sinus, lung, renal disease predictive value 90% sinus and lung disease predictive value of ANCA ~30-60% remains a high potential of infection/neoplasm (+) ANCA has poor positive predictive value in low prevalence populations Diagnosis by biopsy remains necessary in many instances
23 QUESTION You are seeing a 45 year old male with GPA on methotrexate and prednisone 20 mg/day who was hospitalized for cough and fever. He has new B/L interstitial infiltrates on chest CT. The medical team checked an ANCA and this is markedly higher than previously. Which of the following statements is true? A. This is not Pneumocystis as this does not occur with methotrexate B. Methotrexate pneumonitis should be considered C. This is a disease relapse as his ANCA has risen D. PFTs with DLCO will not be useful in this setting
24 Is ANCA a Reliable Measure of Disease Activity? In an individual person ANCA level often varies In large series, patients with active vs inactive disease: were more frequently ANCA (+) had higher titers of ANCA Question: can ANCA be used to predict relapse and guide treatment?
25 ANCA - Utility in Guiding Treatment Kerr et al. Arthritis & Rheum 1994; Boomsma et al. Arthritis & Rheum % with a 4 fold rise in titer did not relapse Finkielman JD, et al. Ann Intern Med 2007;147: patients with GPA (Wegener s) ANCA levels were only weakly associated with disease activity Changes in ANCA levels explained < 10% of the variation in disease activity Decreases in ANCA levels were not associated with shorter time to remission Increases in ANCA levels were not associated with relapse 43% relapsed within 1 year of an increase in ANCA levels ANCA titers do not correlate well with disease activity and should not be used to guide immunosuppressive therapy
26 Treatment of Granulomatosis with Polyangiitis (Wegener s) Patient survival What are the goals of treatment? Induce remission of active disease Remission - absence of disease activity Avoid disease relapse Relapse - return of disease activity after remission Minimize therapeutic toxicity
27 Treatment of Granulomatosis with Polyangiitis (Wegener s) Untreated disease Walton 1958: median survival time 5 months Glucocorticoids alone Hollander & Manning 1967: median survival time 12.5 months Daily cyclophosphamide and prednisone Fauci & Wolff 1973: remission in 12 / 14 patients Hoffman et al 1992: 75% complete remission 90% improvement 80% survival
28 GPA Effective Treatment New Challenges Relapse Occurs in 50-70% of patients May be similar or different from initial presentation Treatment Toxicity Complications of Cyclophosphamide Infection 46% Bladder injury Hemocytopenias Infertility Myelodysplasia Transitional cell carcinoma Complications of Glucocorticoids Damage Can occur as a result of disease or treatment Continues to accumulate over time Can be difficult to differentiate from active disease
29 GPA - Induction-Maintenance Staged Treatment Approaches Active Disease Remission (3-6 months) Maintain Remission Induction Maintenance Cyclophosphamide Methotrexate Cyclophosphamide Cyclophosphamide Azathioprine Mycophenolate mofetil
30 QUESTION A 65 year old female with GPA has been on cyclophosphamide for 3 months and is ready to switch to a maintenance option. Her creatinine is 2.3 mg/dl, urinalysis is negative for blood with trace protein, chest CT shows significant improvement but not complete resolution of the prior pulmonary nodules. Which of the following would you recommend she be switched to? A. Azathioprine B. Mycophenolate mofetil C. Methotrexate D. Rituximab
31 GPA - Induction-Maintenance Staged Treatment Approaches Active Disease Remission (3-6 months) Maintain Remission Induction Maintenance Cyclophosphamide Cyclophosphamide Cyclophosphamide Methotrexate Azathioprine Mycophenolate mofetil Langford et al. A&R 1999 Reinhold-Keller et al, 2002 Jayne et al, NEJM 2003 Heimstra et al, 2010 Langford et al, A&R 2004 Pagnoux et al, NEJM 2010 Methotrexate vs Azathioprine similar rates of toxicity and relapse Azathioprine vs Mycophenolate MMF may have a higher rate of relapse
32 Daily versus IV Cyclophosphamide for Remission Induction degroot et al. Ann Int Med 2009;150:670, Harper et al. Ann Rh Dis 2012;71:955 Daily IV OR 15 mg/kg q 2 wks x 3 doses 2 mg/kg 1.5 mg/kg 15 mg/kg Every 3 weeks Azathioprine 2mg/kg/d Azathioprine 2mg/kg/d Prednisolone Remission Induction Consolidation Phase // Months patients Time to remission: IV=PO, median 3 mo Long term relapse: IV 40% PO 21% (p=0.03) Total CYC dose: IV < PO (similar at 3 mo) Leukopenia: IV < PO (PO after mo 2) Issues to consider Consolidation phase CBC frequency (wbc drops over time) IV days 10, 14, before pulse; PO QW x 1 mo, QOW x 1 mo, then monthly IV CYC - effective but not superior to daily CYC higher relapse rate When using PO CYC aim for 3-4 months, with q 1-2 week CBC
33 QUESTION A 55 year old female was just diagnosed with GPA with sinus disease, migratory arthralgias, three 1x2cm pulmonary nodules without respiratory compromise, (+) PR3-cANCA, lung biopsy showing granulomatous vasculitis. Her urinalysis and creatinine are normal. During your overall discussion of treatment, you learn she is extremely fearful of rituximab. Which of the following would you recommend she be treated with? A. Prednisone alone B. Prednisone and cyclophosphamide C. Prednisone and methotrexate D. Prednisone and azathioprine
34 GPA Recognition of the Spectrum of Disease Severity Severe Non-Severe Alveolar hemorrhage Glomerulonephritis CNS Mononeuritis multiplex Pericarditis Vision threatening scleritis Influences treatment decisions Sinonasal disease Oral mucosa Skin Conductive hearing loss Musculoskeletal Lung no respiratory compromise Cyclophosphamide or rituximab Methotrexate
35 Methotrexate for Remission Induction of Non-Severe degroot Disease et al. Arthritis Rheum 2005;52:2461 Cyclophosphamide 2 mg/kg/d 15mg/wk Cyclophosphamide 1.5 mg/kg/d OR Methotrexate 20-25mg/week Prednisolone Remission Months patients Induction of remission within 6 months Methotrexate 90% Cyclophosphamide 93% Methotrexate is not inferior to cyclophosphamide for remission induction of non-severe disease
36 Treatment of Granulomatosis with Polyangiitis (Wegener s) What about Biologic Agents? Specific immunologic targeting may: reduce toxicity reduce relapse induce remission Specific targeting may be insufficient Unexpected effects on disease Disease-specific toxicities Investigation of specific agents has been guided by hypotheses regarding pathophysiologic mechanisms
37 Use of Etanercept in GPA (Wegener s) Standard Therapy + Randomization Induction Prednisone with taper Etanercept Placebo Maintenance // Close of trial 180 patients Objective: determine the efficacy of etanercept to sustain remission Sustained remission > 6 months etanercept 69.7% placebo 75.3% P=0.39 Does not support the use of etanercept in the treatment of GPA Wegener s Granulomatosis Etanercept Trial (WGET), NEJM 2005; 352:19
38 Cyclophosphamide vs Rituximab for Stone et al. NEJM 2010; 363:221 Remission Induction (RAVE) Cyclophosphamide Azathioprine OR (Blinded) Rituximab (375mg/M2/week x 4 weeks) Prednisone Significant exclusions: - creatinine > 4.0 mg/dl - mechanical ventilation 0 6 // 18 Months Primary Endpoint = in remission and off prednisone at 6 months 197 ANCA (+) GPA or MPA Rate of adverse events: RTX = CYC Meeting primary endpoint All patients: RTX 64%, CYC 53% (p<0.001) Relapsing patients: RTX 67%, CYC 42% Severe relapse: RTX 6%, CYC 10% Mortality rate: 2% (1 RTX, 2 CYC) For remission induction, rituximab is as effective as cyclophosphamide This was the basis for FDA approval of RTX for GPA/MPA in April 2011
39 CYC vs Rituximab for Remission Induction Jones et al. NEJM 2010; 363:211 (RITUXVAS) CYC IV CYC Prednisolone Azathioprine VERSUS RANDOMIZED 3:1 RTX:CYC-AZA (Non-Blinded) Rituximab (375mg/M2/week x 4 weeks) RTX IV CYC x 2 Prednisolone 0 6 // 24 Months Primary Endpoint = sustained remission (BVAS=0 for 6 months) 44 ANCA (+) patients, new dx Rate of adverse events: RTX = CYC Hypothesis: RTX better than CYC Primary endpoint: RTX 76%, CYC 82% Mortality rate: 18% (6 RTX, 2 CYC) - Median age 68, 20% dialysis - GFR: RTX 20, CYC 12 ml/min Rituximab was effective but was not superior to cyclophosphamide
40 Rituximab (RTX) in GPA Remission induction Question Rituximab is as effective as cyclophosphamide for remission induction of severe disease does that mean it should be used to induce remission in everyone? No there is not strong data for use in all populations Relapsing severe disease Newly diagnosed severe disease Non-severe relapse, occurring on methotrexate Non-severe relapse, never received methotrexate Newly diagnosed non-severe disease Fulminant disease (creatinine > 4.0, mechanical ventilation) Strength of evidence for use Strong Weak
41 Probability of Remaining in Complete Remission Rituximab (RTX) in GPA Rituximab induces remission So what happens after rituximab? Specks U et al. N Engl J Med 2013; 369:417 No difference in RTX vs CYC/AZA 30% relapse within 1 year Relapses occur after rituximab so a plan for remission maintenance needs to be considered in each patient P = 0.76 CYC/AZA (n=70) RTX (n=76) Options Close clinical observation and retreat with rituximab for relapse Conventional maintenance agent (MTX, AZA, MMF) Scheduled repeat rituximab infusions Time from Complete Remission to Relapse (days) At the current time the optimal approach to maintenance after rituximab has not been determined
42 GPA (Wegener s) - Current Treatment Remission Induction (3-6 months) Maintenance (at least 2 years) Severe Disease Cyclophosphamide + GC Methotrexate Azathioprine (Mycophenolate mofetil) Rituximab + GC Non-severe Disease Methotrexate + GC Methotrexate Factors to take in consideration in determining treatment: Level of disease severity Medication contraindications Initial disease or relapse Past treatment history
43 Summary GPA (Wegener s) Multi-system disease characterized by: small/medium vasculitis of the lungs, kidneys, nerve, skin necrotizing, granulomatous inflammation crescentic glomerulonephritis with few to no immune complexes frequent presence of circulating ANCA Since the introduction of effective treatment: opportunity for long term survival and follow-up recognition of relapse frequency and medication side effects exploration of immunologic mechanisms Questions for the future: why do we see this pattern of organ involvement? different tissues respond individually to injury and treatment why? are there triggers for disease and relapse? can treatment strategies be individualized based on these?
Rituximab treatment for ANCA-associated vasculitis in childhood
Rituximab treatment for ANCA-associated vasculitis in childhood DISCLOSURE I have no relevant financial relationships to disclose Katharine Moore MD Nov 14, 2012 University of Colorado School of Medicine
More informationANCA+ VASCULITIDES CYCAZAREM,
ANCA+ VASCULITIDES CYCAZAREM, q Comparison of 3 to 6 mo. oral CYC + CS then azathioprine or oral CYC for 12 mo.+ 10 mg/d CS. After 12 mo all the patients were treated with azathioprine q 150 patients followed
More informationAtlas of the Vasculitic Syndromes
CHAPTER e40 Atlas of the Vasculitic Syndromes Carol A. Langford Anthony S. Fauci Diagnosis of the vasculitic syndromes is usually based upon characteristic histologic or arteriographic findings in a patient
More informationTREATMENT OF ANCA-ASSOCIATED VASCULITIS
TREATMENT OF ANCA-ASSOCIATED VASCULITIS Loïc Guillevin Hôpital Cochin, Université Paris Descartes Cours DU, 11 mars 2016 1 Disclosure of interest regarding this presentation Roche has provided, in part,
More informationTell me more about vasculitis. Lisa Willcocks Consultant in Nephrology and Vasculitis, Addenbrooke s Hospital
Tell me more about vasculitis Lisa Willcocks Consultant in Nephrology and Vasculitis, Addenbrooke s Hospital Talk overview Case study ANCA-associated vasculitis What is ANCA vasculitis? What causes ANCA
More informationSMALL TO MEDIUM VASCULITIS: RENAL ASPECT RATANA CHAWANASUNTORAPOJ UPDATE IN INTERNAL MEDICINE 2018
SMALL TO MEDIUM VASCULITIS: RENAL ASPECT RATANA CHAWANASUNTORAPOJ UPDATE IN INTERNAL MEDICINE 2018 OUTLINE Renal involvement in vasculitis Curr Rheumatol Rep 2013 Renal involvement in ANCA vasculitis GN***:
More informationVasculitis. Edward Dwyer, M.D. Division of Rheumatology. Vasculitis
Edward Dwyer, M.D. Division of Rheumatology VASCULITIS is a primary inflammatory disease process of the vasculature Determinants of the Clinical Manifestations of : Target organ involved Size of vessel
More informationRecent advances in management of Pulmonary Vasculitis. Dr Nita MB
Recent advances in management of Pulmonary Vasculitis Dr Nita MB 23-01-2015 Overview of the seminar Recent classification of Vasculitis What is new in present classification? Trials on remission induction
More informationPAEDIATRIC VASCULITIS
PAEDIATRIC VASCULITIS Lawrence Owino Okong o, Mmed (UoN); Mphil. (UCT). Lecturer, Department of Paediatrics and Child Health, University of Nairobi. Paediatrician/ Rheumatologist. OUTLINE Introduction
More informationManagement of Acute Vasculitis. CMT teaching 3 rd June 2015 Caroline Wroe
Management of Acute Vasculitis CMT teaching 3 rd June 2015 Caroline Wroe Vasculitis pub quiz Match the date with the event Dr Peter McBride, Scottish Otolaryngologist describes a disease of rapid destruction
More informationANCA associated vasculitis in China
ANCA associated vasculitis in China Min Chen Renal Division, Peking University First Hospital, Beijing 100034, P. R. China 1 General introduction of AAV in China Disease spectrum and ANCA type Clinical
More informationDr Rodney Itaki Lecturer Anatomical Pathology Discipline. University of Papua New Guinea School of Medicine & Health Sciences Division of Pathology
Vasculitis Dr Rodney Itaki Lecturer Anatomical Pathology Discipline University of Papua New Guinea School of Medicine & Health Sciences Division of Pathology Disease Spectrum Hypersensitivity vasculitis/microscopic
More informationVASCULITIS. Case Presentation. Case Presentation
VASCULITIS Case Presentation The patient is a 24 year old woman who presented to the emergency room with left-sided weakness. She was confused and complained of a severe headache. She was noted to have
More informationanti-neutrophil cytoplasmic antibody, myeloperoxidase, microscopic polyangiitis, cyclophosphamide, corticosteroid
Online publication June 24, 2009 ANCA JMAAV 1 2 ANCA JMAAV MPO-ANCA 18 17 50 J Jpn Coll Angiol, 2009, 49: 53 61 anti-neutrophil cytoplasmic antibody, myeloperoxidase, microscopic polyangiitis, cyclophosphamide,
More informationTREATMENT OF ANCA-ASSOCIATED VASCULITIS AN UPDATE. Loïc Guillevin. Hôpital Cochin, Université Paris Descartes. DU MALADIES SYSTEMIQUES, 7 March 2014
TREATMENT OF ANCA-ASSOCIATED VASCULITIS AN UPDATE Loïc Guillevin Hôpital Cochin, Université Paris Descartes DU MALADIES SYSTEMIQUES, 7 March 2014 1 Disclosure of interest regarding this presentation Former
More informationRATIONALE. K Without therapy, ANCA vasculitis with GN is associated. K There is high-quality evidence for treatment with
http://www.kidney-international.org chapter 13 & 2012 KDIGO Chapter 13: Pauci-immune focal and segmental necrotizing glomerulonephritis Kidney International Supplements (2012) 2, 233 239; doi:10.1038/kisup.2012.26
More informationSHO Teaching. Dr. Amir Bhanji Consultant Nephrologist, Q.A hospital, Portsmouth
SHO Teaching Vasculitis Renal medicine Dr. Amir Bhanji Consultant Nephrologist, Q.A hospital, Portsmouth OUTLINE What is vasculitis Causes Classification Brief look into ANCA Associated Vasculitis (AAV)
More informationVascularites rénales associées aux ANCA
Vascularites rénales associées aux ANCA Société Médicale des Hôpitaux de Paris 16 Mars 2012 Philippe Vanhille Néphrologie et Médecine Interne Hôpital de Valenciennes Classification of systemic vasculitis:
More informationCase Presentation VASCULITIS. Case Presentation. Case Presentation. Vasculitis
Case Presentation VASCULITIS The patient is a 24 year old woman who presented to the emergency room with left-sided weakness. She was confused and complained of a severe headache. She was noted to have
More informationANCA-associated vasculitis. Vladimir Tesar Department of Nephrology, General University Hospital, Prague, Czech Republic
ANCA-associated vasculitis Vladimir Tesar Department of Nephrology, General University Hospital, Prague, Czech Republic Disclosure of Interests Abbvie, Amgen, Baxter, Bayer, Boehringer-Ingelheim, Calliditas,
More informationSmall Vessel Vasculitis
Banff- Rocky Mountain Barry Kassen, MD, FRCPC,FACP Head, Division of Internal Medicine UBC/VGH/SPH Acting Head, Division of Community Internal Medicine November, 2009 Objectives 1. To understand small
More informationWegener s Granulomatosis JUN-KI PARK
Wegener s Granulomatosis JUN-KI PARK Definition History Epidemiology Clinical symptoms Pathophysiology Treatment Wegener granulomatosis (WG) is a complex, immunemediated disorder, which along with microscopic
More informationCase Rep Nephrol Urol 2013;3: DOI: / Published online: January 27, 2013
Published online: January 27, 2013 1664 5510/13/0031 0016$38.00/0 This is an Open Access article licensed under the terms of the Creative Commons Attribution- NonCommercial-NoDerivs 3.0 License (www.karger.com/oa-license),
More informationEULAR/ERA-EDTA recommendations for the management of ANCAassociated
EULAR/ERA-EDTA recommendations for the management of ANCAassociated vasculitis Dr. Meharunnisha Syed III year DNB Resident (General Medicine) Narayana Health-MSH Fifteen recommendations were developed,
More informationReview Article. Current concepts in Anti-Neutrophil Cytoplasmic Antibody (ANCA) associated Vasculitis. Milind Aurangabadkar
Review Article Vidarbha Journal of Internal Medicine Volume 23 July 207 Current concepts in Anti-Neutrophil Cytoplasmic Antibody (ANCA) associated Vasculitis Milind Aurangabadkar ABSTRACT Anti-neutrophil
More informationUpdate on Wegener granulomatosis
MEDICAL GRAND ROUNDS TAKE-HOME POINTS FROM LECTURES BY CLEVELAND CLINIC AND VISITING FACULTY Update on Wegener granulomatosis CAROL A. LANGFORD, MD, MHS Director, Center for Vasculitis Care and Research,
More informationMohammad Reza Shakibi M.D Kerman university of medical sciences (KMU) Shafa Hospital, Rheumatology ward
VASCULITIS SYNDROMES Mohammad Reza Shakibi M.D Kerman university of medical sciences (KMU) Shafa Hospital, Rheumatology ward ILLUSTRATED CASE 1 A 56 years old lady refered me for prolonged fever, arthritis
More informationGRANULOMATOSIS WITH POLYANGIITIS
What is granulomatosis with polyangiitis (GPA)? Granulomatosis with polyangiitis (GPA) is a form of vasculitis a family of rare disorders characterized by inflammation of the blood vessels, which can restrict
More informationSmall Vessel Vasculitis
Small Vessel Vasculitis Paul A Brogan Professor of Vasculitis and Consultant Paediatric Rheumatologist Department of Rheumatology Institute of Child Health and Great Ormond St Hospital, London UK P.brogan@ucl.ac.uk
More informationVasculitis local: systemic
Vasculitis Inflammation of the vessel wall. Signs and symptoms: 1- local: according to the involved tissue 2- systemic:(fever, myalgia, arthralgias, and malaise) Pathogenesis 1- immune-mediated 2- infectious
More informationNew Evidence reports on presentations given at EULAR Rituximab for the Treatment of Rheumatoid Arthritis and Vasculitis
New Evidence reports on presentations given at EULAR 2011 Rituximab for the Treatment of Rheumatoid Arthritis and Vasculitis Report on EULAR 2011 presentations Anti-TNF failure and response to rituximab
More informationUpdate and Review on Vasculitis. Ramona Raya, MD ACP Internal Medicine Congress August 7, 2015
Update and Review on Vasculitis Ramona Raya, MD ACP Internal Medicine Congress August 7, 2015 Outline Classification and nomenclature updates Small vessel vasculitis Pathophysiology Diagnosis Management/therapeutic
More informationSupplementary Appendix
Supplementary Appendix This appendix has been provided by the authors to give readers additional information about their work. Supplement to: Jones RB, Cohen Tervaert JW, Hauser T, et al. Rituximab versus
More informationVasculitis local: systemic
Vasculitis Inflammation of the vessel wall. Signs and symptoms: 1- local: according to the involved tissue 2- systemic:(fever, myalgia, arthralgias, and malaise) Pathogenesis 1- immune-mediated inflammation
More informationYear In Review: VasculitisPers. Disclosures. Learning Objectives. none 4/16/2018. Describe new medications for the treatment of vasculitis
Year In Review: VasculitisPers Cailin Sibley, M.D., M.H.S. Director, Vasculitis Clinic April 27 th, 2018 NTEREST DISCLOSURE Disclosures none Learning Objectives Describe new medications for the treatment
More informationClinical Case Conference Tuesday August 25, 2015 Dana Assis, MD
Clinical Case Conference Tuesday August 25, 2015 Dana Assis, MD CC: LE swelling and pain for three weeks HPI: 71F hx HTN, DM (A1c 9.6), HFrEF (45%), asthma, OA, cryptogenic cirrhosis p/w bilateral lower
More informationCHECK LIST FORM-MONTH 27 (Please note Month 27 is from enrolment not randomisation)
CHECK LIST FORM-MONTH 27 (Please note Month 27 is from enrolment not randomisation) Participant Initials: Date of Birth: Were the following forms completed for this visit? Follow Up Form Done t Done BVASWG
More informationEUVAS update June 5 th Marinka Twilt
EUVAS update June 5 th 2012 Marinka Twilt Chapel Hill 2012 Classification Large Vessel Vasculitis (LVV) Medium Vessel Vasculitis (MVV) Small Vessel Vasculitis (SVV) Variable Vessel Vasculitis (VVV) Single
More informationScleritis LEN V KOH OD
Scleritis LEN V KOH OD 2014 PUCO 1 Introduction A painful, destructive, and potentially blinding disorder Highly symptomatic High association with systemic disease Immunosuppresssive agents 2014 PUCO 2
More informationRheumatology Primer: What Labs and When
Rheumatology Primer: What Labs and When Irina Konon, MD Department of Internal Medicine Division of Rheumatology Medical College of Wisconsin Disclosures None 1 Objective Discuss principles of laboratory
More informationProtocol Version 2.0 Synopsis
Protocol Version 2.0 Synopsis Title Short Title Plasma exchange and glucocorticoid dosing in anti-neutrophil cytoplasm antibody associated vasculitis: a randomized controlled trial. PEXIVAS PEXIVAS Clinical
More informationClinical Commissioning Policy: Rituximab For ANCA Vasculitis. December Reference : NHSCB/ A3C/1a
Clinical Commissioning Policy: Rituximab For ANCA Vasculitis December 2012 Reference : NHSCB/ A3C/1a NHS Commissioning Board Clinical Commissioning Policy: Rituximab For The Treatment Of Anti-Neutrophil
More informationEveryday Vasculitis (or what questions do we get asked most!) Lucy Smyth Renal Consultant
Everyday Vasculitis (or what questions do we get asked most!) Lucy Smyth Renal Consultant What is it? Why have I got it? How can we treat it? Why do I feel like I do? What do the blood tests mean? Will
More informationA COST EFFECTIVENESS ANALYSIS OF WEEKLY COMPLETE BLOOD COUNT MONITORING FOR LEUKOPENIA IN PATIENTS WITH GRANULOMATOSIS WITH POLYANGIITIS (GPA) ON
A COST EFFECTIVENESS ANALYSIS OF WEEKLY COMPLETE BLOOD COUNT MONITORING FOR LEUKOPENIA IN PATIENTS WITH GRANULOMATOSIS WITH POLYANGIITIS (GPA) ON CYCLOPHOSPHAMIDE By ATUL ASHOK KHASNIS, MD Submitted in
More informationApproach to Glomerular Diseases: Clinical Presentation Nephrotic Syndrome Nephritis
GLOMERULONEPHRITIDES Vivette D Agati Jai Radhakrishnan Approach to Glomerular Diseases: Clinical Presentation Nephrotic Syndrome Nephritis Heavy Proteinuria Renal failure Low serum Albumin Hypertension
More informationThe systemic vasculitides were traditionally classified. Treatment of ANCA-associated Systemic Vasculitis. H. Michael Belmont, M.D.
60 Treatment of ANCA-associated Systemic Vasculitis H. Michael Belmont, M.D. Abstract The antineutrophil cytoplasmic antibodies (ANCA)-associated small vessel vasculitides include Wegener s granulomatosis,
More informationDecember 6, 2010 Asthma and Rheumatic Disorders and Vasculitis
December 6, 2010 Asthma and Rheumatic Disorders and Vasculitis Lanny J. Rosenwasser, M.D. Dee Lyons/Missouri Endowed Chair in Immunology Research Professor of Pediatrics Allergy-Immunology Division Childrens
More informationDiagnostic Procedures for Vasculitis
Diagnostic Procedures for Vasculitis Toshiharu Matsumoto, MD Clinical Professor of Department of Diagnostic Pathology Juntendo University Nerima Hospital, Tokyo, Japan Introduction In 1994, the International
More informationAN OVERVIEW OF ANCA-ASSOCIATED VASCULITIS
GRANULOMATOSIS WITH POLYANGIITIS (GPA), MICROSCOPIC POLYANGIITIS (MPA), and EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA) AN OVERVIEW OF ANCA-ASSOCIATED VASCULITIS What is ANCA-associated Vasculitis?
More informationGlucocorticoids and Relapse and Infection Rates in Anti-Neutrophil Cytoplasmic Antibody Disease
Article Glucocorticoids and Relapse and Infection Rates in Anti-Neutrophil Cytoplasmic Antibody Disease JulieAnne G. McGregor, Susan L. Hogan, Yichun Hu, Caroline E. Jennette, Ronald J. Falk, and Patrick
More informationThe Vasculitis Syndromes
The Vasculitis Syndromes Definition Inflammation and damage of blood vessels Single organ skin Several organ systems Primary Secondary Heterogeneity Overlap Primary Vasculitis Syndromes Wegener s granulomatosis
More informationManaging Acute Medical Problems, Birmingham Vasculitis. David Jayne. University of Cambridge
Managing Acute Medical Problems, Birmingham 2016 Vasculitis David Jayne University of Cambridge Disclosures Astra Zeneca, Aurinia, BIOGEN, Boehringer, Chemocentryx, Genzyme/Sanofi, GSK, Lilly, Medimmune,
More informationVASCULITIC SYNDROMES. Howard L. Feinberg, D.O., F.A.C.O.I., F.A.C.R. OPSC 2018
VASCULITIC SYNDROMES Howard L. Feinberg, D.O., F.A.C.O.I., F.A.C.R. OPSC 2018 2012 REVISED CHAPEL HILL CONSENSUS CONFERENCE Large vessel Takayasu arteritis Giant cell arteritis Medium Vessel Polyarteritis
More informationEvidence-based therapy for the ANCAassociated vasculitides: what do the trials. show so far? Clinical Trial Outcomes
associo far? Evidence-based therapy for the ANCAassociated vasculitides: what do the trials show so far? Anti-neutrophil cytoplasm antibody-associated vasculitides are rare multisystem inflammatory diseases
More informationEfficacy and Safety of Rituximab in the Treatment of Rheumatoid Arthritis and ANCA-associated Vasculitis
New Evidence reports on presentations given at ACR/ARHP 2010 Efficacy and Safety of Rituximab in the Treatment of Rheumatoid Arthritis and ANCA-associated Vasculitis Report on ACR/ARHP 2010 presentations
More informationPlasma exchanges in ANCA-associated vasculitis
Plasma exchanges in ANCA-associated vasculitis Xavier Puéchal, MD, PhD Centre de Référence des Maladies auto-immunes systémiques rares d Ile de France Hôpital Cochin Université Paris Descartes http://www.vascularites.org
More informationEDITORIAL. Issue Seventeen, October Editorial Team. Issue Seventeen. Info link
EDITORIAL, October 2004 Welcome to the Spring 2004 Edition of InfoLink. The feature article in this edition has been written by Dr Rodger Laurent, Head of Department, PaLMS Rheumatology Laboratory. The
More informationRevised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis.
Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Bossuyt X, Cohen Tervaert JW, Arimura Y, Blockmans D, Flores-Suárez LF, Guillevin
More informationRecent Evidence in VasculitIs ScIence and Treatment. Management of AAV in the clinical setting
Recent Evidence in VasculitIs ScIence and Treatment Management of AAV in the clinical setting LEARNING OBJECTIVES At the end of this educational program, the participants should be able to: 1. Make a diagnosis
More informationTITLE: Rituximab for Granulomatosis with Polyangiitis or Microscopic Polyangiitis: A Review of the Clinical and Cost-effectiveness
TITLE: Rituximab for Granulomatosis with Polyangiitis or Microscopic Polyangiitis: A Review of the Clinical and Cost-effectiveness DATE: 28 January 2015 CONTEXT AND POLICY ISSUES Granulomatosis with polyangiitis
More informationRate of infections in severe necrotising vasculitis patients treated with cyclophosphamide induction therapy: a meta-analysis
Rate of infections in severe necrotising vasculitis patients treated with cyclophosphamide induction therapy: a meta-analysis M. Jung 1, L. Barra 2 1 Division of Rheumatology, Department of Medicine, University
More informationANCA-associated systemic vasculitis (AASV)
PAPER 2007 Royal College of Physicians of Edinburgh ANCA-associated systemic vasculitis (AASV) 1 DC Kluth, 2 J Hughes 1 Reader in Nephrology, MRC Centre for Inflammation Research, University of Edinburgh,
More informationCHECK LIST FORM-SCREENING
CHECK LIST FORM-SCREENING Participant Initials: Date of Birth: Evaluation Date: Were the following forms completed for this visit? Eligibility Form Done t Done Baseline medical History Form Done t Done
More informationJones slide di 23
1 di 23 The patient with ANCA- associated vasculitis and pulmonary haemorrhage Rachel B Jones, Cambridge, UK Chairs:Hans-Joachim Anders, Munich, Germany Vladimir Tesar, Prague, Czech Republic Prof. Rachel
More informationVasculitis and Vasculitides. OMONDI OYOO Physician/Rheumatologist; Senior Lecturer, Department of Medicine University of Nairobi
Vasculitis and Vasculitides OMONDI OYOO Physician/Rheumatologist; Senior Lecturer, Department of Medicine University of Nairobi Definition Presence of leucocytes in the vessel wall with reactive damage
More informationPharmacy Management Drug Policy
SUBJECT: : Nucala (mepolizumab), Cinqair (reslizumab), & Fasenra (benralizumab) POLICY NUMBER: Pharmacy-62 EFFECTIVE DATE: 12/15 LAST REVIEW DATE: 3/5/2018 If the member s subscriber contract excludes
More informationVasculitis (Polyarteritis Nodosa, Microscopic Polyangiitis, Wegener s Granulomatosis, Henoch- Schönlein Purpura)
Vasculitis (Polyarteritis Nodosa, Microscopic Polyangiitis, Wegener s Granulomatosis, Henoch- Schönlein Purpura) J. Charles Jennette Ronald J. Falk The kidneys are affected by a variety of systemic vasculitides
More informationVascularites associées aux ANCA Traitement par le RITUXIMAB
Vascularites associées aux ANCA Traitement par le RITUXIMAB Philippe Vanhille Néphrologie Médecine Interne Hôpital de Valenciennes Aix- en- Provence 2013 Cyclophosphamide therapy of severe systemic necrotizing
More informationAntineutrophil cytoplasm antibody associated vasculitis: recent developments
mini review http://www.kidney-international.org & 2013 International Society of Nephrology Antineutrophil cytoplasm antibody associated vasculitis: recent developments Shunsuke Furuta 1 and David R.W.
More informationDiagnostic Tests in Rheumatic Disease: What s Old, What s New & What s Useful? COPYRIGHT
Diagnostic Tests in Rheumatic Disease: What s Old, What s New & What s Useful? Robert H. Shmerling, M.D. Beth Israel Deaconess Medical Center Boston, MA Diagnostic Tests in Rheumatic Disease: What's Old,
More informationDone by: Shatha Khtoum
Done by: Shatha Khtoum Overview Vasculitis -Vasculitis is a general term for vessel wall inflammation -Symptoms and signs depend on the tissue of which the vessels are affected. (slide 2) -There are usually
More informationOverview. = inflammation of vessel wall. Symptoms and signs depend on the tissue of which the vessels are affected
Vasculitis (1+2) Overview = inflammation of vessel wall Symptoms and signs depend on the tissue of which the vessels are affected Often with systemic symptoms fever, myalgia, arthralgia, malaise etc. Most
More informationA. Smržová. III. Interní klinika FN Olomouc nefrologická, revmatologická a endokrinologická
A. Smržová III. Interní klinika FN Olomouc nefrologická, revmatologická a endokrinologická Systemic vasculitis destructive inflamatory of the walls of blood vessels. Pathologist Inflammatory destruction
More informationCHECK LIST FORM-SCREENING
CHECK LIST FORM-SCREENING Participant Initials: Date of Birth: Evaluation Date: Were the following forms completed for this visit? Eligibility Form Done t Done Baseline medical History Form Done t Done
More informationGIANT CELL ARTERITIS. Page 1 of 6 Reproduction of this material requires written permission of the Vasculitis Foundation. Copyright 2018.
What is giant cell arteritis (GCA)? Giant cell arteritis (GCA) is a form of vasculitis a family of rare disorders characterized by inflammation of the blood vessels, which can restrict blood flow and damage
More informationWhere a licence is displayed above, please note the terms and conditions of the licence govern your use of this document.
Rituximab for treatment of severe renal disease in ANCA associated vasculitis Geetha, Duvuru; Hruskova, Zdenka; Segelmark, Marten; Hogan, Jonathan; Morgan, Matthew; Cavero, Teresa; Eriksson, Per; Seo,
More informationa mimicker of Wegener s Granulomatosis
a mimicker of Wegener s Granulomatosis Combined Meeting October 2009 a story of 2 ladies Madam JA 56 year-old Madam RH 36 year-old Madam JA 56 year-old Apr 2008 May Jun Jul Aug Sept Oct Nov 2008 Madam
More informationDisclosures. Objectives. Vasculitis: What The Primary Care Physician Needs To Know. Definition & Classification of Vasculitis
Vasculitis: What The Primary Care Physician Needs To Know Lynn Fussner, MD Assistant Professor - Clinical Division of Pulmonary, Critical Care, and Sleep Medicine The Ohio State University Wexner Medical
More informationVasculitis: What The Primary Care Physician Needs To Know. Lynn Fussner, MD
Vasculitis: What The Primary Care Physician Needs To Know Lynn Fussner, MD Assistant Professor - Clinical Division of Pulmonary, Critical Care, and Sleep Medicine The Ohio State University Wexner Medical
More informationRARE JUVENILE PRIMARY SYSTEMIC VASCULITIS
www.pediatric-rheumathology.printo.it RARE JUVENILE PRIMARY SYSTEMIC VASCULITIS WHAT IS IT? Vasculitis is an inflammation of blood vessels. Vasculitides cover a wide group of diseases. Primary vasculitis
More informationNew Developments in ANCA-associated Vasculitis
New Developments in ANCA-associated Vasculitis Second International Renal Conference Bruges, March 2018 Ulrich Specks, M.D. Connor Group Foundation Professor of Medicine Mayo Clinic College of Medicine
More informationANCA Associated vasculitis presenting as bilateral pleural effusion: A rare case report
International Journal of Current Research in Medical Sciences ISSN: 2454-5716 P-ISJN: A4372-3064, E -ISJN: A4372-3061 www.ijcrims.com Case Report Volume 3, Issue 10-2017 DOI: http://dx.doi.org/10.22192/ijcrms.2017.03.10.015
More informationGranulomatosis with Polyangiitis (Wegener s)
August 2011 Granulomatosis with Polyangiitis (Wegener s) Andrew Noll, Harvard Medical School, Year III Agenda Patient presentation Overview of granulomatosis with polyangiitis (Wegener s), abbreviated
More informationUNFOLDING NATURE S ORIGAMI: MEDICAL TREATMENT OF TAKAYASU ARTERITIS AND GIANT CELL ARTERITIS
UNFOLDING NATURE S ORIGAMI: MEDICAL TREATMENT OF TAKAYASU ARTERITIS AND GIANT CELL ARTERITIS CanVasc meeting Montreal Nov 22 2012 Patrick Liang Service de rhumatologie Centre Hospitalier Universitaire
More informationVasculitis Prof. Dr. med. Katharina Glatz Pathologie
Vasculitis 08-21-2018 Prof. Dr. med. Katharina Glatz Pathologie Agenda Anatomy and histology Vasculitis: Chapel Hill Classification Examples Giant cell arteritis Single organ vasculitis Artery or Vein?
More informationUpdate in Vasculitis. Anatomy of vasculitis. General Principles of Vasculitis. Not necessarily as rare as one might think
Black Hole Vasculitis Update in Vasculitis Advances In Internal Medicine 2015 Jonathan Graf, MD Professor of Clinical Medicine, UCSF Division of Rheumatology, SFGH Rare Poorly understood mystery of universe
More informationCENTRAL NERVOUS SYSTEM VASCULITIS
What is central nervous system (CNS) vasculitis? Central nervous system (CNS) vasculitis is among a family of rare disorders characterized by inflammation of the blood vessels, which restricts blood flow
More informationTreatment of Severe Renal Disease in ANCA Positive and Negative Small Vessel Vasculitis with Rituximab
American Journal of Nephrology Original Report: Patient-Oriented, Translational Research Am J Nephrol 2015;41:296301 Received: March 12, 2015 Accepted: May 6, 2015 Published online: June 2, 2015 Treatment
More informationFAQ Identifying and enrolling participants
FAQ Identifying and enrolling participants WHO IS ELIGIBLE - CASES? Patients with a new diagnosis of primary systemic vasculitis Patients suitable as cases are over 18 years with a new presentation or
More informationA number of factors point to the likelihood of a person with RA developing RV:
What is rheumatoid vasculitis (RV)? Rheumatoid vasculitis (RV) is a rare but serious complication of rheumatoid arthritis (RA) an inflammatory disease that causes severe joint pain and damage, and can
More informationGranulomatosis with Polyangiitis (Wegener s) and Review of Latest Updates on Pathogenesis and Treatment
Proceedings of UCLA Healthcare -VOLUME 19 (2015)- CLINICAL REPORT Granulomatosis with Polyangiitis (Wegener s) and Review of Latest Updates on Pathogenesis and Treatment Jimmy L. Zhao, Ph.D.; G. Xon Ng,
More informationT he primary systemic vasculitides (PSV) are clinically
605 EXTENDED REPORT EULAR recommendations for conducting clinical studies and/ or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis Bernhard Hellmich,
More informationOptimizing the Therapeutic Strategies in ANCA-Associated Vasculitis Single Centre Experience with International Randomized Trials
Prague Medical Report / Vol. 107 (2006) No. 2, p. 199 212 199) Optimizing the Therapeutic Strategies in ANCA-Associated Vasculitis Single Centre Experience with International Randomized Trials Vaňková
More informationCombined Infliximab and Rituximab in Necrotising Scleritis
This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs 3.0 License (www.karger.com/oa-license), applicable to the online version of the article
More informationTechnology appraisal guidance Published: 26 March 2014 nice.org.uk/guidance/ta308
Rituximab in combination with glucocorticoids for treating antineutrophil cytoplasmic antibody- associated vasculitis Technology appraisal guidance Published: 26 March 2014 nice.org.uk/guidance/ta308 NICE
More informationLupus Related Kidney Diseases. Jason Cobb MD Assistant Professor Renal Division Emory University School of Medicine October 14, 2017
Lupus Related Kidney Diseases Jason Cobb MD Assistant Professor Renal Division Emory University School of Medicine October 14, 2017 Financial Disclosures MedImmune Lupus Nephritis Kidney Biopsy Biomarkers
More informationVCRC-OMERACT ANCA-Vasculitis Outcome Measure Initiative Comparative Outcome Measure Exercise-Training Cases
Introduction to Training Cases The purpose of this preliminary exercise is to familiarize you with the rules and logistics of the five instruments under study. Please do not read the Training Cases until
More informationVasculitides in Surgical Neuropathology Practice
Vasculitides in Surgical Neuropathology Practice USCAP requires that all faculty in a position to influence or control the content of CME disclose any relevant financial relationship WITH COMMERCIAL INTERESTS
More informationVasculitis Update. A selective review of what s new. Dr Jonathan Akikusa MBBS FRACP
Vasculitis Update A selective review of what s new Dr Jonathan Akikusa MBBS FRACP Consultant Paediatric Rheumatologist Royal Children s Hospital, Melbourne Honorary Research Fellow Murdoch Children s Research
More information