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Interesting case conference A woman with hemoptysis and renal failure ประจำวันที่  27  ตุลาคม  2553 นำเสนอโดย :  นพ . ปรัชญา ภัสสรานนท์
History ,[object Object],[object Object],[object Object]
Underlying  disease ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Present  illness ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],4.7  7300  N 81% 15  100,000  L 16% ,[object Object],[object Object]
Past  history ,[object Object],[object Object],[object Object],[object Object],[object Object]
Physical  examination ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Physical  examination ,[object Object],[object Object],[object Object],[object Object],[object Object]
Problem list ,[object Object],[object Object],[object Object],[object Object]
21/01/51  28/09/53  30/09/53  3/10/53 11/10/53  16/10/53  21/10/53  26/10/53
21/01/51  28/09/53  30/09/53  3/10/53 11/10/53  16/10/53  21/10/53  26/10/53
21/01/51  28/09/53  30/09/53  3/10/53 11/10/53  16/10/53  21/10/53  26/10/53
21/01/51  28/09/53  30/09/53  3/10/53 11/10/53  16/10/53  21/10/53  26/10/53
CBC 99 / 4.5 89 / 4.3 89 / 4.2 86 / 4.0 53 / 4.3 5.8 / 0.5 BUN/Cr 28 PTT 12 PT 26.6 25.9 26.4 26.1 12.3 22.4 RDW 81.9 77.3 75.3 75.8 83 93 61 MCV 103,000 100,000 64,000 30,000 100,000 136,000 225,000 Platelet 4% 6% 5% 10% 16 19% 7% L% 93% 91% 91% 84% 81 70% 85% N% 10800 9760 8230 10310 7300 4100 7830 WBC 26.1 26.9 23.5 29.1 15 31.2 22.6 Hct 2/10/53 1/10/53 30/9/53 28/9/53 21/9/53 27/10/52 22/12/51
Lab   – referer hospital ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Lab ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Lab ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
What to do next? Bronchoscope? CT scan? Dexamethasone? hemodialysis? Plasma exchange? Continue antibiotic? Kidney biopsy? Doppler ultrasound? Consult nephro? V/Q scan? CPR? Factor VIIa ? Echo? Cyclophosphamide? S p u t u m   P C R - T B
28 29 30 1 2 3 4 Pip/Taz FOB
Bronchoscope (30/9/53) ,[object Object],[object Object],[object Object],[object Object],[object Object]
What to do next? Bronchoscope? CT scan? Dexamethasone? hemodialysis? Plasma exchange? Continue antibiotic? Kidney biopsy? Doppler ultrasound? Consult nephro? V/Q scan? CPR? Factor VIIa ? Echo? Cyclophosphamide? S p u t u m   P C R - T B
Management ,[object Object],[object Object],[object Object]
28 25 11 18 4 Pip/Taz dexa 5q8 meropenem colistin// colistin Methyl P x3 GTC PRES WBC  , schistocyte PT,PTT   , haptoglobin <10 Sputum – A.baum tracheostomy levofloxacin dexa 5q6 vanco ampho dexa 5q12 Sputum – MRSA  yeast P.Ex x7
 
Hct WBC Plt
21/01/51  28/09/53  30/09/53  3/10/53 11/10/53  16/10/53  21/10/53  26/10/53  ไข้  Sputum – A.baum, S.maltophilia
21/01/51  28/09/53  30/09/53  3/10/53 11/10/53  16/10/53  21/10/53  26/10/53
21/01/51  28/09/53  30/09/53  3/10/53 11/10/53  16/10/53  21/10/53  26/10/53  ยังมีเสมหะปนเลือด  ,Hct 25  Cr 3.9
21/01/51  28/09/53  30/09/53  3/10/53 11/10/53  16/10/53  21/10/53  26/10/53  รู้ตัวดี ไม่มีไข้  Sputum - MRSA
Microscopic polyangiitis
MPA ,[object Object]
History ,[object Object],[object Object],[object Object],[object Object],[object Object],Rheum Dis Clin North Am . 2010 Aug;36(3):545-58
1990 ACR classification criteria ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Wegener’s granulomatosis Polyarteritis nodosa
Polyarteritis nodosa vs. microscopic polyangiitis  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Microscopic polyangiitis Polyarteritis nodosa
1994 Chapel Hill nomenclature Arthritis Rheum . 1994 Feb;37(2):187-92.  Microscopic polyangiitis
Clincical Dx primary vasculitis WG surrogate marker Upper airway - bloody nasal discharge/crusting > 1 mo. - sinusitis, otitis media, mastoiditis > 3 mo. - retro-orbital mass or inflammation - subglottic stenosis - Saddle nose, destructive sinonasal disease Lower airway - fixed pulm. infiltrates, nodules, cavity > 1 mo. - bronchial stenosis Ann Rheum Dis . 2007 February;  66 (2):  222–227   classificaton algorithm
What is ANCA  ? ,[object Object],[object Object],[object Object],CHEST 2009;136:1101–1111
Indirect immunofluorescence assay
cANCA
Granulocyte antigen of ANCA MPA, SLE, RA,  UC, CD, PSC, AIH  Clin Exp Rheumatol 2000: 18: 629-635.
Problem with pANCA detection Perinuclear staining by ANA granulocyte-specific ANA? Epitope conformation
does ANCA has role in pathogenesis  ? ,[object Object],[object Object],[object Object],[object Object],[object Object],Nat Rev Rheumatol . 2010 Nov;6(11):653-64.
does ANCA has role in pathogenesis ? I have no MPO Anti-MPO  Pauciimmune NCGN Systemic vasculitis lipopolysaccharide Now I have MPO Anti-PR3  and  pauciimmune glomerulonephritis ! Normal mice reduced leukocyte rolling  augmented adhesion and transmigration  across the endothelium Nat Rev Rheumatol . 2010 Nov;6(11):653-64.
What does ANCA do with body  ? Kelly’s textbook of Rheumatology, 8 th  ed.
Nat Rev Rheumatol . 2010 Nov;6(11):653-64.
Nat Rev Rheumatol . 2010 Nov;6(11):653-64.
RITUXIVAS N Engl J Med . 2010 Jul 15;363(3):211-20  N Engl J Med . 2010 Jul 15;363(3):221-32.  RAVE Steroid Rituximab   x 4 weeks IVCY x 2 pulses Steroid IVCY x 3-6 mo. Azathioprine New Dx AAV and renal involvement Non-blind RCT   severe new or relapse 148 WG, 24 MPA double - dummy, noninferiority db-RCT Stone JH.  Massachusetts General Hospital  . USA Jones RB.  Addenbrooke's Hospital . UK  Pulse + pred 5 mo. Rituximab x 4 weeks placebo CY Pulse + pred 5 mo. oral CY x 3-6 mo. placebo Rituximab Placebo Aza Azathioprine noninferior I M 18% 18% mortality 36% 42% Severe AE 82% 76% remission Cy (11) Ritu (33) 62%  67% MPA 41% * 57% DAH pt. 42% * 67% relapse pt. 53%  64% Remission   without pred Cy (98) Ritu (99) 66% 66% Renal 24% 27% DAH 54% 52% Pulmonary Cy (98) Ritu (99)
Where ANCA come frome  ? Nat Rev Rheumatol . 2010 Nov;6(11):653-64.
Epidemiology ,[object Object],Rheum Dis Clin North Am . 2010 Aug;36(3):545-58
Clinical presentation ,[object Object],[object Object],[object Object],Murray and Nadel’s Textbook of Respiratory Medicine, 5 th  ed.
Autoimmun Rev . 2010 Oct;9(12):812-9
Clinical presentation Murray and Nadel’s Textbook of Respiratory Medicine, 5 th  ed. 10–50% of patients .  Mononeuritis multiplex reported cerebral infarction  Neuro 35–60% of patients .  Commonly purpura skin 35–55% of patients .  Pain, bleeding, ischemia .  Rare visceral aneurysms .  GI 10–15% of patients .  CHF and pericarditis have been described .  Cardiac 0–30% of patients .  Scleritis, episcleritis, uveitis .  eyes Arthralgias and myalgias in at least 50% of patients   musculoskeletal 5–30% of patients, with sinus disease most common   Upper airway Segmental necrotizing glomerulonephritis and RPGN is almost universal .  renal 25–55% of patients .  10–30% with diffuse alveolar hemorrhage .  Fibrosis, focal infiltrates, effusions, pulmonary arterial hypertension pulmonary
Pathology Neutrophilic capillaritis   without granuloma,   fibrinoid necrosis and arterial wall disruption, intra alveolar  RBC  and hemosiderin-laden macrophages Focal segmental necrotizing glomerulonephritis
Murray and Nadel’s Textbook of Respiratory Medicine, 5 th  ed.
Imaging of pulmonary vasculitis ,[object Object],[object Object],J Comput Assist Tomogr . 2004 Sep-Oct;28(5):710-6  CT finding in 51  MPO-ANCA patients
 
Radiology . 2010 May;255(2):322-41
Radiographics . 2002 Jul-Aug;22(4):739-64 consolidation with interlobular septal thickening Ground-glass opacity
fine reticular pattern superimposed on a background of groundglass attenuation that is the crazy-paving pattern J Comput Assist Tomogr . 2004 Sep-Oct;28(5):710-6
F 74y MPO. F/U HRCT show new honeycombing at 5 months 3 mo.
M 60Y MPO resemble IPF CT  show GGO and reticulation in subpleural region VAT Bx  shows irregular interstitial fibrotic thickening, hemosiderin laden macrophages  Radiology . 2010 May;255(2):322-41
Prognosis of MPA ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Murray and Nadel’s Textbook of Respiratory Medicine, 5 th  ed.
Arthritis Rheum . 1999 Mar;42(3):421-30
Prognosis of MPA with DAH ,[object Object],[object Object],[object Object],Murray and Nadel’s Textbook of Respiratory Medicine, 5 th  ed.
Treatment – systemic vasculitis ,[object Object],[object Object],Chest . 2006 Feb;129(2):452-65.
Treatment - MPA ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Induction Maintenance WG MPA oral cyclophophamide+bactrim steroid oral CY
Induction Maintenance WG MPA oral cyclophophamide+bactrim steroid pulse IVCY     less SE Azathioprine = oral CY Methotrexate = Aza MTX = oral CY  non-organ threatening AAV NORAM 2005 CYCLOPS 2009 Rituximab = IVCY RITUXIVAS 2010 Rituximab > oral CY relapse AAV, DAH RAVE 2010 PE > methylpred severe renal AAV MEPEX 2007 Etanercept add-on  WGET 2005 oral CY CYCAZAREM 2003 Pagnoux 2008 Leflunomide > MTX more AE Metzler 2007 MMF > IVCY mild renal MPA IMPROVE (terminated) Weixin 2010 open RCT n=35 MMF < Aza Bactrim add on Nat Rev Rheumatol.  2010 Nov;6 ( 11 ): 653-64 .
Treatment - DAH ,[object Object],[object Object],[object Object],[object Object],[object Object]
Thank You
Further readings ,[object Object],[object Object],[object Object]

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case microscopic polyangiitis

  • 1. Interesting case conference A woman with hemoptysis and renal failure ประจำวันที่ 27 ตุลาคม 2553 นำเสนอโดย : นพ . ปรัชญา ภัสสรานนท์
  • 2.
  • 3.
  • 4.
  • 5.
  • 6.
  • 7.
  • 8.
  • 9. 21/01/51 28/09/53 30/09/53 3/10/53 11/10/53 16/10/53 21/10/53 26/10/53
  • 10. 21/01/51 28/09/53 30/09/53 3/10/53 11/10/53 16/10/53 21/10/53 26/10/53
  • 11. 21/01/51 28/09/53 30/09/53 3/10/53 11/10/53 16/10/53 21/10/53 26/10/53
  • 12. 21/01/51 28/09/53 30/09/53 3/10/53 11/10/53 16/10/53 21/10/53 26/10/53
  • 13. CBC 99 / 4.5 89 / 4.3 89 / 4.2 86 / 4.0 53 / 4.3 5.8 / 0.5 BUN/Cr 28 PTT 12 PT 26.6 25.9 26.4 26.1 12.3 22.4 RDW 81.9 77.3 75.3 75.8 83 93 61 MCV 103,000 100,000 64,000 30,000 100,000 136,000 225,000 Platelet 4% 6% 5% 10% 16 19% 7% L% 93% 91% 91% 84% 81 70% 85% N% 10800 9760 8230 10310 7300 4100 7830 WBC 26.1 26.9 23.5 29.1 15 31.2 22.6 Hct 2/10/53 1/10/53 30/9/53 28/9/53 21/9/53 27/10/52 22/12/51
  • 14.
  • 15.
  • 16.
  • 17. What to do next? Bronchoscope? CT scan? Dexamethasone? hemodialysis? Plasma exchange? Continue antibiotic? Kidney biopsy? Doppler ultrasound? Consult nephro? V/Q scan? CPR? Factor VIIa ? Echo? Cyclophosphamide? S p u t u m P C R - T B
  • 18. 28 29 30 1 2 3 4 Pip/Taz FOB
  • 19.
  • 20. What to do next? Bronchoscope? CT scan? Dexamethasone? hemodialysis? Plasma exchange? Continue antibiotic? Kidney biopsy? Doppler ultrasound? Consult nephro? V/Q scan? CPR? Factor VIIa ? Echo? Cyclophosphamide? S p u t u m P C R - T B
  • 21.
  • 22. 28 25 11 18 4 Pip/Taz dexa 5q8 meropenem colistin// colistin Methyl P x3 GTC PRES WBC  , schistocyte PT,PTT  , haptoglobin <10 Sputum – A.baum tracheostomy levofloxacin dexa 5q6 vanco ampho dexa 5q12 Sputum – MRSA yeast P.Ex x7
  • 23.  
  • 25. 21/01/51 28/09/53 30/09/53 3/10/53 11/10/53 16/10/53 21/10/53 26/10/53 ไข้ Sputum – A.baum, S.maltophilia
  • 26. 21/01/51 28/09/53 30/09/53 3/10/53 11/10/53 16/10/53 21/10/53 26/10/53
  • 27. 21/01/51 28/09/53 30/09/53 3/10/53 11/10/53 16/10/53 21/10/53 26/10/53 ยังมีเสมหะปนเลือด ,Hct 25 Cr 3.9
  • 28. 21/01/51 28/09/53 30/09/53 3/10/53 11/10/53 16/10/53 21/10/53 26/10/53 รู้ตัวดี ไม่มีไข้ Sputum - MRSA
  • 30.
  • 31.
  • 32.
  • 33.
  • 34. 1994 Chapel Hill nomenclature Arthritis Rheum . 1994 Feb;37(2):187-92. Microscopic polyangiitis
  • 35. Clincical Dx primary vasculitis WG surrogate marker Upper airway - bloody nasal discharge/crusting > 1 mo. - sinusitis, otitis media, mastoiditis > 3 mo. - retro-orbital mass or inflammation - subglottic stenosis - Saddle nose, destructive sinonasal disease Lower airway - fixed pulm. infiltrates, nodules, cavity > 1 mo. - bronchial stenosis Ann Rheum Dis . 2007 February; 66 (2): 222–227 classificaton algorithm
  • 36.
  • 38. cANCA
  • 39. Granulocyte antigen of ANCA MPA, SLE, RA, UC, CD, PSC, AIH Clin Exp Rheumatol 2000: 18: 629-635.
  • 40. Problem with pANCA detection Perinuclear staining by ANA granulocyte-specific ANA? Epitope conformation
  • 41.
  • 42. does ANCA has role in pathogenesis ? I have no MPO Anti-MPO Pauciimmune NCGN Systemic vasculitis lipopolysaccharide Now I have MPO Anti-PR3 and pauciimmune glomerulonephritis ! Normal mice reduced leukocyte rolling augmented adhesion and transmigration across the endothelium Nat Rev Rheumatol . 2010 Nov;6(11):653-64.
  • 43. What does ANCA do with body ? Kelly’s textbook of Rheumatology, 8 th ed.
  • 44. Nat Rev Rheumatol . 2010 Nov;6(11):653-64.
  • 45. Nat Rev Rheumatol . 2010 Nov;6(11):653-64.
  • 46. RITUXIVAS N Engl J Med . 2010 Jul 15;363(3):211-20 N Engl J Med . 2010 Jul 15;363(3):221-32. RAVE Steroid Rituximab x 4 weeks IVCY x 2 pulses Steroid IVCY x 3-6 mo. Azathioprine New Dx AAV and renal involvement Non-blind RCT severe new or relapse 148 WG, 24 MPA double - dummy, noninferiority db-RCT Stone JH. Massachusetts General Hospital . USA Jones RB. Addenbrooke's Hospital . UK Pulse + pred 5 mo. Rituximab x 4 weeks placebo CY Pulse + pred 5 mo. oral CY x 3-6 mo. placebo Rituximab Placebo Aza Azathioprine noninferior I M 18% 18% mortality 36% 42% Severe AE 82% 76% remission Cy (11) Ritu (33) 62% 67% MPA 41% * 57% DAH pt. 42% * 67% relapse pt. 53% 64% Remission without pred Cy (98) Ritu (99) 66% 66% Renal 24% 27% DAH 54% 52% Pulmonary Cy (98) Ritu (99)
  • 47. Where ANCA come frome ? Nat Rev Rheumatol . 2010 Nov;6(11):653-64.
  • 48.
  • 49.
  • 50. Autoimmun Rev . 2010 Oct;9(12):812-9
  • 51. Clinical presentation Murray and Nadel’s Textbook of Respiratory Medicine, 5 th ed. 10–50% of patients . Mononeuritis multiplex reported cerebral infarction Neuro 35–60% of patients . Commonly purpura skin 35–55% of patients . Pain, bleeding, ischemia . Rare visceral aneurysms . GI 10–15% of patients . CHF and pericarditis have been described . Cardiac 0–30% of patients . Scleritis, episcleritis, uveitis . eyes Arthralgias and myalgias in at least 50% of patients musculoskeletal 5–30% of patients, with sinus disease most common Upper airway Segmental necrotizing glomerulonephritis and RPGN is almost universal . renal 25–55% of patients . 10–30% with diffuse alveolar hemorrhage . Fibrosis, focal infiltrates, effusions, pulmonary arterial hypertension pulmonary
  • 52. Pathology Neutrophilic capillaritis without granuloma, fibrinoid necrosis and arterial wall disruption, intra alveolar RBC and hemosiderin-laden macrophages Focal segmental necrotizing glomerulonephritis
  • 53. Murray and Nadel’s Textbook of Respiratory Medicine, 5 th ed.
  • 54.
  • 55.  
  • 56. Radiology . 2010 May;255(2):322-41
  • 57. Radiographics . 2002 Jul-Aug;22(4):739-64 consolidation with interlobular septal thickening Ground-glass opacity
  • 58. fine reticular pattern superimposed on a background of groundglass attenuation that is the crazy-paving pattern J Comput Assist Tomogr . 2004 Sep-Oct;28(5):710-6
  • 59. F 74y MPO. F/U HRCT show new honeycombing at 5 months 3 mo.
  • 60. M 60Y MPO resemble IPF CT show GGO and reticulation in subpleural region VAT Bx shows irregular interstitial fibrotic thickening, hemosiderin laden macrophages Radiology . 2010 May;255(2):322-41
  • 61.
  • 62. Arthritis Rheum . 1999 Mar;42(3):421-30
  • 63.
  • 64.
  • 65.
  • 66. Induction Maintenance WG MPA oral cyclophophamide+bactrim steroid oral CY
  • 67. Induction Maintenance WG MPA oral cyclophophamide+bactrim steroid pulse IVCY  less SE Azathioprine = oral CY Methotrexate = Aza MTX = oral CY non-organ threatening AAV NORAM 2005 CYCLOPS 2009 Rituximab = IVCY RITUXIVAS 2010 Rituximab > oral CY relapse AAV, DAH RAVE 2010 PE > methylpred severe renal AAV MEPEX 2007 Etanercept add-on WGET 2005 oral CY CYCAZAREM 2003 Pagnoux 2008 Leflunomide > MTX more AE Metzler 2007 MMF > IVCY mild renal MPA IMPROVE (terminated) Weixin 2010 open RCT n=35 MMF < Aza Bactrim add on Nat Rev Rheumatol. 2010 Nov;6 ( 11 ): 653-64 .
  • 68.
  • 70.

Editor's Notes

  1. Schematic representation of the immune mechanisms hypothetically involved with antineutrophil cytoplasmic antibody ( ANCA ) enhancement of vascular injury . An infectious trigger or other environmental stimulus leads to a burst of cytokines, which primes the neutrophils or monocytes and may lead to the local upregulation of adhesion molecules on endothelium . The priming process within the inflammatory cells leads to enhanced expression of ANCA antigens on the cell surface . Activated neutrophils or monocytes may degranulate and release reactive oxygen species and lysosomal enzymes, leading to endothelial injury and further activation of the endothelial cell surface . The magnitude of this effect is influenced by the specificity of ANCA for proteinase 3 ( PR3 ) or myeloperoxidase ( MPO ) , as well as different epitopes of these respective antigens . The reaction may be further influenced by the immunoglobulin G ( IgG ) and Fc γ receptor phenotype engaged . Products released from degranulated inflammatory cells become bound to endothelial cells and further serve as targets of ANCA . Release of chemotactic chemokines such as interleukin-8 ( IL-8 ) and macrophage chemoattractant protein-1 ( MCP-1 ) serve to augment chemotaxis and inflammatory cell transmigration, in conjunction with other adhesion molecules . Thus, the scheme provides the prerequisites for endothelial and vascular injury induced by ANCA : the presence of ANCA, the expression of target antigens for ANCA on primed neutrophils and monocytes, the interaction between primed neutrophils and endothelium via adhesion molecules, and, finally, the activation of endothelial cells and ultimate efflux of inflammatory cells to the extravascular and perivascular tissues . Fc γ R, Fc γ receptor;ICAM-1, intercellular adhesion molecule-1; PMN, polymorphonuclear leukocyte; TNF, tumor necrosis factor .
  2. Rituximab versus cyclophosphamide for ANCA - associated vasculitis .
  3. Perl Prussian blue stain