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肺血管炎

肺血管炎. 首都医科大学附属北京朝阳医院 呼吸与危重症医学科 北京呼吸疾病研究所 崔 瑷 201 4 - 04 - 19. 1. 系统性血管炎（Systemic Vasculitides） CHCC II, 2012.

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肺血管炎

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  1. 肺血管炎 首都医科大学附属北京朝阳医院 呼吸与危重症医学科 北京呼吸疾病研究所 崔 瑷 2014-04-19 1

  2. 系统性血管炎（Systemic Vasculitides）CHCC II, 2012 • Vasculitis is inflammation of blood vessel walls. Inflammation of blood vessel walls at least at some time during the course of the disease is a shared defining feature of all categories of vasculitis. Some categories of vasculitis also have characteristic tissue injury unrelated to the vasculitis. 2

  3. 系统性血管炎（Systemic Vasculitides） Inflammation Local Symptoms Systemic Symptoms fever fatigue malaise appetite and weight loss et al pain claudication bruits absent or diminished pulses loss of blood pressure haemorrhage et al 3

  4. 血管炎的命名和定义 （CHCC II, 2011） • Large-vessel vasculitis (LVV) • Takayasu arteritis (TAK) • Giant cell arteritis (GCA) • Medium-vessel vasculitis (MVV) • Polyarteritis nodosa (PAN) • Kawasaki disease (KD) 4

  5. 血管炎的命名和定义 （CHCC II, 2011） • Small-vessel vasculitis (SVV) • Anti-neutrophil cytoplasmic antibody (ANCA)-associatedvasculitis (AAV) • Microscopic polyangiitis (MPA) • Granulomatosis with polyangiitis (Wegener’s) (GPA) • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) • Immune complex SVV • Anti-glomerular basement membrane (anti-GBM) disease • Cryoglobulinemic vasculitis (CV) • IgA vasculitis (Henoch-SchÖnlein) (IgAV) • Hypocomplementemic urticarial vasculitis (HUV) (anti-C1qvasculitis) 5

  6. 血管炎的命名和定义 （CHCC II, 2011） • Variable vessel vasculitis (VVV) • Behcet’s disease (BD) • Cogan’s syndrome (CS) • Single-organ vasculitis (SOV) • Cutaneous leukocytoclastic angiitis • Cutaneous arteritis • Primary central nervous system vasculitis Isolated aortitis • Others 6

  7. 血管炎的命名和定义 （CHCC II, 2011） • Vasculitis associated with systemic disease • Lupus vasculitis • Rheumatoid vasculitis • Sarcoid vasculitis • Others • Vasculitis associated with probable etiology • Hepatitis C virus-associated cryoglobulinemic vasculitis • Hepatitis B virus-associated vasculitis • Syphilis-associated aortitis • Drug-associated immune complex vasculitis • Drug-associated ANCA-associated vasculitis • Cancer-associated vasculitis • Others 7

  8. 血管炎的命名和定义 （CHCC II, 2011） 8

  9. 大动脉炎 （Large-Vessel Vasculitis, LVV）CHCC II, 2011 giant cell arteritis (GCA) Takayasu Arteritis (TA) Often granulomatous; predominantlyaffects the aorta and its major branches. Often granulomatous predominantlyaffects the aorta and its major branches ( carotid and vertebral arteries), often temporal artery. 50 LVV is defined as vasculitis affecting the aorta and its major branches more often than other vasculitides; however, any size artery may be affected. 9

  10. 大动脉炎 （Takayasu Arteritis, TA） 10

  11. 大动脉炎 （Takayasu Arteritis, TA） • 5000, Japan (1990) / incidence: 2.6/million/year, North American patients (1985) • Sympotoms: • pre-pulseless: non-specific inflammatory features (fever, night sweats, malaise, weight loss, arthralgia, myalgia, mild anaemia); • chronic phase: vascular insufficiency. • Pulmonary artery involvement in 14-100% of patients, Oligaemic lung fields on plain chest X ray correlate with pulmonary vasculopathy in approximately a third of cases. 11

  12. 大动脉炎 （Takayasu Arteritis, TA） • Pathological feature: • Vessel inflammation leads to wall thickening, fibrosis, stenosis, and thrombus formation. • extensive destruction of the • medial elastic fibers leads to • aneurysm formation. 12

  13. 大动脉炎 （Takayasu Arteritis, TA） 13

  14. 大动脉炎 （Takayasu Arteritis, TA） 14

  15. 大动脉炎 （Takayasu Arteritis, TA） • Treatment CORTICOSTEROIDS (CS) • standard initial dose: 1mg/kg/day prednisolone or its equivalents • two-thirds of the total daily dose is given early in the morning the rest of the total daily dose is given in the evening after meals. 15

  16. 大动脉炎 （Takayasu Arteritis, TA） • Treatment IMMUNOSUPPRESSIVE (IS) • MTX • AZA • CYP (2mg/kg/day): retinal vasculitis; pulmonary artery involvement; severe aortic regurgitation; myocarditis. • MMF • CSA、FK-506、LEF 16

  17. 大动脉炎 （Takayasu Arteritis, TA） • Treatment Biologic agents • anti-TNF agents: infliximab (IFX) • CD20 monoclonal antibody: RTX • IL-6 monaclonal antibody: Tocilizumab 17

  18. 大动脉炎 （Takayasu Arteritis, TA） • Treatment Surgical treatment and invasive interventions • surgical treatment: hypertension with critical renal artery stenosis, et al. • balloon angioplasty • stent • percutaneous transluminal angioplasty (PTA) 18

  19. 大动脉炎 （Takayasu Arteritis, TA） • Treatment Supportive measures • Diet • Low salt intake • Calcium and vitamin D supplementation • Regular exercise • Antiplatelet agents: 80 mg/day aspirin 19

  20. 大动脉炎 （Takayasu Arteritis, TA） • Turkish TA Study Group: refractory disease in TA angiographic or clinical progression despite treatment or the presence of any of the following characteristics • prednidolone dose>7.5mg/day after 6 months of treatment, despite administration of conventional IS agents; • new surgery due to persistent disease activity; • frequent attacks (more than three per year); • death associated with disease activity. 20

  21. 大动脉炎 （Takayasu Arteritis, TA） • Kerr criteria two of the following four criteria shows active disease: • systemic features like fever and arthralgia that cannot be explained by other reasons; • elevated ESR; • findings of vascular ischaemia and inflammation; • typical angiographic findings. 21

  22. 大动脉炎 （Takayasu Arteritis, TA） • 56y, female, “Exertional dyspnea for 10 years, Lower extremity edema for 9 years” 22

  23. 大动脉炎 （Takayasu Arteritis, TA） 23

  24. 大动脉炎 （Takayasu Arteritis, TA） 24

  25. ANCA相关血管炎 （ANCA-associated vasculitis） • Antineutrophil cytoplasmic autoantibody (ANCA) (1988, International Workshop on ANCA, Copenhagen) 25

  26. ANCA相关血管炎 （ANCA-associated vasculitis） • Antineutrophil cytoplasmic autoantibody (ANCA) 26

  27. ANCA相关血管炎 （ANCA-associated vasculitis） • Antineutrophil cytoplasmic autoantibody (ANCA) 27

  28. ANCA相关血管炎 （ANCA-associated vasculitis） • Antineutrophil cytoplasmic autoantibody (ANCA) • Radiopraphic abnormalities: nodules, bilateral fluffy opacities; lobar consolidation, honeycomb lung. 28

  29. ANCA相关血管炎 （ANCA-associated vasculitis） • ANCA-associated vasculitis vs NSIP 29

  30. ANCA相关血管炎 （ANCA-associated vasculitis） • ANCA-associated vasculitis vs NSIP 30

  31. ANCA相关血管炎 （ANCA-associated vasculitis） • ANCA-associated vasculitis vs NSIP 31

  32. ANCA相关血管炎 （ANCA-associated vasculitis） • ANCA-associated vasculitis vs NSIP 32

  33. ANCA相关血管炎 （ANCA-associated vasculitis） • Antineutrophil cytoplasmic autoantibody (ANCA) Csernok E, et al. Blood. 2006;107(11):4440-4448. Voswinkel J, et al. Ann Rheum Dis. 2006;65(7):859-864. 33

  34. ANCA相关血管炎 （ANCA-associated vasculitis） • Antineutrophil cytoplasmic autoantibody (ANCA) Csernok E, et al. Blood. 2006;107(11):4440-4448. Voswinkel J, et al. Ann Rheum Dis. 2006;65(7):859-864. 34

  35. ANCA相关血管炎 （ANCA-associated vasculitis） • Antineutrophil cytoplasmic autoantibody (ANCA) • mm • Drug-induced systemic vasculitis • Inflammatory bowel disease and autoimmune liver disease • Arthritis • Lung diseases: cystic fibrosis • Infections 35

  36. ANCA相关血管炎 （ANCA-associated vasculitis） • Antineutrophil cytoplasmic autoantibody (ANCA) 36

  37. ANCA相关血管炎 （ANCA-associated vasculitis） • Pulminary affectation of ANCA-associated vasculitis 37

  38. ANCA相关血管炎 （ANCA-associated vasculitis） • Granulomatosis with polyangiitis (GPA) • A mean age of disgnosis: 65-70 years, without clear sex predominance. • Rriggering factors: infectious agents, environmentaltriggers, drug. • Clinical features: cutaneous signs, ear, nose, upper airway, lower airway manifestations,cardiovascular, gastrointestinal, renal, CNS presents • M 38

  39. ANCA相关血管炎 （ANCA-associated vasculitis） • Granulomatosis with polyangiitis (GPA) 39

  40. ANCA相关血管炎 （ANCA-associated vasculitis） • Microscopic polyangiitis (MPA) • A mean age of disgnosis: 60-65 years, men slightly predominance. 40

  41. ANCA相关血管炎 （ANCA-associated vasculitis） • Microscopic polyangiitis (MPA) 41

  42. ANCA相关血管炎 （ANCA-associated vasculitis） • Esinophilic granulomatosis with with polyangiitis (EGPA) • A mean age of disgnosis: 48 years, without clear sex predominance. • Rriggering factors: infectious agents, drugs, desensitization, vaccination. • Clinical features: mononeuritis multiplex; necrotic vascular purpura, gut, kideney, heart • Pathogenic features: eosinophil infiltration (52%), granuloma (18%), vasculitis (55%) • Laboratory anomalies: blood hypereosinophilia, high IgE titers (75%), anti-MPO P-ANCA-positivity (30-40%); anemia (83%), rheumatoid factor-positivity (53.6%), • Imagery: bilateral and migratory infiltrates or mixed interstitial patchy alveolar opacities; typical stenoses consistent with vasculitis 42

  43. ANCA相关血管炎 （ANCA-associated vasculitis） • Esinophilic granulomatosis with with polyangiitis (EGPA) 43

  44. ANCA相关血管炎 （ANCA-associated vasculitis） 44

  45. ANCA相关血管炎 （ANCA-associated vasculitis） 45

  46. ANCA相关血管炎 （ANCA-associated vasculitis） 46

  47. ANCA相关血管炎 （ANCA-associated vasculitis） 47

  48. ANCA相关血管炎 （ANCA-associated vasculitis） • Criteria for treatment response in ANCA-associated vasculitieds 48

  49. ANCA相关血管炎 （ANCA-associated vasculitis） 49

  50. ANCA相关血管炎 （ANCA-associated vasculitis） 50

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