肺血管炎课件

1 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 崔崔 瑷瑷 2014-04-19 系统性血管炎（系统性血管炎（Systemic VasculitidesSystemic Vasculitides） CHCC II, 2012CHCC II, 2012 2 pVasculitis 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. 系统性血管炎（系统性血管炎（Systemic VasculitidesSystemic Vasculitides） 3 Local Symptoms pain claudication bruits absent or diminished pulses loss of blood pressure haemorrhage et al Systemic Symptoms fever fatigue malaise appetite and weight loss et al Inflammation 血管炎的命名和定义血管炎的命名和定义 （CHCC II, 2011CHCC II, 2011） pLarge-vessel vasculitis (LVV) nTakayasu arteritis (TAK) nGiant cell arteritis (GCA) pMedium-vessel vasculitis (MVV) nPolyarteritis nodosa (PAN) nKawasaki disease (KD) 4 血管炎的命名和定义血管炎的命名和定义 （CHCC II, 2011CHCC II, 2011） pSmall-vessel vasculitis (SVV) pAnti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) nMicroscopic polyangiitis (MPA) nGranulomatosis with polyangiitis (Wegeners) (GPA) nEosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) pImmune complex SVV nAnti-glomerular basement membrane (anti-GBM) disease nCryoglobulinemic vasculitis (CV) nIgA vasculitis (Henoch-Schnlein) (IgAV) nHypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis) 5 血管炎的命名和定义血管炎的命名和定义 （CHCC II, 2011CHCC II, 2011） pVasculitis associated with systemic disease nLupus vasculitis nRheumatoid vasculitis nSarcoid vasculitis nOthers pVasculitis associated with probable etiology nHepatitis C virus-associated cryoglobulinemic vasculitis nHepatitis B virus-associated vasculitis nSyphilis-associated aortitis nDrug-associated immune complex vasculitis nDrug-associated ANCA-associated vasculitis nCancer-associated vasculitis nOthers 7 8 血管炎的命名和定义血管炎的命名和定义 （CHCC II, 2011CHCC II, 2011） 大动脉炎大动脉炎 （Large-Vessel Vasculitis, LVVLarge-Vessel Vasculitis, LVV） CHCC II, 2011 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 giant cell arteritis (GCA) Often granulomatous predominantly affects the aorta and its major branches ( carotid and vertebral arteries), often temporal artery. Takayasu Arteritis (TA) Often granulomatous; predominantly affects the aorta and its major branches. 50 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 10 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 11 p 5000, Japan (1990) / incidence: 2.6/million/year, North American patients (1985) p Sympotoms: n pre-pulseless: non-specific inflammatory features (fever, night sweats, malaise, weight loss, arthralgia, myalgia, mild anaemia); n chronic phase: vascular insufficiency. n 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. 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 12 p Pathological feature: n Vessel inflammation leads to wall thickening, fibrosis, stenosis, and thrombus formation. n extensive destruction of the medial elastic fibers leads to aneurysm formation. 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 13 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 14 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 15 p Treatment CORTICOSTEROIDS (CS) n standard initial dose: 1mg/kg/day prednisolone or its equivalents n 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. 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 16 p Treatment IMMUNOSUPPRESSIVE (IS) n MTX n AZA n CYP (2mg/kg/day): retinal vasculitis; pulmonary artery involvement; severe aortic regurgitation; myocarditis. n MMF n CSA、FK-506、LEF 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 17 p Treatment Biologic agents n anti-TNF agents: infliximab (IFX) n CD20 monoclonal antibody: RTX n IL-6 monaclonal antibody: Tocilizumab 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 18 p Treatment Surgical treatment and invasive interventions n surgical treatment: hypertension with critical renal artery stenosis, et al. n balloon angioplasty n stent n percutaneous transluminal angioplasty (PTA) 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 19 p Treatment Supportive measures n Diet n Low salt intake n Calcium and vitamin D supplementation n Regular exercise n Antiplatelet agents: 80 mg/day aspirin 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 20 p Turkish TA Study Group: refractory disease in TA angiographic or clinical progression despite treatment or the presence of any of the following characteristics n prednidolone dose7.5mg/day after 6 months of treatment, despite administration of conventional IS agents; n new surgery due to persistent disease activity; n frequent attacks (more than three per year); n death associated with disease activity. 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 21 p Kerr criteria two of the following four criteria shows active disease: n systemic features like fever and arthralgia that cannot be explained by other reasons; n elevated ESR; n findings of vascular ischaemia and inflammation; n typical angiographic findings. 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 22 p 56y, female, “Exertional dyspnea for 10 years, Lower extremity edema for 9 years” 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 23 大动脉炎大动脉炎 （Takayasu Arteritis, TATakayasu Arteritis, TA） 24 ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 25 p Antineutrophil cytoplasmic autoantibody (ANCA) (1988, International Workshop on ANCA, Copenhagen) ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 26 p Antineutrophil cytoplasmic autoantibody (ANCA) ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 27 p Antineutrophil cytoplasmic autoantibody (ANCA) ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 28 p Antineutrophil cytoplasmic autoantibody (ANCA) p Radiopraphic abnormalities: nodules, bilateral fluffy opacities; lobar consolidation, honeycomb lung. ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 29 p ANCA-associated vasculitis vs NSIP ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 30 p ANCA-associated vasculitis vs NSIP ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 31 p ANCA-associated vasculitis vs NSIP ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 32 p ANCA-associated vasculitis vs NSIP ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 33 p 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. ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 34 p 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. ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 35 p Antineutrophil cytoplasmic autoantibody (ANCA) n mm n Drug-induced systemic vasculitis n Inflammatory bowel disease and autoimmune liver disease n Arthritis n Lung diseases: cystic fibrosis n Infections ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 36 p Antineutrophil cytoplasmic autoantibody (ANCA) ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 37 p Pulminary affectation of ANCA-associated vasculitis ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 38 p Granulomatosis with polyangiitis (GPA) n A mean age of disgnosis: 65-70 years, without clear sex predominance. n Rriggering factors: infectious agents, environmentaltriggers, drug. n Clinical features: cutaneous signs, ear, nose, upper airway, lower airway manifestations,cardiovascular, gastrointestinal, renal, CNS presents n M ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 39 p Granulomatosis with polyangiitis (GPA) ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 40 p Microscopic polyangiitis (MPA) n A mean age of disgnosis: 60-65 years, men slightly predominance. ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 41 p Microscopic polyangiitis (MPA) ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 42 p Esinophilic granulomatosis with with polyangiitis (EGPA) n A mean age of disgnosis: 48 years, without clear sex predominance. n Rriggering factors: infectious agents, drugs, desensitization, vaccination. n Clinical features: mononeuritis multiplex; necrotic vascular purpura, gut, kideney, heart n Pathogenic features: eosinophil infiltration (52%), granuloma (18%), vasculitis (55%) n Laboratory anomalies: blood hypereosinophilia, high IgE titers (75%), anti-MPO P-ANCA-positivity (30-40%); anemia (83%), rheumatoid factor-positivity (53.6%), n Imagery: bilateral and migratory infiltrates or mixed interstitial patchy alveolar opacities; typical stenoses consistent with vasculitis ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 43 p Esinophilic granulomatosis with with polyangiitis (EGPA) ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 44 ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitisANCA-associated vasculitis） 45 ANCAANCA相关血管炎相关血管炎 （ANCA-associated vasculitis