肺血管炎（888）.ppt

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,医学类,系统性血管炎（Systemic Vasculitides） CHCC II, 2012,2,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,医学类,系统性血管炎（Systemic 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,3,医学类,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),4,4,医学类,血管炎的命名和定义 （CHCC II, 2011）,Small-vessel vasculitis (SVV) Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) Microscopic polyangiitis (MPA) Granulomatosis with polyangiitis (Wegeners) (GPA) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Immune complex SVV Anti-glomerular basement membrane (anti-GBM) disease Cryoglobulinemic vasculitis (CV) IgA vasculitis (Henoch-Schnlein) (IgAV) Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis),5,5,医学类,血管炎的命名和定义 （CHCC II, 2011）,Variable vessel vasculitis (VVV) Behcets disease (BD) Cogans syndrome (CS) Single-organ vasculitis (SOV) Cutaneous leukocytoclastic angiitis Cutaneous arteritis Primary central nervous system vasculitis Isolated aortitis Others,6,6,医学类,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,7,7,医学类,8,血管炎的命名和定义 （CHCC II, 2011）,8,医学类,大动脉炎 （Large-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.,50,9,医学类,大动脉炎 （Takayasu Arteritis, TA）,10,10,医学类,大动脉炎 （Takayasu Arteritis, TA）,11,5000, 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,医学类,大动脉炎 （Takayasu Arteritis, TA）,12,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,医学类,大动脉炎 （Takayasu Arteritis, TA）,13,13,医学类,大动脉炎 （Takayasu Arteritis, TA）,14,14,医学类,大动脉炎 （Takayasu Arteritis, TA）,15,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,医学类,大动脉炎 （Takayasu Arteritis, TA）,16,Treatment IMMUNOSUPPRESSIVE (IS) MTX AZA CYP (2mg/kg/day): retinal vasculitis; pulmonary artery involvement; severe aortic regurgitation; myocarditis. MMF CSA、FK-506、LEF,16,医学类,大动脉炎 （Takayasu Arteritis, TA）,17,Treatment Biologic agents anti-TNF agents: infliximab (IFX) CD20 monoclonal antibody: RTX IL-6 monaclonal antibody: Tocilizumab,17,医学类,大动脉炎 （Takayasu Arteritis, TA）,18,Treatment Surgical treatment and invasive interventions surgical treatment: hypertension with critical renal artery stenosis, et al. balloon angioplasty stent percutaneous transluminal angioplasty (PTA),18,医学类,大动脉炎 （Takayasu Arteritis, TA）,19,Treatment Supportive measures Diet Low salt intake Calcium and vitamin D supplementation Regular exercise Antiplatelet agents: 80 mg/day aspirin,19,医学类,大动脉炎 （Takayasu Arteritis, TA）,20,Turkish TA Study Group: refractory disease in TA angiographic or clinical progression despite treatment or the presence of any of the following characteristics prednidolone dose7.5mg/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,医学类,大动脉炎 （Takayasu Arteritis, TA）,21,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,医学类,大动脉炎 （Takayasu Arteritis, TA）,22,56y, female, “Exertional dyspnea for 10 years, Lower extremity edema for 9 years”,22,医学类,大动脉炎 （Takayasu Arteritis, TA）,23,23,医学类,大动脉炎 （Takayasu Arteritis, TA）,24,24,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,25,Antineutrophil cytoplasmic autoantibody (ANCA) (1988, International Workshop on ANCA, Copenhagen),25,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,26,Antineutrophil cytoplasmic autoantibody (ANCA),26,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,27,Antineutrophil cytoplasmic autoantibody (ANCA),27,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,28,Antineutrophil cytoplasmic autoantibody (ANCA) Radiopraphic abnormalities: nodules, bilateral fluffy opacities; lobar consolidation, honeycomb lung.,28,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,29,ANCA-associated vasculitis vs NSIP,29,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,30,ANCA-associated vasculitis vs NSIP,30,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,31,ANCA-associated vasculitis vs NSIP,31,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,32,ANCA-associated vasculitis vs NSIP,32,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,33,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,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,34,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,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,35,Antineutrophil cytoplasmic autoantibody (ANCA) mm Drug-induced systemic vasculitis Inflammatory bowel disease and autoimmune liver disease Arthritis Lung diseases: cystic fibrosis Infections,35,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,36,Antineutrophil cytoplasmic autoantibody (ANCA),36,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,37,Pulminary affectation of ANCA-associated vasculitis,37,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,38,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,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,39,Granulomatosis with polyangiitis (GPA),39,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,40,Microscopic polyangiitis (MPA) A mean age of disgnosis: 60-65 years, men slightly predominance.,40,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,41,Microscopic polyangiitis (MPA),41,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,42,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,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,43,Esinophilic granulomatosis with with polyangiitis (EGPA),43,医学类,ANCA相关血管炎 （ANCA-associated vasculitis）,44,44,医学类,ANCA相关血管炎 （ANCA-asso