肺血管炎

16
大动脉炎 （Takayasu Arteritis, TA）
Treatment
Biologic agents
anti-TNF agents: infliximab (IFX) CD20 monoclonal antibody: RTX IL-6 monaclonal antibody: Tocilizumab
Treatment
IMMUNOSUPPRESSIVE (IS)
MTX
AZA
CYP (2mg/kg/day): retinal vasculitis; pulmonary artery involvement; severe aortic regurgitation; myocarditis. MMF CSA、FK-506、LEF
Kawasaki disease (KD)
4
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 (Wegener’s) (GPA) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Immune complex SVV Anti-glomerular basement membrane (anti-GBM) disease Cryoglobulinemic vasculitis (CV)
11
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.
6
血管炎的命名和定义 （CHCC II, 2011）
Vasculitis associated with systemic disease

7
Lupus vasculitis Rheumatoid vasculitis Sarcoid vasculitis Others 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
19
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
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.
Takayasu Arteritis (TA)
Often granulomatous; predominantly affects the aorta and its major branches.
giant cell arteritis (GCA)
Often granulomatous predominantly affects the aorta and its major branches ( carotid and vertebral arteries), often temporal artery.
12
大动脉炎 （Takayasu Arteritis, TA）
13
大动脉TA）
14
大动脉炎 （Takayasu Arteritis, TA）
Treatment
CORTICOSTEROIDS (CS)
standard initial dose: 1mg/kg/day prednisolone or its equivalents
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
1830 Yamamoto
1905 Takayasu Onishi Kagosha 1920 Numano 1951
10
Shimizu Sano
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,
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
大动脉炎 （Takayasu Arteritis, TA）
50
9
大动脉炎 （Takayasu Arteritis, TA）
Year Author Characters of patients
fever, weight loss, dyspnoea, impalpable upper limb and carotid pulses (45y man) dundal arteriovenous anastamoses (21y woman) absent radial pulses absent radial pulses panarteritis, retinal ischaemia (25y woman) "pulseless disease"
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
系统性血管炎（Systemic Vasculitides）
CHCC II, 2012
Vasculitis is inflammation of blood vessel walls. Inflammation of blood
3
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)
18
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
Vasculitis associated with probable etiology
血管炎的命名和定义 （CHCC II, 2011）
8
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.
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.