免疫系统的疾病课件.ppt

1、Chapter 5：Diseases of the Immune System,Department of Pathology Peking Union Medical College Lu Zhaohui,Immunity and Disease,The normal immune response is best understood in the context of defense against infectious pathogens, the classical definition of immunity. Innate immunity Adaptive immunity,A

2、UTOIMMUNE DISEASES,Immune reactions against self-antigens-autoimmunity result in the damage to tissues and single or mutiple organs,Mechanism,Immunological tolerance is the phenomenon of unresponsiveness to an antigen as a result of exposure of lymphocytes to that antigen. Self-tolerance refers to l

3、ack of responsiveness to an individuals own antigens, and it underlies our ability to live in harmony with our cells and tissues.,Lose of Immunological Tolerance,Central Tolerance peripheral tolerance,Role of genetic factors Role of Infections Role of Estrogen,Types of Autoimmune Diseases,Organ / Ce

4、ll specific AD Hashimoto thyroiditis autoimmune hemolytic anemia autoimmune atrophic gastritis of pernicious anemia autoimmune encephalomyelitis autoimmune orchitis Goodpasture syndrome autoimmune thrombocytopenia Type diabetes mellitus myasthenia gravis Graves disease primary biliary cirrhosis auto

5、immune hepatitis ulcerative colitis membranous glomerulonephritis,Types of Autoimmune Diseases,Systemic or multiple organs systemic lupus erythematosus rheumatoid arthritis Sjgren syndrome Reiter syndrome imflammatory myopathy systemic sclerosis polyarteritis nodosa,Systemic Lupus Erythematosus, SLE

6、,SLE is the prototype of a multisystem disease of autoimmune origin, characterized by a vast array of autoantibodies, particularly antinuclear antibodies (ANAs). Acute or insidious in its onset, it is a chronic, remitting and relapsing, often febrile illness characterized principally by injury to th

7、e skin, joints, kidney, and serosal membranes.,Etiology and Pathogenesis of SLE,Genetic Factors Immunological Factors Environmental Factors Others,Mechanisms of Tissue Injury,SLE is a complex disorder of multifactorial origin resulting from interactions among genetic, immunological, and environmenta

8、l factors that act in concert to cause activation of helper T cells and B cells and result in the production of several species of pathogenic autoantibodies.,mesangial proliferative (class II),Morphology,Kidney minimal mesangial (class I); mesangial proliferative (class II); focal proliferative (cla

9、ss III); diffuse proliferative (class IV); membranous (class V). None of these patterns is specific for lupus.,Morphology,Skin. Joints Central Nervous System. Pericarditis and Other Serosal Cavity Involvement Cardiovascular system Spleen Lung Other Organs and Tissues.,Rheumatoid Arthritis, RA,Rheuma

10、toid arthritis is a chronic systemic inflammatory disorder that may affect many tissues and organs-skin, blood vessels, heart, lungs, and muscles-but principally attacks the joints, producing a nonsuppurative proliferative and inflammatory synovitis that often progresses to destruction of the articu

11、lar cartilage and ankylosis of the joints,Etiology and Pathogenesis,Genetic susceptibility Environmental arthritogen Autoimmunity,Morphology,Joints infiltration of synovial stroma by a dense perivascular inflammatory infiltrate increased vascularity aggregation of organizing fibrin accumulation of n

12、eutrophils osteoclastic activity in underlying bone pannus Skin. Rheumatoid nodules Blood Vessels.,Rheumatoid nodules of Skin,SJGREN SYNDROME,Sjgren syndrome is a chronic disease characterized by dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) resulting from immunologically mediated

13、 destruction of the lacrimal and salivary glands.,Etiology and Pathogenesis,lymphocytic infiltration and fibrosis of the lacrimal and salivary glands Autoimmune factors RF: 75% ANAs: 50%-80% RNP,SS-A,SS-B: 90% Genetic factors HLA-B8,HLA-B3,DRW52,HLA-DQA1,HLA-DQB1,Morphology,Lacrimal and salivary per

14、iductal and perivascular lymphocytic infiltration. The ductal lining epithelial cells may show hyperplasia atrophy of the acini fibrosis, and hyalinization Kidney Lung Skin Central Nervous System,Involvement of salivary gland,INFLAMMATORY MYOPATHIES,dermatomyositis, polymyositis, and inclusion-body

15、myositis probably immunologically mediated,SYSTEMIC SCLEROSIS,chronic inflammation thought to be the result of autoimmunity widespread damage to small blood vessels progressive interstitial and perivascular fibrosis in the skin and multiple organs,Etiology and Pathogenesis,progressive fibrosis Micro

16、vascular disease,Morphology,Skin Alimentary Tract Musculoskeletal System Kidneys Lungs Heart,extensive subcutaneous fibrosis,Vasculitis,GIANT-CELL ARTERITIS,Giant-cell (temporal) arteritis is the most common form of vasculitis among elderly chronic, typically granulomatous inflammation of large to s

17、mall-sized arteries principally the temporal arteries,Morphology,nodular intimal thickening granulomatous inflammation elastic lamina fragmentation,GIANT-CELL ARTERITIS,POLYARTERITIS NODOSA,Polyarteritis nodosa (PAN) is a systemic vasculitis of small or medium-sized muscular arteries typically invol

18、ving renal and visceral vessels,Morphology.,segmental transmural necrotizing inflammation of small to medium-sized arteries weakens the arterial wall Impaired perfusion transmural inflammation fibrinoid necrosis thickening of the vessel wall,POLYARTERITIS NODOSA,THROMBOANGIITIS OBLITERANS,Thromboang

19、iitis obliterans (Buerger disease) is a distinctive disease that often leads to vascular insufficiency segmental, thrombosing, acute and chronic inflammation of medium-sized and small arteries tibial and radial arteries,Pathogenesis,The strong relationship to cigarette smoking,Morphology,sharply seg

20、mental acute and chronic vasculitis of medium-sized and small arteries microabscesses,WEGENER GRANULOMATOSIS,Acute necrotizing granulomas of the upper respiratory tract Necrotizing or granulomatous vasculitis affecting small to medium-sized vessels Renal disease,Morphology.,Upper respiratory tract l

21、esions granulomas with geographic patterns of central necrosis vasculitis renal lesions,Rejection of Tissue Transplants,Mechanisms of Recognition and Rejection of Allografts,T Cell-Mediated Reactions Antibody-Mediated Reactions,Rejection of Solid organ transplantation (Kidney),Hyperacute Rejection.

22、Acute Rejection. Acute cellular rejection Acute humoral rejection (rejection vasculitis) Chronic Rejection.,Acute rejection of liver transplantation,Transplantation of Hematopoietic Cells,GVH disease occurs in any situation in which immunologically competent cells or their precursors are transplante

23、d into immunologically crippled recipients, and the transferred cells recognize alloantigens in the host Acute GVH disease Chronic GVH disease,Immunodeficiency Syndromes,primary immunodeficiency disorders, always genetically determined secondary immunodeficiency states, which may arise as complications of various diseases , ect. Infection of virus primary immunodeficiency Very rare,ACQUIRED IMMUNODEFICIENCY SYNDROME (AIDS),Caused by the retrovirus human immunodeficiency virus (HIV) Profound immuno