第9章 免疫性疾病

免疫系统疾病

讲课目录

⏹免疫系统疾病总论

⏹川崎病

⏹风湿病

原发性免疫缺陷病immmunodeficency

目的要求

⏹了解原发性免疫缺陷病的分类

⏹熟悉各种原发性免疫缺陷病的临床特点及实

验室检查

⏹熟悉原发性免疫缺陷病的治疗

⏹掌握原发性免疫缺陷病的诊断步骤和方法

重点内容

⏹先天性低丙种球蛋白血症出现时间及感染特点，与暂时性低丙种蛋白血症的鉴别

⏹选择性IGA缺乏症的诊断

⏹先天胸腺发育不全的临床特点和实验室检查特点

⏹SCID病的临床表现，X线检查特点及实验室检查

免疫发展简史(developmental history)：

经验免疫学时代(empirical era )科学免疫学时代(science era)现代免疫学时代(modern era)

免疫学的基本概念(terms

and definitions)

Immune:

1. Defender:ability of organism to resist and overcome infection

2. Stabilizer:ability of organism to exclude disintegration cells

3. Monitor:ability of organism to kill mutant cells

Immune system:

⏹Immune organ: thyrum, bone marrow;

spleen, lymph node

⏹Immunocompetent cells:

⏹Immune molecules :

Immune organs

⏹Center immune organ:

thymus,

bone marrow,

mucosal associated lymphoid tissue (MALT)

⏹Peripheral immune organs :;

spleen,

lymph node,

Immune cells (1)

Multiple hematopoietic stem cells

Myeloid progenitor Lyphiod progenitor

RBC, Platelet, PMN,Macrophage, Dendritic cells T cells , B cells, NK cells, Dendritic cells

Immune cells (2)

⏹T cells

⏹ B cells

⏹Neutrophil cells

⏹Macrophages

⏹Red blood cells

⏹Platelet cells

⏹Dendritic cells

T cells

TcR: αβT and γδT

CD: CD3，CD4，CD8；Functional: DTH, Th, Tc, Ts, Tr；Th (cytokines): Th1，Th2, Th3 ；

Th0Th2IL-4Th0Th1IL-12

IFN γ，IL-2，TNF IL-4，IL-10+－

－

+Thr Th3

IL-10

TGF-

B cells

⏹Immunoglobulins ⏹Humoral immunity

Other cells

⏹Neutrophil cells ⏹Macrophages ⏹Red blood cells ⏹Platelet cells ⏹Dendritic cells ⏹NK cells

Immune molecules

⏹Membrane molecules: TCR; BCR; MHC;

⏹Soluble molecules

⏹Chemokinesis

Membrane molecules:

⏹TCR;

⏹BCR;

⏹MHC;

⏹Receptors

⏹Costimulator factors

Costimulated factors

当共刺激缺乏时，将发生细胞凋亡和免疫耐受

T细胞

未成熟的树突状细胞

Chemokinesis ( adhesin molecules)

Signal transductional molecule

免疫细胞的信号传递

Soluble molecules

⏹Immunoglobulin

⏹Complement

⏹Opsonize : manan binding lectin (MBL)⏹Cytokines and receptors

Immunoglobulin : IgG (1-4) IgM

IgA IgD IgE Complement :

C1-9 and

5 regulated protein

IgG

⏹IgG1-4

⏹IgG1:viral; bacteria; extrotoxin ⏹IgG2: Lps of bacteria

Complement

⏹Membrane attack complex ⏹Opsonizing

⏹Chemotaxis

⏹Exclude immune complex