Autoimmune Diseases

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Name antibody mediated diseases (type II hypersensitivity)

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autoimmune hemolytic anemia
- RBC antigens
goodpastures syndrome
- basement membranes of kideny glomeruli and lung alveoli

acute rheumatic fever
- antibody to strep cell wall antigen cross reacts with myocaridal antigen

myasthenia gravis
- Ach receptor

Graves disease which is
(hyperthryroidism)

pernicious anemia

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graves disease

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hyperthryroidism
target antigen is thyroid stimulating hormone receptor
antibody stimulates TSH receptors causing hyperthyroidism

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pernicious anemia

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intrinsic factor of gastric parietal cells is target, antibody causes neutralization of intrinsic factor, leads to decreased absorbition of vit B12
abnormal erythrpoiesis, anemia

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immune complex diseases (Type III hypersensitivity)

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systemic lupus erythematosus (antibody against DNA and nucleoproteins)

serum sickness
arthus reaction

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T cell mediated diseases (Type IV hypersensitivity)

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type 1 insulin dependent diabetes mellitus - t cells attack pancreatic islet antigens

rheumatoid arthritis

multiple sclerosis target myelin proteins

contact sensitivity like poison ivy

chronic infections like tuberculosis get granulomatous

viral hepatitis

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cyclosporine

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blocks T cell cytokine production of IL-2

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mycophenolate mofetil

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blocks lymphocyte proliferation by inhibiting nucleotide synthesis in lymphocytes

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rapamycin

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blocks lymphocyte proliferation by inhibiting IL-2 signaling

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corticosteroids

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reduce inflammation by inhibiting macrophage cytokine secretion

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anti CD3 monoclonal antibody

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depletes T cells by binding to CD3 and promoting phagocytosis
treats acute rejection

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anti IL-2 receptor antibody

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inhibits T cell proliferation by blocking IL-2 binding

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x-linked SCID

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common gamma chain mutation which is component of cytokine receptors, no proliferation of T cells and NK cells, low Ig levels due to lack of T cell help

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autosomal SCID

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caused by ADA (adenosine deaminase) or PNP deficency

proliferating lymphocytes accumulate toxin levels of purine metabolites, limits proliferation of lymphocytes

decrease in B and T cells and Ig levels with ADA

Can also be caused by RAG gene mutations for Rag recombinase, leads to no antigen recgonition and proliferation

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X-linked agammaglobulinemia

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decrease in serum Ig levels, reduced B cell levels

block in pre-B cell maturation due to lack of tyrosine kinase (Btk)

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Digeorge syndrome

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lack of thymus, low T cell count due to failure of T cell maturation

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X-linked hyper IgM syndrome

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CD40 ligand mutations

cause defective B cell heavy chain isotype switching

T-cells don't bind to B cells to activate the B cells through the CD40 ligand

also defective marcophage activation by T cells

Have only IgM antibodies

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bare lymphocyte syndrome

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defective class II MHC expression due to mutations in transcrpition factors

inpaired CD4+ T cell activation, defective cell-mediated immunity and T cell dependent humoral immunity

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chronic granulomatous disease

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defective production of reactive oxygen species by phagocytes

marcophages build up and can't destroy microbes effectively which leads to granulomas

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leukocyte adhesion deficiency

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mutations in genes encoding integrins, can't stop rolling leukocytes so defective adhesion
leukocytes not recruited normally to infection sites

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chediak-higashi syndrome

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defective lysomal function of lysomal granules of neutrophils, macrophages, dendritic cells and NK cells don't function right, can't kill bacteria