B&L Unit 2 Flashcards
Type 1 Response
IgE mediated. Cross linkage of adjacent IgE on mast cells that activates release granules and histamine in first phase. Late phase occure 5 to 10 hours later. Major treatment is avoidance. Epipen with epinephrin can help. Ex. allergic reaction Ex. serum sickness? Ex. Asthma Ex. Eczema Ex. Hay Fever
Type 2 Response
Specific antibody mediated reaction via complement mediated damage or stimulatory hypersensitivity. Immunofluorescence as linear.
Ex. Myasthenia gravis
Ex. Graves disease
Ex. Hashimoto Thyroiditis
Type 3 Response
Immune complexes trapped in basement membrane of blood vessels. Immunofluorescence is lumpy bumpy. Causes lumpy bumpy glomerulus.
Ex. Lupus
Ex. Initial Hypersensitivity Pneumonitis (Famer’s Lung)
Ex. Arthus Reaction
Ex. IgA Nephropathy
Ex. one shot serum sickness
Ex. Rheumatoid Arthritis (IgM against IgG)
Type 4 Response
T cell mediated. Ex. TB Test Ex. Contact Dermatitis (poison ivy) Ex. Multiple Sclerosis Ex. first set graft rejection Ex. second set graft rejection Ex. Sjogren Syndrome
DiGeorge
Parathyroid should be carefully evaluated. Thymus does not form correctly. PreT cells have nowhere to mature. Results in lack of mature T cells.
Myasthemia Gravis
Type 2 autoimmunity.
Autoantibody to acetylcholine receptor
Systemic Lupus Erythematosis
Type 2 autoantibody to dsDNA. Anti-nuclear antibody (ANA) will be present. Type 3 lumpy bumpy complexes present in glomerulus.
Rheumatoid Arthritis
Pollution is an important risk factor- it increases citrullinated proteins in the lung.
Mostly type 4 T cell damage.
Autoantibody is present (RF) but is not only factor. This is also type 2.
Celiac Disease
Type 4.
HLA DQ2 and DQ8. Body accidentally phagocytoses gluten with tissue transglutaminase 2 and presents it on MHC 2s. T cell mediated immunity to gliadin (wheat peptide).
Graves Disease
Type 2 hypersensitivity immune reaction. IgG antibody binds and activates TSH receptor and activates signaling pathway leading to hyperthyroidism.
Chronic Frustrated Immune Response
Body makes immune response to something that doesn’t go away. Skewed toward inflammatory response and away from Tregs.
Ex. IBD, Crohn disease, celiac disease, chronic Be disease, psoriasis, periodontal disease
Acute Promyelotic Leukemia
Tx: all trans retinoic acid (ATRA)
Goodpasture Disease
Autoantibodies to the lung and kidney basement membranes (type 4 collagen).
Type 2 autoimmunity.
HLA DR2
Autoimmune Thrombocytic Purpura (ATP)
Antibodies destroy platelets.
Type 2 autoimmunity.
Autoimmune Hemolytic Anemia
Autoantigen to RBC antigen.
Hashimoto Thyroiditis
Type 2 & 4.
Body creates antibodies to thyroid antigens.
Causes hypothyroidism.
Includes T cell mediated destruction of thyroid.
Aire
Thymic transcription factor that affects gene expression that helps regulate and destroy T cells that attack self. Defect in aire leads to T cell mediated autoimmunity.
Dressler Syndrome
Post- MI autoimmune response.
Type 2.
Specific autoantibody against epitope in pericardial or myocardial antigens.
Chronic Be Disease
Type 4. Chronic frustrated immune response.
SCID
No B or T cells made.
X-linked: defect in gamma chain of IL2 receptor.
Autosomal recessive: adenosine deaminase (ADA) deficiency.
Brutons
No B cell development.
X linked: bruton’s tyrosine kinase (BTK) is normally important in B cell signaling. Causes agammaglobulinemia.
X linked Hyper IgM
Defective class switching due to CD40/CD40L leads to high IgM with low IgG and IgA
Common Variable Immunodefiency
Unknown cause.
It is difficult for these pts to trigger antibody production by B cells.
Rheumatic Heart Disease
Autoantibody to laminin on heart valves.
Major Basic Protein
Secreted by eosinophils (attracted by EFC-A, mixture of leukotrienes and prostaglandins, is secreted from mast cells) as part of late reaction to parasites.
Job Syndrome (hyperIgE syndrome)
Autosomal dominant.
Develop hyperreactivity to own cells.
Inability to make IFN-gamma effectively leading to poor Th1 responses and predominance of Th2.
Selective IgA Deficiency
increased frequency and severity of allergies
Ataxia Teangiectasia
autosomal recessive
Wiskott-Aldrich Syndrome
X linked
platelet and B cell deficiency
eczema
Ankylosing Spondylitis
HLA-B27
Type 1 Diabetes
Antibodies made to islet associated antigens.
Considered to be Type 4, because T cell mediated damage causes pathology.
Autoimmune Polyglandular Syndrome Type 1
Mutation on Aire causes T cells to be selected inappropriately, allowing T cells to survive that are antibodies to Th17 cell and IL17 signaling factors.
- prevents attraction of M1 macrophages
- oral candidiasis common
- Addison’s disease (adrenal insufficiency) common
- hypoparathyroidism common
- diabetes common
Chronic Granulomatous Disease (CGD)
- primary immunodeficiency that affects neutrohphil function
- characterized by recurrent life threatening fungal infections
- neutrophilia- but neutrophils cannot produce ROS (no NADPH oxidase) to kill pathogens
- formation of granulomas occurs
- X linked 65% of cases
- tx: prophylactic antibiotics, stem cell transplant, gene therapy
