Autoimmune Flashcards
Autoimmune diseases
- breakdown of tolerance
- genetic factors are important
- original insult
- some autoimmune diseases are organ-specific (type II) and others systemic in distribution (type III)
Breakdown of tolerance
“tolerance” is developed as part of becoming immunocompetent
genetic factors important in autoimmune disease
affected members may not develop the same autoimmune disease; some people may have more than one autoimmune disease (tendencies are what’s inherited)
Original insult in autoimmune
- may be identifiable - often drug, virus, or bacteria
- often preexisting infections that leave no trace
Theories of autoimmune disease development
- Normal self-antigens are altered by a drug, pathogen, or other mutation and are no longer seen as “self” (no longer look like your own)
- antibodies against foreign antigens cross-react with a similar self-antigen
- Clonal deletion theory
Clonal deletion theory
suggests that self-reactive lymphoid cells are eliminated during immunological development
SLE stands for?
Systemic Lupus Erythematousus
Epidemiology for SLE
- 1 in 2,500 people
- 10x more women than men
- familial (ID twins have 30% concordance rate)
- 5 year survival rate = 93-95%
- mortality is low, but impose on quality of life is high
Type III SLE
Has a wide range of symptoms including:
- arthritis (90%) - complexes stuck in synovial membranes
- vasculitis and rash (75%) - stuck in blood vessels of skin
- kidney disease (40-50%) - stuck in glomerulus
- blood abnormalities (50%)
- cardiovascular disease (30-50%) - unknown mechanism but there’s correlation
SLE info
large variety of autoantibodies are produced including ones against DNA and RNA ( = ANA’s: anti-nuclear antibodies)
Treatment of SLE
- glucocorticoids/ corticosteroids - strong anti-inflammatory
- antimalarials - no known reason
- monoclonal antibody (anti-body against autoantibodies)
Downside of long-term corticosteroid treatment of SLE
- increased susceptibility to infections
- atherosclerosis -> stroke, Coronary Artery Disease (heart attack)
- obesity -> hypertension, diabetes type 2
- Cushing’s disease (hypercorticoidism: over tim. adrenal gland)
- others
Immunodeficiency diseases
- primary (congenital)
- secondary (acquired): as a result of infections, metabolic diseases, cancer or treatment
- may involve primarily B cells or different types of T cells or it may be generalized
All immunodeficiencies are characterized by ___________
lymphopenia (lymphocytes not making antibodies)
Primary Immunodeficiency
- SCID (severe combined immunodeficiency)
- lack both T and B cells
- thymus is hypoplastic (never gets big) and lymph nodes are small
- used to die early in infancy
- new treatments are bone marrow transplant and gene therapy trial (14/16 worked)