Chapter 4 Flashcards
What are hypersensitivity reactions
Injurious immune reactions
secondary tissue injury
What are the 3 common causes
Autoimmunity
Microbial rxns (TB)
Environmental
What is type I hypersensitivity?
Allergies
How quickly does type I hypersensitivity happen
Immediate (within minutes)
What happens in a Type I hypersensitivity rxn
1) Helper T cells activated
2) Increase IgE production
3) Mast Cell degranulation (Histamine)
4) Edema, increased mucus secretion, Bronchoconstriction
What is the worst type of reaction to a type I hypersensitivity
Severe anaphylaxis
What can occur with late-phase Type I hypersensitivity
Eipthelial damage
Type II hypersensitivity reactions are know as what
Cytotoxic reactions
What antibodies are involved in Type II hypersensitivity
IgM, IgE
What are two syndromes associated with Type II hypersensitivity
Goodpasture syndrom
Graves disease
What test can be used to indicate Type II hypersensitivity
Coomb’s test
What is Type III hypersensitivity reactions know as
Immune complex formation
What can type III hypersensitivity cause
Systemic and Local inflammation
What are some symptoms due to systems type III hypersensitivity
Fever, arthritis, vasculitis, proteinuria, lymphadenopathy
What are a some diseases associated with Type III hypersensitivity
Lupus (SLE)
Polyarteritis nodosa (PAN)
reactive arthritis
serum sickness
What is Type IV hypersensitivity
T cell-mediated inflammation
or delayed inflammation
What is type IV hypersensitivity associated with
Autoimmune disorders
Persistent infections
How prominent are autoimmune disorders
they are in about 2-5% of the population
What can cause autoimmune disorders
Genetic mutations Environmental factors (infection, injury)
What are the prime targets for autoimmune disorders
Connective tissue and vessels
What is Lupus erythematosus
Failed self-tolerance
Formations of anti-nuclear antibodies
Who is more likely to have Lupus erythematosus
1: 700 women at childbearing age
1: 245 African american women of childbearing age
What autoantibody-mediated tissue injuries associated with Lupus erythematosus
Autoantibodies against cells (Type II)
Immune complex deposition (Type III)
What type of Vessel injury (thrombosis) is associated with Lupus Erythematosus
Fibrinoid necrosis
Acute necrotizing vasculitis
What type of renal failure (glomerulonephritis) is associated with Lupus Erythematosus
Proteinuria, Hematuria
Urinary red blood cell casts
What are the symptoms that may manifest in lupus erythematosus
Thrombosis Glomerulonephritis Fever Photosensitivity Serositis Hair loss Libman-sacks endocarditis of mitral valve G.I discomfort Oral ulcers Arthritis Seizures Fatigue
What photosensitivity rash is associated with lupus erythematosus
Malar (butterfly) rash
Discoid rash
Dermoepidermal degeneration
What is the survival rate for people that have lupus erythematosus
95% 5 year survival rate
What symptoms of lupus cause death
renal failure (glomerulonephritis) Infections
What is Sojgrens syndrome
Exocrine gland destruction and enlargement
What is sjogrens syndrome of the lacrimal glands
Keratoconjunctivitis sicca
What does Keratoconjunctivitis cause
Dry eyes
Inflammation of eyes
erosion
ulceration
What is sjogrens syndrome of salivary glands
Xerostomia
What does xerostomia cause
Dry mouth
Fissuring of mouth/tongue
Ulcerations
What are the two types of sjogrens syndrome
Isolated: sicca syndrome
Secondary autoimmune
What type of sjogrens syndrome is the most common
60% of all cases are secondary autoimmune disorders
What age and sex is most likely to be affects by sjogrens syndrome
Females
35-45
What does sjorgrens syndrome increase the risk of
B Cell lymphoma
What is characteristic of Systemic sclerosis (scleroderma)
Extensive fibrosis in multiple tissues Vascular destriction (small arteries)
What tissue is systemic sclerosis most common in?
95% Skin
What are and sex is systemic sclerosis most common
Females age 40-60
What are some features associated with Systemic sclerosis (scleroderma)
Raynaud Phenomenon Malabsorption Dysphagia Renovascular HTN renal failure pulmonary HTN Dyspnea myalgia arthritis
What are the two types of systemic sclerosis (scleroderma)
Limited
Diffuse
What is the “syndrome” used to describe limited systemic sclerosis (scleroderma)
C.R.E.S.T syndrome
What type of systemic sclerosis (scleroderma) is associated with: mild skin involvement; mainly fingers, face
Limited
What type of systemic sclerosis (scleroderma) is associated with: Rapid/widespread skin involvement
Diffuse
What is “CREST syndrome” in reference to Systemic sclerosis
C: Calcinosis R: Raynaud phenomenon E: Esophageal dysmotility S: Sclerodactyly T: Telangiectasia (spider veins)
What are the two broad types of immune deficiency disorders
Primary = Congenital Secondary = Acquired
What type of immune deficiency is associated with genetic variability
Congenital
How long does it take for a primary immune deficiency to be seen
6-24 month
What are the Primary immune deficiencies talked about in class
Bruton disease Common variable immunodeficiency Isolated IgA deficiency Hyper-IgM syndrome Severe combined immunodeficiency (SCID) Defects in lymphocyte activation Defects in innate immunity
What is the onset, sex, and problem associted with Bruton disease
Onset: 6 months
Males
Failed B cell Maturation = no antibodies
How do you treat bruton disease
IgG infusions
What is the onset, sex and problem associated with Common variable immunodeficiency
Males, Females
10-30 years old
Decreased antibody response to infxn
In common variable immunodeficiency, why is there a decreased antibody response to infxns
There are Normal B cells but no Plasma cells
What race and ratio of people with isolated IgA deficiency
Caucasians
1:700 people
What is the immune problem associated with Isolated IgA deficiency
decreased IgA production
Increased G.I and respiratory infections
What is happening with Hyper-IgM syndrome
increased IgM
decreased IgG, IgA, IgE
recurrent bacterial infections
What type of mutation is associated with hyper-IgM syndrome
70% are X-linked mutations
What is the prevelance of Hyper-IgM syndrome
1:100,000
What is the common name for Severe combined immunodeficiency (SCID)
Bubble Boy syndrome
What is going on in SCID (bubble boy syndrome)
lymphopenia (decreased T and B cells)
Lymphoid atrophy
They are have no immune system
What is associated with Defects in lymphocyte activation
Defective T cell immunity
Chronic fungal infections
What is associated with Defects in innate immunity
Altered complement phagocytosis
Multiple infections
What are some types of secondary deficiency disorders
Infections
Malnutrition
Aging
Cancer
What are the mechanisms of infections for secondary immune deficiency disorders
Suppression of Marrow
Lymphocyte dysfunction
What causes Acquired Immunodeficiency syndrome (AIDS)
Human immunodeficiency virus (HIV)
What is the most common way to transmit HIV
Blood, Semen, Vaginal fluid, breast milk
75% sexually transmitted
What does HIV do
Destroys CD4+T cells and macrophages
What does HIV allow for
Opportunistic infections that are rare in age/race/region
What is the most common way to transmit HIV in the US
Male-Male sex
What is the most common way to transmit HIV in the world
Male-Female sex
What is the most common cause in transmission of HIV (I.e what happens during unprotected sex)
Mircoabrasions and semen
What are the two tests used to determine HIV
ELISA test
Western Blot
How can a person carry HIV but be immune to it
They have a mutation in CCR5 receptor protein
Prevents HIV from entering cell
How long does the acute stage of HIV/AIDS last
3-6 weeks after infection
What is associted with the acute stage of HIV/AIDS
Pyrexia
Pharyngitis
Myalgia
Viremia
After initial HIV infection how long does it take for Viremia to decrease
~12 weeks
How long does the Chronic stage of HIV/AIDS last
2-10 years after infection
What is happening at a cellular level during the chronic stage of HIV/AIDS
Steady decline of CD4+T Cells
Gradually increase Viremia
What infections are associated with the Chronic stage of HIV/AIDS
Pyrexia
Fatigue
Candidiasis
When does the Crisis stage of HIV/AIDS occur
when there are less than 200 CD4+Tcells per ul
What is associated with the crisis stage of HIV/AIDS
increased viremia Cachexia fatigue pyrexia diarrhea wt loss CNS defects
What is a persons who has HIV/AIDS most likely to die from?
Opportunistic infections (80%)
What is the “AIDS-defining” Pathogen
Fungal: Pneumocytosis, Candidiasis
What causes CNS injury due to AIDS/HIV
infected monocytes –> Meningitis
What can someone take to prolong their life when they have AIDS/HIV
antiretoviral meds
What is the actual origin of AIDS
Eating of primates
What is amyloidosis
inflammaroty or inherited diseases caused due to misfolded proteins
what is an Amyloid
non-branching fibrillar proteins
How do amyloids cause problems in the body
They are not phagocytised and accumulate causeing tissue damage and disrupt functions of tissues
What are the types of Amyloids
Amyloid light (AL)
Amylod-associtaed (AA)
B-Amyloid (AB)
Of those in the crisis stage of AIDS; what viruses are most likely to cause: Neoplasia/ CNS lymphoma
KSHV
EBV
HPV
What type of amyloid plaque is strongly associated with Alzheimer Disease
B-Amyloid
What type of Amyloid is found in the liver and chronic inflammation
Amyloid-Associated (AA)
What type of amyloid is associated with immunoglobulin light chains and Bence-Jones proteins
Amyloid Light (AL)
What are the types of Amyloidosis (not amaloids)
Systemic
Localized
Hereditary
What are the 3 subtypes of systemic amyloidosis
Primary
Secondary
Aging
What type of systemic amyloidosis is associated with malignant plasma cells, what amyloids are there too
Primary
Amyloid light proteins
What type of systemic amyloidosis is associated with chronic inflammation. What amyloids are associated with it
Secondary
Amyloid-associated
What type of systemic amyloidosis is associated with elderly
Aging (senile systemic amyloidosis)
What type of amyloidosis is associated with multiple organ systems
Systemic
What type of amyloidosis is associated with single organ/tissue
Localized
What type of amyloidosis is rare, family related and/or geographic
Hereditary
What is the most common organ to have amyloid plaques
Kidney
What type of amyloids are commonly found in the brain
B-amyloid plaques
