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Immunity & Immunopathy PT. 2
Oral Path. Chapter 3
59
Pathology
Professional
02/04/2013

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Term
What is an autoimmune disease?
Definition
certain self cells are no longer tolerated, immune system treats them as antigens
Term
Does an autoimmune disease effect just organs?
Definition
no, it can be for a single cell type, single organ, or more extensive
Term
What is something that influences our tendency to develop an autoimmune disease?
Definition
genetic factors, but some are induced by microbes
Term
What are some microbes that may induce an autoimmune disease?
Definition
-streptococcal infections
-epstein barr virus
Term
Can we only acquire immunodeficiencies?
Definition
no they may be congenital or acquired
Term
What can immunodeficiencies be associated with?
Definition
a systemic disease like diabetes or a result of treatment with certain drugs (chemothearpy, steriods, radiation)
Term
How can immunodeficiences be acquired?
Definition
by infection like HIV
Term
What is a primary immunodeficiency caused by? What does it result in?
Definition
-a genetic or congenital abnormality
-results in defective functioning of at least one part of the inflammatory or immune process
Term
What do primary immunodeficiencies result in?
Definition
defective functioning of at least one part of the inflammatory or immune process
Term
What are two examples of primary immunodeficiences?
Definition
-bruton's disease
-Degeorge syndrome
Term
What happens with Bruton's disease? How is it treated?
Definition
-B cells don't mature, so there are no antibodies, more at risk
-need injections of immunoglobulin
Term
What happens in DiGeorge syndrome? What is the treatment?
Definition
-3rd and 4th pharyngeal pouches fail to develop, T cells absent or diminished B cell activity
-can include bone marrow transplant
Term
What is another word for Aphthous ulcers? How common are they?
Definition
canker sores, occur in about 20% of the population
Term
What causes aphthous ulcers? What type of immunity is seen?
Definition
-trauma often causes them
-T cells seen, cell mediated immunity
Term
Where are aphthous ulcers found? How long does it take them to heal?
Definition
-found on lining mucosa
-heals in 7-10 days
Term
What are the three types of aphthous ulcers?
Definition
-minor(most common)
-major(larger, deeper, takes longer to heal)
-herpetiform(very tiny, multiple)
Term
What type of immunity is erythema multiforme?
Definition
Type III immunity in which circulating immune complexes provoke vascular injury
Term
What is erythema multiforme?
Definition
a vesiculobullous disease that involves the skin and mucous membranes
Term
What are the lesions associated with erythema multiforme called?
Definition
Target lesions/Bull's eye
Term
What are the symptoms associated with erythema multiforme?
Definition
low grade fever, malaise, headache
Term
What are some precipitating factors to erythema multiforme?
Definition
viral, bacterial, fungal infections, stress, allergy to drugs (sulfa, barbituates)
Term
What is the onset of erythema multiforme like? What is the treatment?
Definition
explosive onset, treat with corticosteroids
Term
What is the severe form of erythema multiforme called?
Definition
Steven-Johnson syndrome- triad of lesions: eye, genital, oral
Term
What is lichen planus? How prevalent it is?
Definition
-chronic disease effecting the skin and oral mucosa
-prevalence in the US is about 1%
Term
Who is lichen planus most common in? What is the etiology?
Definition
-middle aged people, more common in women
-unknown, worse in a time of stress
Term
What happens in lichen planus? How do you diagnose it?
Definition
-degeneration of basal layer of epilthelium separation from CT
-diagnosis made based on clinical appearance
Term
What does lichen planus look like?
Definition
-Wickman's striae characteristic lesion(white lacy, interconnecting lines formed from raised white plaques)
-bulla, erosions, and desquamative gingivitis
Term
Who is Reiter's syndrome seen in?
Definition
more in males with genetic marker (HLA- B27)
Term
What is Reiter's Syndrome?
Definition
an abnormal immune response seen after a venereal or GI infection
Term
What are the symptoms of reithers syndrome?
Definition
arthritis(knee and foot), urethritis, conjunctivitis, geographic tongue
Term
What is the treatment of Reiter's syndrome?
Definition
may have spontaneous remission, NSAID's used
Term
What is Langerhan's disease?
Definition
a tumor forming disease in which Langerhans cell(a type of macrophage) proliferates (tumor encased in bone)
Term
What is another name for Langerhan's disease?
Definition
Histiocytosis X
Term
Who is Lagerhan's disease usually seen in?
Definition
children and young adults
Term
What are the three categories of Langerhan's disease?
Definition
1.Letterer-Siwe
2.Hand-Schuller-Christian
3.Eosinophilic granuloma
Term
What is letterer-siwe?
Definition
-seen in children under 3
-rapid progression
-resembles lymphoma
-generally fatal
Term
What is Hand-Schuller-Christian?
Definition
-seen in young
-multiple lesions
-punched out areas of skull exophthalmos, diabeter insipidus
-oral bone loss, poor healing, sore mouth
Term
What is Eosinophilic granuloma?
Definition
-affects older kids
-solitary form
-radiographically resembles periodontal or periapical disease or appear as a well circumcised radiolucency in the skull or mandible
Term
What is Raynaud Phenomenon?
Definition
-common autoimmune symptom
-fingers turn WHITE because of lack of blood flow, then BLUE as vessels dilate to keep blood in tissues, then RED as blood flow returns
Term
What is Sjogren Syndrome?
Definition
-autoimmmune disease that attacks salivary and lacrimal glands, causes severe xerostomia and dry eyes
-these glands enlarge because of inflammatory scar tissue
Term
Who is Sjogren Syndrome usually seen in?
Definition
mostly women(~90%)
Term
What is Pemphigus Vulgaris?
Definition
a severe, progressive autoimmune disease that affects the skin and mucous membranes
Term
****Pemphigus Vulgaris causes Acantholysis. What is this?*****
Definition
desmosomal junctions between epithelial cells break down
Term
What is he age range and symptoms for Pemphigus Vulgaris?
Definition
-broad age range
-bullae, ulcers
-Nikolsky sign useful in diagnosis (press on mucosa with finger to see cleavage form from ruptured bullae)
Term
What are some other names for cicatricial pemphigold?
Definition
-mucous membrane
-benign mucous membrane pemphigold
Term
What is cicatricial pemphigold?
Definition
chronic autoimmune disease that affects oral mucosa, conjuntiva, genital mucosa, and skin, like pemphigus vulgaris but not as severe
Term
What happens in cicatricial pemphigold?
Definition
hemidesmosomes are attacked, epithelium separates from CT at the basement membrane
Term
What can cicatricial pemphigold be treated with?
Definition
a topical or systemic corticosteriod
Term
What is Bullous Pemphigold similar to? How are they dissimilar?
Definition
-similer to mucous membrane pemphigold
-dissimilar because autoantibodies are circulating and do not correlate with disease activity
-oral lesions are less common
Term
What is Behcet Syndrome?
Definition
chronic, recurrent autoimmune disease
Term
What are the symptoms of Behcet Syndrome?
Definition
oral ulcers, genital ulcers, ocular inflammation
Term
What happens in Behcet Syndrome?
Definition
antibodies form against human mucosa, may be genetic or caused from a virus
Term
When is a diagnosis of Behcet Syndrome made? How is it treated?
Definition
-diagnosis made when 2 of the 3 symptoms are present
-treatment based on symptoms(topical or systemic corticosteroids)
Term
What is rheumatoid arthritis?
Definition
affects the synovium lining joints
Term
What is scleroderma?
Definition
affects the fibrous intercellular tissue
Term
What is rheumatic fever?
Definition
-precipitated by A streptococcal pharyngitis.
-antibodies produced against the strept attack heart and joints
Term
What is Hashimoto's Thyroiditis?
Definition
-endocrine disorder
-antithyroid antibodies block production of thyroid hormones
Term
What is Myasthenia gravis?
Definition
antibodies block transmission of nerve signals to muscles
Term
What is multiple sclerosis?
Definition
myelin sheath covering nerves replaced by inflammatory cells impairing nerve conduction
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