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50 Cards in this Set
- Front
- Back
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What are the three requirements to call a disease autoimmune?
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Presence of autoimmune rxn
evidence that the rxn isnt secondary to tissue damage absence of another well defined disease |
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What is self tolerance?
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the bodys lack of immune response to its own tissues
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what two main mechanisms explain self tolerance?
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clonal deletion- deletes self reactive B/T cells during development
Clonal anergy- T cells that are self reactive still require the recognition of APC's MHC AND a costimulators. -anergic signals lack the costimuator |
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What two things is autoimmunity related to?
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Inheritance of susceptibility genes
Environmental triggers (this allows self-reactive lymphocytes that would otherwise be anergic, to become active) |
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What gene is often related with autoimmune diseases?
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HLA-B27
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What are the two ways infections spur autoimmune diseases?
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Provide costimulation to self reactive T cells that would otherwise be anergic
Have antigens that are very similar to self tissues, causes normal T-cells to be self reactive |
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What are the three immune privileged sites?
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Testis, eye, brain
this means there is no self tolerance for these regions |
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What is an ANA?
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Anitnuclear antibody
so antibodies to anything useful in the nucleus like DNA, histones, nonhistone proteins bound to RNA, nucleolar antigens |
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What is the fundamental defect in SLE?
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This seems to be a failure to maintain self tolerance.
many antibodies produce tissue damage |
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is the generic ANA positve in most SLE pts?
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The generic ANA is sensitive, but not SLE specific
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What is the positive ANA diagnostic of SLE?
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Antibodies to double stranded DNA and Smith (SM)
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What two other autoantibodies are associated with SLE?
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Antibodies against red cells, platelets, lymphocytes (blood stuff)
and antiphospholipids in 30-40% of people (this can prolong clotting time) |
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What can lupis pts test false positive for?
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syphilis!
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What are the four genetic factors in SLE?
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High concordance with monozygotic twins
Family members increase risk NA whites positive association with SLE and Class II HLA ags inherited defects in complement |
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What are the three Non genetic factors in SLE?
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Some drugs- procainamide, hydralazine
Sex hormones UV light can trigger Anti-DNA immune complexes |
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What is the main immunologic factor in SLE?
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B cell hyperactivity
CD4+ cells drive self reactive B cells to make auto antibodies |
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What are the main clinical mainfestations of SLE?
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Hematologic
Arthritis Skin problems Fatigue Fever |
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What type hypersensitivity mechanisms occur in SLE
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Type III- immune complexes, deposits crap in the glomeruli
Type II- antibodies against RBCs,WBCs, and Platelets |
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In SLE, how do ANA's cause damage?
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these bind to exposed cell nuclei.
the damaged nuclei loses chromatin pattern and become homogenous- called an LE body |
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What does SLE do to small blood vessels? how?
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this causes acute necrotizing vasculitis.
necrosis and fibrinoid deposits in a vessel wall- containing Ig's, C3, DNA and fibrinogen |
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What does SLE do to the skin?
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Erythema or maculopapular rash over nose and cheeks- called butterfly rash.
Made worse by UV light Liquefactive degeneration of basal layer of epidermis deposits of Ig and complement @ dermoepidermal junction |
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What is discoid lupus?
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this affects 20-30% of pts
shows as disc like erythematous patches with scale anywhere on the body. may not be systemic, but may progress to SLE |
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How does SLE affect serosal membranes?
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this causes effusions, exudate, and apacifiction (fibrous)
affects the pericardium and pleura |
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What does SLE affect the heart?
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pericarditis
sometimes myocarditis or valvular endocarditis (Libman-Sacks nodules) |
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How are joints damaged in SLE?
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swelling and nonspeific mononuclear cell infiltrate
no major deformities |
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How does the CNS get damaged in SLE?
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microvascular injury- lots of mini infarcts
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What is the age and sex of a typical SLE pt?
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A younger woman, 20's or younger
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What are the five Major findings likely in SLE?
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ANA- (anti dsDNA, or Smith)
Malar (butterfly) rash Arthritis Photosensitivity- (induces rash) Discor rash (raised erythematous patches with keratin scaling) Hematologic disorder*** (other ones to know, serosistis (pleural/pericardial), neruo disorders) |
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What is the usual clinical course for SLE?
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variable
- some may degrade rapidly - MOST with relapsing remitting course |
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Whats the 10 year survival rate in SLE?
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70%
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What are the major causes of death in SLE?
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renal failure
intercurrent infections and diffuse CNS involvement |
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What is Chronic Discoid Lupus Erythematosus and how is it different than SLE?
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Affects face and scalp with characteristic Discoid lesions- Raised plaques with edema, erythema, scaliness, follicular plugging, and skin atrophy.
Only has skin manifestations without the systemic effects |
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How is subacute cutaneous lupus erythematosus different than SLE?
Associations? |
this has lots of skin involvement with mild systemic symptoms
Associated with SS-A antigen, and HLA-DR3 |
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What is common, and what is rare in drug induced SLE?
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common to have positive ANA, and common anti-histone antibodies
uncommon to have anti-dsDNA antibodies |
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What are the two main clinical features of Sjogren syndrome? due to?
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Dry eyes
Dry mouth due to immune destruction of lacrimal and salivary glands |
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What are the ANA's assocaited with Sjogren syndrome
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Anti SS-A, and Anti SS-B
(stands for Anti- Sjogrens Syndrome, should be easy to remember) |
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What Diseases are pts with Sjogren at higher risk for?
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Other connective tissue disease
B cell lymphomas Pesudolymphomas |
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Whats the typical age and sex in Sjogrens?
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Women over age 40
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What are the symptoms people present with, when they have Sjogrens?
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Dry mouth, lack of tears
enlarged salivary glands due to infiltrate biopsy shows infiltrates in salivary glands |
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Define systemic sclerosis
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These are inflammatory and fibrotic changes throughout interstitium of many organs of the body
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What are the major organs affected by scleroderma?
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Skin- chronic ischemia with traumatic ulcerations (distal to proximal)
GI- atrophy, fibrosis of esophageal wall Musculoskeletal- both joints and muscles are involved Lungs- intersitial fibrosis Kidneys- smaller arteries intimal proliferation and hyalinization Heart- focal fibrosis |
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What are the two categories of systemic sclerosis
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Diffuse- widespread skin involvement, rapid course, and early visceral involvement
Limited- limited to skin, fingers, face, LATE visceral involvment, and CREST |
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What are the major findings associated with systemic sclerosis?
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Fibrosis is hallmark
Scl-70 antibodies- against DNA toposiomerasel (very specific test) Anticentromere antibody |
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What is the typical age and sex of a systemic sclerosis pt?
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Women in their 3rd to 5th decades
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What are the two major clinical findings in systemic sclerosis?
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Difficulty swallowing from esophageal fibrosis
atrophy of hands, due to collagenization of skin Can also get Reynauds phenomenon due to thick vessel walls that become hyalinized |
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What does CREST stand for?
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Calcinosis
Raynauds Esophageal dysmotility Sclerodactylyl Telangiectasia |
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What is the ANA for CREST?
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anticentromere antibodies
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What is the ANA associated with the inflammatory myopathies?
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Antinuclear abs, Jo-1 against t-RNS synthetase
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What are the features of Mixed Connective Tissue Disease?
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this is like SLE, inflammatory myopathy, and SS
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What is the ANA for MCTD?
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antibodies against ribonucleoprotein ag U1RNP
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