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41 Cards in this Set
- Front
- Back
What are the major factors in autoimmunity?
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Age (more common during puberty and middle age), sex (more common in females) and environmental factors (infections)
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What is the underlying cause of SLE?
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Presence of anti-nuclear antibodies, specifically anti-dsDNA or Sm antigen. This is diagnostic of SLE
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How can someone get a false positive on a VDRL test if they have SLE?
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Antiphospholipid antibodies present in 50% of SLE pts bind to cardiolipin antigen
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What does homogenous nuclear staining indicate?
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Antibodies to chromatin, hostones, and dsDNA
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What does nuclear rim or periphery staining indicate?
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Antibodies to dsDNA
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What does speckled nuclear staining indicate?
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Antibodies to histones and ribonucleoproteins (Sm antigen and RNP)
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What does nucleolar staining indicate?
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Antibodies to nucleolar RNA (most often seen in systemic sclerosis)
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What is the mechanism of tissue injury in SLE?
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Mostly mediated via type 3 hypersensitivity (antigen/antibody complexes)
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What is an LE body?
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It is when a neutrophil or macrophage engulfs the denatured nucleus of an injured cell. Found in about 70% of SLE
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What is the most clinically evident sign of SLE?
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Maculopapular rash due to deposition of Ig at the dermo-epidermal junction
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What are the cardiac effects of SLE?
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Pericarditis, myocarditis, or Libman-Sacks endocarditis (valvular lesions)
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What is the most common cause of death in SLE?
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Class IV-diffuse proliferative glomerulonephritis (kidney failure).
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How do you treat SLE?
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There isn't much you can do besides immune suppresion with steroids
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What genes are associated with RA?
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HLA-DR4 or HLA/DR1
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What is the clinical presentation most common in RA?
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Symmetric polyarticular arthritis. Sometimes they have vasculitis of the extremities with Raynaud phenomenon
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What is the mechanism of tissue damage in RA?
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Rheumatoid factors composed of anti-IgG Fc IgM causes injury by type 3 hypersensitivity
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What are some suspected environmental triggers for RA?
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EBV, Mycoplasma species, and mycobacteria
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What is the most diagnostic feature of RA?
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Anti-citrulline antibodies present in about 96% of RA patients
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What is the common pattern of hand deformity in RA?
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Radial deviation of the wrist and ulnar deviation of the fingers (swan-neck deformity). Distal IP joints spared.
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What is formed in the synovial joint spaces in RA?
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A pannus, which is a proliferation of synovial cells and inflammatory infiltrate. It eventually fills the joint space.
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What is the difference between primary and secondary Renaud's disease/phenomenon?
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Primary Renaud's is a hereditary disease on its own, secondary is an effect of all the autoimmune disorders.
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What is Renaud's phenomenon?
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It is a vascular disorder affecting blood supply to the extremities.
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What are the other spondyloarthropathies associated with RA?
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Ankylosing spondylitis, Reiter syndrome, and Psoriatic arthritis. All associated with presence of HLA B27
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What is Sjogren syndrome
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Autoantibodies to SS-A and SS-B (RNP's) that affect the exocrine glands and cause dry eyes and dry mouth.
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What is the relative risk of non-Hodgkin b-cell lymphoma for Sjogrens pts?
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About 40x higher risk
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What are the 2 types of systemic sclerosis?
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Diffuse and limited (CREST syndrome)
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What are the characteristics of diffuse systemic sclerosis?
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Widespread skin involvement, early visceral involvement, and rapid progression. Death within 1-2 years
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What does CREST stand for?
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Calcinosis, Raynauds, esophageal dysmotility, sclerodactyly, and telangiectasia. This is the limited form of systemic sclerosis that is much less serious
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What autoantibodies are associated with systemic sclerosis?
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Anti-DNA topoisomerase in those with diffuse, and anti-centromere in those with CREST.
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What are the most common visceral lesions in systemic sclerosis?
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Esophagus/GI tract, joints, and lungs
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Epidemiology of multiple sclerosis
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Affects caucasians twice as much, has a big genetic factor associated with HLA, more common in northern latitudes and in higher socioeconomic groups.
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Which virus is sometimes associated with onset of multiple sclerosis?
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EBV, varicella, and measles. EBV antigens resemble those of myelin basic protein
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What is the mechanism of CNS tissue destruction in MS?
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T and B lymphocytes become activated and can more easily cross blood-brain barrier where they then destroy myelin
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What is the name for an area of demyleinization in the CNS of an MS patient?
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Plaque. They are usually full of inflammatory cells and antibodies as well.
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What is the most common initial symptom of MS?
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Some degree of sensory loss, like a feeling of numbness. Motor defecits, visual loss or diplopia, and cognitive changes are also common.
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What is the most common form of MS initially?
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Relapsing remitting disease, which will progress to secondary progressive disease eventually.
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What paercentage of MS pts present with benign and primary progressive disease?
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About 20% present with benign and 15% with primary progressive that leads to rapid decline
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What are the most common conditions that exacerbate MS?
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Fever (most common), infections, and emotional or physical stress
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What effect does pregnancy have on MS symptoms?
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Disease activity and frequency of attacks tend to decrease
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What is the most accurate diagnostic technique for MS?
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Electrophoresis of CSF will reveal banding pattern of Ig's called oligoclonal bands. Present in up to 90% of MS pts. They would also look for presence of myelin basic protein
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What is the preferred treatment for MS?
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High dose steroids for the acute attack and Interferon B for prevention of relapses.
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