Term
| What disease is associated w/HLA B27? |
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Definition
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Definition
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Definition
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| What is central tolerance? |
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Definition
clonal deletion of self-reactive T and B cells during maturation in central lymphoid organs
negative selection of developing T-cells that express high-affinity receptors for self-antigens |
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Term
| What are the mechanisms of peripheral tolerance? |
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Definition
| clonal deletion, clonal anergy, periph suppression by T cells |
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Term
| How can microbial agents trigger autoimmunity? |
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Definition
| tissue necrosis/inflam can cause up-reg of co-stim molecules on resting Ag-presenting cells, epitope spreading, superantigens can activate a large pool of T and B cells some of which may be autoreactive |
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Term
| How many criteria are required to diagnose SLE? |
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Definition
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| Who is most commonly affected by SLE? |
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Definition
| black women between ages of 20-64 |
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Term
What do the different immunofluorescence patters indicate?
homogenous/diffuse nuclear staining
rim or periph
speckled pattern
nucleolar pattern |
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Definition
homogenous: Abs to chromatin, histones and occasionally dsDNA
rim/periph: dsDNA
speckled (most common): Abs to non-DNA nuclear constituents i.e. Sm, RNP, SS-A, SS-B
nucleolar: Abs to nucleolar RNA (most often seen in patients w/systemic sclerosis) |
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Term
| Which ANAs are diagnostic of SLE? |
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Definition
| Antibody to dsDNA and Smith antigen |
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Term
| What drugs can induce an SLE-like response? |
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Definition
| hyrdalazine, procainamide, D-penicillamine |
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Term
| What is an LE cell (hematoxylin body) |
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Definition
| cell is damaged and nucleus reacts w/ANA causing loss of chromatin pattern; poly or macrophage engulfs the denatured nucleus |
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Term
| WHO classification of lupus nephritis |
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Definition
I:normal
II: mesangial lupus GN
III: focal prolif GN
IV: diffuse prolife GN
V: membranous GN |
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Term
| What is seen in the joints of a patient with SLE? |
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Definition
nonerosive synovitis w/little deformity
polys and fibrin are seen in the synovium |
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Term
| What does the spleen look like in a patient w/SLE? |
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Definition
| enlarged, capsular thickening, follicular hyperplasia, onion-skinning pencillary arteries |
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Term
| What does a "typical" lupus patient look like? |
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Definition
| young woman w/butterfly rash, fever, jt pain, pleuritic CP and photosensitivity |
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Term
| What is the most common cause of death in patients w/SLE? |
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Definition
renal failure or infection
also CAD |
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Term
| chronic discoid lupus erythematosus |
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Definition
characterized by skin plaques: edema, erythema, scaliness, follicle plugging, and skin atrophy w/ surrounding elevated erythematous border
rarely see systemic manifestations
IgG & C3 |
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Term
| subacute cutaneous lupus erythematosus |
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Definition
widespread but superficial and non-scarring: mild systemic symptoms
anti-SSA and HLA-DR3 |
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Term
| What gene increases one's risk of developing SLE from hydralazine? |
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Definition
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Term
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Definition
immune destruction of lacrimal and salivary glands results in keratoconjuctivitis sicca and xerostomia
lymphoid infiltration of lacrimal and salivary glands by activated T helper cells and B cells |
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Term
| What antibodies are seen in up to 90% of patients w/Sjogren's? |
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Definition
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Term
| What is seen in the salivary glands of a patient with Sjogren's? |
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Definition
monoclonal B cell population
may be a precursor for lymphoma |
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Term
| What is Mikulicz's disease? |
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Definition
| lacrimal and salivary gland enlargement- can be secondary to sarcoid, leukemia, lymphoma, Sjogren's |
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Term
| How do you diagnose Sjogren's? |
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Definition
| lip biopsy: examine minor salivary glands for presence of periductal and perivascular inflamm and lymphoid follicles w/germinal centers |
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Term
| What is systemic sclerosis (scleroderma)? |
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Definition
characterized by the presence of excess fibrosis throughout the body w/the skin being most commonly affected, but also affecting the GI tract, kidneys, heart, muscles, and lungs
more common in females, peaks from age 50-60, most severe in black patients |
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Term
| diffuse v local scleroderma |
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Definition
diffuse (most common): disease progresses to visceral involvement with death from renal failure, cardiac failure, pulm insuff, or intestinal malabsorption
localized: disease shows relatively limited skin involvement with later visceral involvement: Calcinosis, Raynauds, Esophageal dysmotility, Sclerodactyly, and Telangiectasia |
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Term
| Explain the immunologic and vascular hypotheses of collagen deposition. |
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Definition
immunologic: antigen causes T cells to respond/release cytokines and other mediators which enhance collagen synthesis by fibroblasts
vascular: endothelial injury causes release of platelet factors>triggers periadventitial fibrosis; activated cells release PDGF and chemotactic factors for fibroblasts |
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Term
| In what disease do you see anti-Sc170 (DNA topoisomerase 1) and anticentromere antibodies? |
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Definition
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Term
| What antibody is associated w/CREST syndrome? |
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Definition
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Term
| In what disease do you see claw-like fingers? |
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Definition
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Term
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Definition
| somewhat specific for inflammatory myopathies, also a marker of co-existing interstitial pulmonary fibrosis |
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Term
| Where do you see a distinctive lilac (heliotrope) skin rash, discoloration of the upper eyelids, periorbital edema, Grotton's lesion, and muscle weakness? |
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Definition
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Term
| What is the mechanism involved in dermatomyositis? |
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Definition
| capillary attack by antibody )from B cells) and complement w/resultant myocyte necrosis |
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