WVSOM -- Immuno/Micro -- Diseases of Disrodered Immune Regulation
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| how are mature lymphoid cells in normal adults that react to self antigens controlled? | clonal anergy and suppression from T reg cells
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| What is immunologic tolerance? | state of unresponsiveness which is specific for a particular antigen and is induced by prior exposure to that antigen
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| What is autoimmunity? | immune response against self
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| What are the 3 mechanisms to induce or maintain tolerance? | clonal deletion; clonal anergy; suppression
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| What is clonal deletion | destruction of self reactive T cells in thymus
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| What is clonal anergy | cells that are self reactive that are released form thymus are turned off; partially due to t cell requireing many signals to be activated
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| What is suppresion in tolerance? | regulatory t cells can actively repress otehr T and B cells thru IL-10 and TGF beta
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| Why do antigens not "seen" in teh thymus not normally trigger auto immunity ? | antigens mayb e sequestererd, levels may be too low to stimulate response, insufficietn levels of MHC I or II; accessory signals mayb e missing; active suppresion
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| What happens in the break down of tolerance | release of autoantigen, inappropriate MHC expression; failure of suppression; bypass of T-helper regulation; cross-reactivity adn molecular mimicry
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| What characteristics define the autoimmune poly-glandular syndromes? | circulating autoantibody against multiple endocrine glands (type I, II, III).
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| what is good pasture's syndrome | Ig and complement doposition in kidneys and lung
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| What is the triad of goodpastures disease? | pulmonary hemorrhage, glomerulonephriteis and circulating antibody to basement membranes
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| What is treatment of goodpasture's | plasma exchange and corticosteroids
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| What is addison's disease | disease of adrenal insufficiency; auto-antibody against adrenal cells
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| What is the target of addison's disease | enzymes involved in steroid synthesis
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| What autoimmune disease features autoantibody against double stranded DNA? | SLE
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| what immunologic mechanisms are involved int eh pathology of insulin-dependent diabetes? | autoantibodies and auto-reactive T cells are produced against the beta cells of the pancreatic Islets of langerhans, resulting in diminished insulin production adn chronic hyperglycemia
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| A patient presenting with arthritis, nephritis, chest pain, fever, wieght loss, immune complexes in the skin and circulation, and anti-nuclear antibodies is likely to have what autoimmune disease? | SLE
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| What is seen in lab tests with SLE? | anti-nuclear antibody; antibody against dsDNA; increased serum complement levels; immune complex deposition; numerous other autoantibodies
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| What are the most common clincal manifestations of SLE? | hematologic, arthritis, skin, feaver, renal
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| How can the administration of an antibiotic result in hemolytic anemia | bind to RBC membranes and induce antibody responses, and thus cause the immune-mediated destruction of teh RBC
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| What roles do proteins encoded by the major histocompatibility complex play in autoimmune diseases? | insufficent levels of MHC can induce autoimmune disease
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| What does releative risk mean in terms of auto immunity? | patients with the particular HLA have an increased risk of developing the disease
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| What autoimmune diease feature autoantibody against the AChR? | myasthenia gravis
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| What happens to muscle in myasthenia gravis? | imparied muscle contraction do to internalized and degraded AChR
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| What is rheumatoid factor | a factor that is not specific for rheumatoid arthritis but characteristic of the disease; it is an abnormal Ab that reacts to teh Fc region of the IgG
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| What is rheumatoid arthritis? | chronic, recurrent systemic inflammatory disease that primarily involves the joints
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| What are the immunologic features of RA | rheumatoid factor; immune complexes; inflammation within the joints; joint deformity and destruction; rheumatoid nodules
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| What is the treatment for RA? | Disease modifying anti-rheumatic drugs (DMARDs); NSAIDS; steroids; OMT
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| How does the destruction of erythrocytes occur in immune hemolytic anemia? | group of disorders that cause immunologic reactions against RBC proteins, usually mediated by antibody, complement or phagocytes
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| What are the three types of autoimmune-antibody types? | warm and cold antibody types as well as paroxysmal cold antibody type
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| what is warm-antibody type autoimmune hemolytic anemia | occurs at body temp; IgG mediated; 70% of hemolytic anemia cases
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| What is cold-antibody type autoimmune hemolytic anemia? | lower than body temp; IgM; second most common
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| What is direct Coomb's Test | Anti-Ig to see how much RBC has antibody-coated RBCs
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| When is direct coomb's test used? | hemolytic anemia
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| What is an indirect coomb's test | RBC not coated with antibody added to serum with antibody. anti-IG then added to see agglutination of RBC
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| What is treatment for hemolytic anemia? | splenectomy
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| What is multiple sclerosis? | chronic debilitating disease that features inflammatoyr demyelination of the CNS, resultig in progresive motor weakness, parethesia, visual impairment, ataxia
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| What are the immunologic features of MS? | inflammatory demyelination via macrophages and CTL; oligoclonal immunoglobulin bands in CSF; HLA antigen associations; altered immunoregulatory T cell populations
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| What is the disease course of MS? | Relapsing-remitting; benign; secondary progressive and Primary progressive
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| What is the relapsing-remitting phase of MS? | characterized by unpredictable relapses followed by periods of months to years of relative quiet (remissiong) with no new signs of disease activity. Deficits suffered during attacks may either resolve or leave sequelae.
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| What is Benign MS? | secondary progressive MS with inital relapsing-remitting MS, who then begin to have progressive neurologic decline between acute attacks without any definite periods of remission. occasinal relapses and minor remissions may appear
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| What is secondary progressive MS? | the primary progressive subtype that entails 10-15% of individuals who never have remission after their inital MS symptoms. Progression of disability from onset, with no, or only occasional and minor, remissions and improvements.
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| What is primary progressive MS? | individuals who, from onset have a seteady neurologic decline but also suffer clear superimposed attacks
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| What is hashimoto's thyroiditis | inflammatory disorder of teh thyroid with unkown etiology
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| What are symptoms in Hiromoto's thyroiditis? | cellular infiltration of the thyroid, high titer autoantibodies, and enlarged thyroid and abnormal thyroid fucntion
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| What is grave's disease | auto Ab that stimulates thyroid fucntion.
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| What is seen in greave's disease? | autoantibody against the TSH receptor; signs of hyperthyroidism and proptosis
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| What are the major clinical features presented by patietns with rheumatoid arthritis? | rheumatoid factor; imune complexes; inflammation within the joints; joint deformity and destruction; rheumatoid nodules
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| What are the S/S of ulcerative colitis | bloody diarrhea, tenesmus, fever adn weakness
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| What are teh S/S of crohn's disease? | ABD pain, diarrhea adn malabsorption
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| What cuases idiopathic inflammatory bowel diseases caused by? | believed to be due, in part, to disregulation of the immune system.
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| What is vasculitis? | inflammation of the blood vessels
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| What are some examples of disease with vasculitis? | RA, SLE, Giant-cell arteritis, wegener's granulomatosis, henoch-scholein purpura
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| What is immunobullous disease? | a group of autoimmune blistering diseases that affect skin and mucous membranes and are caused by or associated with the deposition of specific antibodies on cutaneous structures.
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| What is pemphigus vulgaris? | blistering disease of skin and mucus membranes; autoantibody against keratinocyte antigens
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| What are autoimmune diesase? | arise from an overactive immune response of the body agasint substances and tissue normally present in teh body. The body attacks its own cells
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| Why do most people NOT have autoimmune disease? | antigens not "seen" in teh thymus do not normally trigger auto immunity and you do not have self reactive protiens
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| Why do antigens in the thymus not normally trigger auto immunity? | antigens may be sequestered; antigen levels may be too low to stimulate response; insufficient levels of MHC I or II; accessory signals may be missing; active suppresion
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| What organ, tissue is affected by SLE? | systemic, joints
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| what does rheumatoid arthritis affect? | joints, systemic
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| What does insulin-dependent diabetes affect? | pancreas
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| what does addison's disease affect? | adrenals
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| What does polyglandular autoimmune sydromes affect? | various endocrine glands
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| What does graves disease affect? | thyroid
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| what does goodpastures affect? | kidneys and lungs
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| What does inflammatory bowel disease affect? | intestinal mucosa
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| what does immune hemolytic anema affect? | erythrocytes
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| What does pemphigus affect? | skin
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| what does multiple sclerosis affect? | CNS
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