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disease of imm regul

WVSOM -- Immuno/Micro -- Diseases of Disrodered Immune Regulation

how are mature lymphoid cells in normal adults that react to self antigens controlled? clonal anergy and suppression from T reg cells
What is immunologic tolerance? state of unresponsiveness which is specific for a particular antigen and is induced by prior exposure to that antigen
What is autoimmunity? immune response against self
What are the 3 mechanisms to induce or maintain tolerance? clonal deletion; clonal anergy; suppression
What is clonal deletion destruction of self reactive T cells in thymus
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
What is suppresion in tolerance? regulatory t cells can actively repress otehr T and B cells thru IL-10 and TGF beta
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
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
What characteristics define the autoimmune poly-glandular syndromes? circulating autoantibody against multiple endocrine glands (type I, II, III).
what is good pasture's syndrome Ig and complement doposition in kidneys and lung
What is the triad of goodpastures disease? pulmonary hemorrhage, glomerulonephriteis and circulating antibody to basement membranes
What is treatment of goodpasture's plasma exchange and corticosteroids
What is addison's disease disease of adrenal insufficiency; auto-antibody against adrenal cells
What is the target of addison's disease enzymes involved in steroid synthesis
What autoimmune disease features autoantibody against double stranded DNA? SLE
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
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
What is seen in lab tests with SLE? anti-nuclear antibody; antibody against dsDNA; increased serum complement levels; immune complex deposition; numerous other autoantibodies
What are the most common clincal manifestations of SLE? hematologic, arthritis, skin, feaver, renal
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
What roles do proteins encoded by the major histocompatibility complex play in autoimmune diseases? insufficent levels of MHC can induce autoimmune disease
What does releative risk mean in terms of auto immunity? patients with the particular HLA have an increased risk of developing the disease
What autoimmune diease feature autoantibody against the AChR? myasthenia gravis
What happens to muscle in myasthenia gravis? imparied muscle contraction do to internalized and degraded AChR
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
What is rheumatoid arthritis? chronic, recurrent systemic inflammatory disease that primarily involves the joints
What are the immunologic features of RA rheumatoid factor; immune complexes; inflammation within the joints; joint deformity and destruction; rheumatoid nodules
What is the treatment for RA? Disease modifying anti-rheumatic drugs (DMARDs); NSAIDS; steroids; OMT
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
What are the three types of autoimmune-antibody types? warm and cold antibody types as well as paroxysmal cold antibody type
what is warm-antibody type autoimmune hemolytic anemia occurs at body temp; IgG mediated; 70% of hemolytic anemia cases
What is cold-antibody type autoimmune hemolytic anemia? lower than body temp; IgM; second most common
What is direct Coomb's Test Anti-Ig to see how much RBC has antibody-coated RBCs
When is direct coomb's test used? hemolytic anemia
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
What is treatment for hemolytic anemia? splenectomy
What is multiple sclerosis? chronic debilitating disease that features inflammatoyr demyelination of the CNS, resultig in progresive motor weakness, parethesia, visual impairment, ataxia
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
What is the disease course of MS? Relapsing-remitting; benign; secondary progressive and Primary progressive
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.
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
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.
What is primary progressive MS? individuals who, from onset have a seteady neurologic decline but also suffer clear superimposed attacks
What is hashimoto's thyroiditis inflammatory disorder of teh thyroid with unkown etiology
What are symptoms in Hiromoto's thyroiditis? cellular infiltration of the thyroid, high titer autoantibodies, and enlarged thyroid and abnormal thyroid fucntion
What is grave's disease auto Ab that stimulates thyroid fucntion.
What is seen in greave's disease? autoantibody against the TSH receptor; signs of hyperthyroidism and proptosis
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
What are the S/S of ulcerative colitis bloody diarrhea, tenesmus, fever adn weakness
What are teh S/S of crohn's disease? ABD pain, diarrhea adn malabsorption
What cuases idiopathic inflammatory bowel diseases caused by? believed to be due, in part, to disregulation of the immune system.
What is vasculitis? inflammation of the blood vessels
What are some examples of disease with vasculitis? RA, SLE, Giant-cell arteritis, wegener's granulomatosis, henoch-scholein purpura
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.
What is pemphigus vulgaris? blistering disease of skin and mucus membranes; autoantibody against keratinocyte antigens
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
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
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
What organ, tissue is affected by SLE? systemic, joints
what does rheumatoid arthritis affect? joints, systemic
What does insulin-dependent diabetes affect? pancreas
what does addison's disease affect? adrenals
What does polyglandular autoimmune sydromes affect? various endocrine glands
What does graves disease affect? thyroid
what does goodpastures affect? kidneys and lungs
What does inflammatory bowel disease affect? intestinal mucosa
what does immune hemolytic anema affect? erythrocytes
What does pemphigus affect? skin
what does multiple sclerosis affect? CNS
Created by: tjamrose