6/29 - Diseases of Immune System Flashcards
what is the act of transferring cells, tissues, or organs from one site to another; typically between different individuals
transplantation
can organ system malfunction be corrected w/ transplantation of organ from a donor
YES
what is the most formidable barrier to transplantation as a routing medical treatment? this results in what?
immune system is a barrier - results in rejection of transplanted organs
what are the different graft types
- autograft
- isograft
- allograft
- xenograft
what is a graft in the same person from one part of body to area
autograft
what is the graft between identical twins
isograft
what is the graft between different members of the same species
allograft
what is the graft between two different species
xenograft
the ability of recipient T cells to recognize donor derived antigens is called what
allorecognition
what initiates allograft rejection
allorecognition
once recipient T cells become active, they under go clonal expansion, differentiate into effector cells, and ___
migrate into the graft where they promote tissue destruction
how to increase graft survival
- good MHC (HLA) matching of donor and recipient (MHC I and II)
- immunosuppression
when is immunosuppression not needed when grafting
with identical (monozygotic twins)
what occurs when immunologically compentent cells or their precursors are transplanted into immunologically crippled recipients
graft vs host disease (GVHD)
what occurs when transferred cells recognize alloantigens in the host and attack host tissues
GVHD
what is the pathophysiology of GVHD
The interaction of the donor T cell and the host APC leads to the activation of APC and immunogenic presentation of HLA class I-restricted minorhistocompatibility antigens (“license to kill”) to activate CD8 cells of the transplantation that can attack mHA on epithelial tissue or also on hematopoietic tissue
what is a major cause of morbidity and mortality in allogenic bone marrow transplantation, with increasing incidene of it corresponding to level of HLA mismatch between donor and host
GVHD
what causes epithelial cell neocrosis
GVHD
where does GVHD cause epithelial cell necrosis
liver, skin, GI tract
is GVHD lethal
yes, potentially lethal process
what are immune rxns against self antigens
autoimmune disorders
what is the phenomenon of unresposiveness to an antigen induced by exposure to lymphocytes to that antigen
immunologic tolerance
autoimmunity arises from combination of what
- inheritance of susceptibility genes
- environmental triggers
what contributes to the breakdown of self-tolerance
inheritance of susceptibility genes
what promote the activation of self-reactive lymphocytes, such as infections and tissue damage
environmental triggers
general features of autoimmune diseases
- chronic, w/ relapses and remissions, and damage is progressive
- manifestations determined by nature of underlying immune response
genetic factors in autoimmunity have an evidence of ___ clustering and linkage w/ ___
evidence of familial clustering and linkage w/ certain HLA types, especially class II antigens
who has a greater incidence of most autoimmune disorders
women
what are implicated in autoimmunity
bacteria, mycoplasmas, and viruses
which diseases are exceptions to the concept that women have the greater incidence of autoimmune disorders?
- ulcerative colitis (50:50)
- diabetes mellitus (more men than women)
what is the most common connective tissue disease in US
lupus erythematosus (LE)
what are the 3 forms of LE
- Systemic Lupus erythematosus
- Chronic cutaneous erythematosus
- Subacute cutaneous erythematosus
what disease results from increased activity of humoral limb (B lymph) of immune system
LE
what disease results from abnormal function of T lymphocytes
LE
do we know the exact pahtogenesis of LE
no
does genetic and environmental factors play a role in LE
YES
what disease exhibits butterfly rash
systemic lupus erythematosus
what disease:
- serious multisystem
- variety of cutaneous and oral manifestations
- difficult to diagnose at early stage
- mainly women
systemic lupus erythematosus
what is the average age of diagnosis of systemic lupus erythematosus
31 years
what are common findings of systemic lupus erythematosus
- fever
- weight loss
- arthritis
- fatigue
- general malaise
what percent of systemic lupus erythematosus has arthritis in multiple joints (polyarthritis)
95%
what percentage of systemic lupus erythematosus has butterfly rash
40-50%
does sunlight make systemic lupus erythematosis better?
NO! makes it worse
kidneys are affected in ___% of patients in systemic lupus erythematosus
40-50%
what is the most significant aspect of systemic lupus erythematosus
kidney failure
is cardiac involvement common in systemic lupus? what percent?
common - 50% display vegetation in heart valves
what is the type of cardiac involvement in systemic lupus erythematosus
Libman-Sacks endocartitis (vegetation affects heart valves) - antigen complexes coagulate in ehart
can some patients w/ systemic lupus erythematosus develop superimposed bacterial endocartitis?
YES
what percentage of patients with systemic lupus erythematosus has oral lesions? where?
5-25% of patients in palate, buccal mucosa, and gingiva
what type of oral lesions found in systemic lupus erythematosus
lichenoid or granular or nonspecific
what lupus has the involvement of the vermillion border of the lower lip
lupus cheilitis
T/F: varying degrees of ulcerations, pain, erythema, and hyperkeratosis may be seen in systemic lupus erythematosis oral lesions
TRUE
what disease:
- pt has few or no symptoms
- lesions limited to skin or mucosal surfaces
chronic cutaneous LE
how does chronic discoid LE look
begin as scaly, erythematous patches often distributed on sun exposed skin
chronic discoid LE healing process has what?
scarring and hypo or hyperpigementation
chronic LE oral lesions are identical to what?
lesions of erosive lichen planus
unlike oral lesions of lichen planus, oral lesions of CCLE seldom occur in absense of ___
skin lesions
what has an ulcer in center and white radiating lines on side
lichenoid
what characterizes oral lesions of CCLE
ulterated or atrophic, erythematous central zone, surrounded by find white radiating lines
lesions in what disease may be painful when exposed to spicy or acidic food
CCLE
what disease:
- clinical manifestation between SLE and CCLE
- skin lesions are most prominent feature
- present on sun exposed skin
- lesions do not show scarring
SCLE
what disease:
direct immunofluorescent testing of lesional tissue shows deposition of immunoreactions in a shaggy or granular band at the basement of membrane zone
SLE
what test for SLE
direct immunofluorescent testing
what Ig to look for on positive lupus band test
IgG, IgM, or C3
95% of SLE patients have antibodies against what
anti-nuclear antigens (ANAs)
(has nonspecific findins since seen in other autoimmune disease)
70% of SLE patients have antibodies against what?
double stranded DNA
30% of SLE patients have antibodies against what?
Sm - protein that is complexed w/ small nuclear RNA
what antibodies are very specific against SLE
antibodies against Sm
90% of SLE pt have ___ years to live, 60% are alive at __ years
5, 20
T/F: prognosis of SLE depends on which organs are affected and how frequently the disease is reactivated
TRUE
SLE treatment
- avoid excess sun exposure
- mild active disease: NSAIDs and antimalarial drugs
- severe acute episodes: systemic corticosteroids, other immunosuppresants and immunomodulators
CCLC/SCLE tx
- topical corticosteroids
- if no relief, systemic drugs like SLE