Immune Diseases

Front 1

Hyper-IgM syndrome

Back 1

AID deficiency or CD40L deficiency
-inability to class-switch
-defective opsonization
-defective macrophage/DC function due to CD40L absence
-increased infection by bacteria

Front 2

Factor H/Factor I/MCP
deficiency

Back 2

-hemolytic uremic syndrome
-Absent Factor I means constitutive C3bBb, constitutive cleavage of C3, constitutive production of C3a-->inflammation
-Infections (C3 deficiency-all used up)
-macular degeneration (due to inflammation)

Front 3

C1-INH deficiency

Back 3

-hereditary angioedema (chronic inflammation/perfusion)

Front 4

Crohn's disease

Back 4

chronic bloody diarrhea/infection
-could be due to NOD deficiency, inability to activate defensin secretion in host cells in response to internalized pathogens

Front 5

Septic shock

Back 5

sepsis causes systemic TNF-alpha secretion
-hemodilation, permeability
-systemic platelet activation = inability to clot anywhere
-induced by LPS

Front 6

Chronic granulomatous disease

Back 6

mutation in NADPH oxidase
-inability to make ROS in macros/neutros
-results in chronic bacterial/fungal infections
-antigen presented normally, T cell IFN-gamma released normally. No kill.
-Proliferating T cells form a granuloma around APC

Front 7

Chediak Higashi

Back 7

defective phagosome/lysosome fusion
-normal ROS production
-No APC presentation
-albinism and death by 10 years

Front 8

Tuberculosis

Back 8

impairs macro-kill by blocking phagosome/lysosome fusion

Front 9

Allergy

Back 9

IgE binds to Fc receptors in mast cells--degranulate
-granules: tryptase, chymase, carboxypeptidase, histamine, heparin (toxic to parasites)
-cytokines also released (TNF, IL-4, IL-13, IL-3, IL-5) for T cells, macros, and eosinophils
-specific allergies result from high titer of specific antibody

Front 10

Grave's Disease

Back 10

hyperthyroidism
-autoantibody to TSH-R (agonistic)
-palpitation, nervousness, fatigue, weight loss
-eventually destroys thyroid cells-->no more TH

Front 11

Myasthenia Gravis

Back 11

Antibodies toward ACh-receptor
-antagonistic
-fatigue, muscle weakness
-thymic hyperplasia (auto-antibodies target thymus)
-ACh-R antibodies can be transferred maternal-fetally

Front 12

ICD

Back 12

Immune complex disease
-immune complexes form in vessels, glomeruli
-can develop from over-abundant antigen
-activates complement, mast cell, inflammation, occlusion

Front 13

Goodpasture's syndrome

Back 13

Glomerulonephritis
-Auto-antibodies directed to glomerular basement membrane prevent podocyte filtration

Front 14

Multiple Schlerosis

Back 14

Auto-antibodies for myelin sheath
-mediated by T-cells for CNS antigens
-induced by blood brain barrier permeable to leukocytes/blood proteins
-mast cells, complement, antibodies, cytokines

Front 15

Type 1 DM

Back 15

T-cells specific for beta-cells
-must eliminate 90% of beta-cells (non mitotic) to develop diabetes
-NOT through antibodies

Front 16

Contact sensitivity

Back 16

T cells specific for skin pathogens
-delayed sensitivity reactions
-requires 2 exposures (memory T cells)
-DC's present langerhans to naive capillary T cells

Front 17

AIRE mutation

Back 17

Necessary for tissue-specific random expression in thymic epithelium.
-mutations result in autoimmunity

Front 18

Rheumatic fever

Back 18

After infection w/ Strep pyogenes
-heart, joints, skin, brain
-X-reactive antibodies between microbe and host (heart valves)
-T cell activation, B cell maturation-->auto immunity
-After strep is cleared, usually T-cells inactivate, self-limiting

Front 19

Tuberculoid leprosy

Back 19

Th1 effector
-IL-2, IFN-gamma, TNF
-low [microbe], normal Ig levels, T cells responsive

Front 20

Lepromatous leprosy

Back 20

Th2 effector
-IL-4, IL-5, IL-10
-high [microbe], hypergammaglobulinemia, low T-cell response

Front 21

Direct allorecognition

Back 21

graft antigens are presented by donor MHC
-recognized as a foreign antigen
-presentor is donor APC

Front 22

Indirect allorecognition

Back 22

graft minor histocompatibility antigens (non-MHC) are presented by recipient APCs

Front 23

Hyperacute rejection

Back 23

within 24 hours due to ABO blood mismatch
-mediated by natural antibodies

Front 24

Acute rejection

Back 24

T-cell mediated
-CTL and macro cytotoxic killing
-Delayed effect w/ antibody against donor endothelium

Front 25

Chronic rejection

Back 25

Scarring due to repeated acute rejections
-chronic inflammation
-platelet deposition (arteriosclerosis), ischemia

Front 26

Cross-matching

Back 26

mix transplant recipient serum w/ donor blood
-agglutination/lysis means preformed antibodies to donor blood are present.

Front 27

Graft-vs-host disease

Back 27

transplanted T-cells through bone marrow
-allogenic toward host
-must first destroy donor T cells w/ antibodies (bone marrow will be re-educated by thymus)

Front 28

Microchimerism

Back 28

Graft not killed
-graft tissue detectable in host skin and lymph nodes
-host cells detected in graft tissue
-possible autoimmunity

Front 29

Xenotransplantation

Back 29

hyperacute rejection of galactosyl by natural antibodies.

Front 30

Immune complex disease

Back 30

Autoimmunity, chronic infections/antibiotics
-occurs in zone of equivalence antigen:antibody
-complexes deposit near antigen
-Fc's recruit neutrophils and macrophages
-occludes organs w/ abundant capillaries

Front 31

Lupus Erythematosis

Back 31

Autoimmune
-antigen is RBC nucleus (released in RBC differentiation)
-Rash due to immune complex in skin capillary
-requires auto-reactive T and B cell
-lupus flair w/ cell damage
-complexes not cleared unless w/ immunosuppression

Front 32

Wheal and Flare

Back 32

vasodilation at lesion, bump in middle
-due to mast cell response to intradermal antigen
-immediate allergic-type reaction

Front 33

Asthma

Back 33

Exercise-induced allergic response

Front 34

Dermatographia

Back 34

no specific antigen.
-hypersensitive mast cells

Front 35

Factor I deficiency

Back 35

Poor innate immune response (macrophage doesn't phagocytose)
-eventually reduces C3 due to constant metabolism

Front 36

X-linked agammaglobulinemia

Back 36

Btk tyrosine kinase mutation.
-NO B CELLS
-no serum immunoglobulins
-can be in female if x-inactivated

Front 37

MHC II deficiency

Back 37

Always fatal.
-no thymic selection of T cells
-bone marrow transplant is useless

Front 38

LFA-1, I-CAM mutation

Back 38

T cell-B cell, monocyte adhesion compromised.