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immunology: diseases
mamacitacandida

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antinuclear antibodies (ANA)


SLE


anti dsDNA, anti-Smith


more specific for SLE


antihistone


drug induced lupus (HHIP: hydralazine, INH, procainamide, phenytoin)


anti-IgG (RF)


rhuematoid arthritis


anticentromere


Scleroderma (CREST)


anti-Scl-70 (anti DNA topoisomerase I)


Scleroderma (diffuse)


Antimitochondrial


primary biliary cirrhosis


antigliadin, antiendomysial


celiac disease


anti-basement membrane


Goodpasture\\\'s


anti-desmoglein


Pemphigus vulgaris


antimicrosomal, antithyroglobulin


Hashimotos


Anti-Jo-1


polymyositis, dermatomyositis


Anti-SS-A (anti- Ro)


Sjogren\\\'s Syndrome


Anti-SS-B (anti-La)


Sjogren\\\'s Syndrome


Anti-U1 RNP (ribonucleoprotein)


mixed connective tissue disease


anti-smooth muscle


autoimmune hepatitis


anti-glutamate decarboxylase


TIDM


c-ANCA


Wegener\\\'s


p-ANCA


vasculitides: microscopic polyangitis, Churg-Strauss syndrome


MPO-ANCA


pauci-immune crescentic glomerulonephritis


anti-TSH receptor


Graves disease


anti-ACh receptor


myasthenia gravis


Bruton\\\'s Agammaglobulinemia


X-linked; B cell def --> defective tyrosine kinase gene --> low levels of ALL Ig\\\'s; recurrent bacterial infections after 6m


Thymic Aplasia (DiGeorge\\\'s)


3rd+4th pouches failed to dev=no thymus=no T cells; no PTH --> low Ca --> tetany; congenital heart defect/great vessels; recurrent viral/fungal/protozoal infections; 90% have 22q11 del (FISH)


SCID


defect in early stem cell diff; adenosine deaminase deficiency (purine salvage pway); last defense=NK cells; presentation: severe recurrent infxns (Candida, fatal RSV, VZV, HSV, measles, flu, parainfluenza, PCP pneumo!) + chronic diarrhea + failure to thrive (no thymic shadow on newborn CXR)


Chronic Mucocutaneous Candidiasis


T cell dysfxn (v Candidiasis); Rx=ketoconazole


Wiskott-Aldrich


WAITER: Wiskott-Aldrich, high IgA, Immunodef, Thrombocytopenia+purpura, Eczema (truncal), Recurrent pyogenic infections: no/low IgM; X linked


Ataxia-Telangiectasia


DNA repair defect disorder; IgA deficiency; cerebellar ataxia, poor smooth pursuit of moving target w/eyes; telangectasias of face >5yo; increased CA risk (lymphoma, acute leukemia); radiation sensitivity; +/- high aFP in kids >8 yo; avg age of death=25


X-linked immunodeficiencies (WBC)


Wiskott-Aldrich, Bruton\\\'s, CGD, Hyper-IgM syndrome (high IgM, low IgG), 3 types: X-linked-no CD ligand; AR-no CD40; NEMO deficiency


IgA deficiency


most common; most pts appear healthy; sinus and lung infections; 1/6000 European descent; asscd with atrophy, asthma; possible anaphylaxis due to blood transfusion and blood products


IL12 receptor deficiency


mycobacterial infections (IL12 activates NK, Th1 cells)


Phagocyte Deficiencies


chronic granolumatous dz, Chediak-Higashi disease, Job\\\'s Syndrome, Leukocyte Adhesion Deficiency


CGD


lack of NADPH oxidase activity --> impotent phagocytes --> susceptible to catalse + orgs (S. aureus, E. coli, Klebsiella, Aspergillus, Candida); Dx= (-) nitroblue tetrazolium (NBT) dye; rx=prophylactic TMP/SMX, IFNg helpful


Chediak-Higashi Disease


defective LYST gene (lysosomal transport); defective phagocyte-lysosome interaction --> dx=giant cytoplasmic granules in PMNs; classic triad=partial albinism, recurrent resp tract and skin infections, neurological disorders (eg nystagmus)


Job\\\'s Syndrome aka hyper IgE syndrome


deficient IFNg --> PMNs fail to respond to chemotactic stimuli; high levels of IgE and eosinophils; classic triad=Eczema, recurrent cold staph infections, course facial features (broad nose, frontal bossing, deep set eyes); also common to retain front teeth=2 rows


Leukocyte Adhesion Deficiency (LAD)


abnormal integrins --> phagocytes unable to exit circulation; delayed separation of umbilicus


classic triad: Job\\\'s Syndrome


Eczema, recurrent cold staph infections, course facial features (two rows of teeth)


classic triad: Chediak-Higashi


albinism, recurrent resp tract + skin infections, neurological disorders


classic triad: SCID


severe recurrent infections (including PCP pneumonia), chronic diarrhea, failure to thrive


Type I hypersensitivity (IgE mediated, first and fast, no T cells involved!)


Anaphylaxis (bee sting, food/drug allergies; rx=epi!); Allergic and atopic disorders (rhinitis, hay fever, eczema, hives, asthma)


Type II hypersensitivity (IgG, IgM mediated)


hemolytic anemia (AIHA), pernicious anemia, ITP, erythroblastosis fetalis, acute hemolytic transfusion reactions, rheumatic fever, Goodpasture\\\'s, Bullous pemphigoid, pemphigus vulgaris, Graves\\\' dz, myasthenia gravis


Type III hypersensitivity (immune complex deposits)


SLE, rheumatoid arthritis, polyarteritis nodosa, poststrep glomerulonephritis, serum sickness (first think drugs!), arthus rxn (swelling and inflammation after vaccine), hypersensitivity pneumonitis (farmer\\\'s lung)


Type IV hypersensitivity (aka delayed, T cell mediated, does not involve antibodies!)


TIDM, multiple sclerosis, guillan-barre, hashimotos thyroiditis, graft-v-host dz, PPD test, contact dermatitis (poison ivy, nickel)


deficiency: C1 esterase inhibitor


hereditary angioedema (C1 esterase inhib: suppresses activation of classic pathway on self cells)


deficiency: C3


severe, recurrent pyogenic sinus and respiratory tract infections (strep pneumo, Hib); increased susceptibilty to type III hypersensitivty reactions, esp glomerulonephritis


deficiency: C5-C8 (MAC = C5b-9)


neisseria bacteremia (MAC normally makes a whole in PM)


deficiency: decay accelerating factor (DAF=CD55 + CD59)


complement mediated lysis of RBCs and paroxysmal nocturnal hemoglobinuria (PNH)


main fxn of: C1-C4


viral neutralization


main fxn of: C3b


Binds Bacteria = opsonization


main fxn of: C3a, C5a


A -- think anaphylaxis!


causes of granulomatous dz


TB, fungal infxns (histo), syphilis (gummas!), leprosy, cat scratch fever (bartonella), sarcoidosis, Crohn\\\'s, Berylliosis, Listeria, foreign bodies, Wagener\\\'s, chronic granulomatous disease


when to give preformed antibodies


post exposure: tetanus toxin, botulinum toxin, HBV, Rabies, RSV (To Be Healed Real Rapidly)