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antinuclear antibodies (ANA)
anti dsDNA, anti-Smith
more specific for SLE
drug induced lupus (HHIP: hydralazine, INH, procainamide, phenytoin)
anti-IgG (RF)
rhuematoid arthritis
Scleroderma (CREST)
anti-Scl-70 (anti DNA topoisomerase I)
Scleroderma (diffuse)
primary biliary cirrhosis
antigliadin, antiendomysial
celiac disease
anti-basement membrane
Pemphigus vulgaris
antimicrosomal, antithyroglobulin
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
vasculitides: microscopic polyangitis, Churg-Strauss syndrome
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)
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
WAITER: Wiskott-Aldrich, high IgA, Immunodef, Thrombocytopenia+purpura, Eczema (truncal), Recurrent pyogenic infections: no/low IgM; X linked
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
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!
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