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68 Cards in this Set

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antinuclear antibody (ANA)
SLE (lupus)
antibodies that are specific for SLE (lupus)
anti-dsDNA, anti-smith
Antihistone
drug induced lupus
antibody in rheumatoid arthritis
anti-IgG rheumatoid factor
anticentromere antibodies
scleroderma (CREST) calcinosis, Raynaud's phenomeon, esophageal dysmotility, sclerodactyly, telangictasia
what are the antibodies associated with diffuse scleroderma?
anti-scl 70 (anti-DNA toposiomerase I)
antimitochondrial antibody
primary biliary cirrhosis
anti-basement membrane antibody
Goodpasture's disesae
antibody in pemphigus vulgaris
anti-desmoglein also anti-epithelial cell antibody (potentially fatal autoimmune skin disorder with IgG antibody against desmosomes - get intraepidermal bullae involving skin and oral mucosa - positive nikolsky's sign (separation of epidermis upon manual stroking of skin)
antimicrosomal, antithyroglobulin antibodies
hashimoto's thyroditis
Anti-Jo 1 antibody
polymyositis, dermatomyositis
polymyositis: progressive symmertric proximal muscle weakness - often involves shoulders
dermatomyositis: similar to polymyositis but involves malar rash, mechanic's hands, shawl and face rash, grotton's papules, increased risk of malignancy
Antibodies in Sjogren's syndrome
anti-SS-A (anti-Ro) and anti-SS-B (anti-La)
Sjogren's syndrome: dry eyes, dry mouth, and arthritis
Anti-U1 RNP (ribonucleoprotein)
Mixed connective tissue disease
anti-smooth muscle
autoimmune hepatitis
Anti-glutamate decarboxylase
type 1 DM
c-ANCA
Wegener's granulomatosis
p-ANCA
other vasculitides
Defect in BTK a tyrosine kinase gene causes what defect?
X-linked burton's agammaglobulinemia (most common in boys): B cell maturation is blocked
What defect does a child have if they have recurrent bacterial infections after 6 months? Why at that point?
They have Burton's agammaglobulinemia
Occurs at 6 months because that is when the baby has decreased IgG from mom - has problems with opsonization
What are the labs seen in Burton's agammaglobulinemia?
Normal pro-B, decreased B cell maturation, decreased number of B cells, all immunoglobulins are decreased
A defect in CD40L causes what?
Hyper-IgM syndrome - because the antibody is unable to class switch
CD40L is on T cells
What is the defect in a child that has recurrent pyogenic infections early in life? What would labs show?
Hyper-IgM syndrome: increased levels of IgM, decreased levels of IgG, IgA, IgE
What is selective Ig deficiency? What is the most common one?
A defect in isotype switching - deficiency in a specific class of immunoglobulins
most common: IgA deficiency
What does a child have if they have sinus and lung infections, milk allergies and diarrhea? What would cause them to have an anaphylactic reaction?
selective IgA deficiency (most common of the selective Ig deficiencies).
giving the child IgA would cause them to have an anaphylatic reaciton
What is common variable immunodeficiency (CVID)?
defect in B-cell maturation; there are many causes
What immunodeficiency can be acquired in the 20-30's, they have an increased risk of autoimmune disease, lymphoma and sinopulmonary infections
CVID
What are the labs in CVID?
normal number of B cells, decreased levels of plasma cells (cells that actively secrete antibody) and immunoglobulins
What syndrome is caused by 22q11 deletion? What fails to develop? What are some symptoms they would have?
DiGeorge - CATCH 22
Cardiac anomlies
Abnormal facies
T-cell aplasia
Cleft palate
Hypocalcemia
What failed to develop in 22q11 deletion?
3rd and 4th branchial pouches
What does IL-12 receptor deficiency cause?
decreased Th1 response
What condition do you have if you have disseminated mycobacterial infections?
IL-12 receptor deficiency
What labs will you see in IL-12 receptor deficiency?
decreased IFN-y
What stimulates Th1 cells to be made?
IL-12
What stimulates Th2 cells to be made?
IL-4
What inhibits Th1 cells to be made?
IL-10
What inhibit Th2 cells to be made?
IFN-y
What does Th1 cells make?
IL-2, IFN-y
What is Job's syndrome? What labs will you see in it?
Hyper-IgE syndrome
increased levels of IgE
If Th cells fail to produce IFN-y - you have an inability of neutrophils to respond to chemotactic stimuli what do you have?
Hyper IgE syndrome
FATED: facies (coarse), abscesses (cold staphyloccal), teeth (retained primary teeth), increased IgE, Dermatologic problems (eczema)
What do you have if you have chroinc mucocutaneous candidasis?
T cell dysfunction - would have candida albicans infections of the skin and mucous membranes
If a child has IL-2 receptor deficiency what do they have? What are other forms of it?
SCID - severe combined immunodeficiency
x-linked recessive
Other forms include: adenosine deaminase deficiency, failure to synthesize MHC II antigens
If a child has adenosine deaminase deficiency what do they have? What symptoms would they have?
SCID
recurrent viral, bacterial, fungal, and protozoal infections due to both B and T cells deficiency
What is the treatment for SCID? What are the labs?
Tx. bone marrow transplant (no allograft rejection)
Labs: decreased IL-2 receptor (decreased T cell activation)
increased adenine (toxic to B and T cells), decreased dNTP's and decreased DNA synthesis
What is toxic to both B and T cells? what disorder is this thing high?
adenine - high in SCID
What disorder do you have if you have a defect in DNA repair enzymes?
ataxia-telangiectasia
What are symptoms of ataxia-telangiectasia?
cerebellar dysfunction (ataxia), spider angiomas (telangiectasia), IgA deficiency
What labs would you see in ataxia-telangiectasia?
Decreased IgA levels
What is LAD 1?
Defect in LFA-1 integrin (CD18) protein on phagocytes (neutrophils)
If a child has recurrent bacterial infections, absent pus formation and delayed separation of the umbilicus what do they have?
LAD-1 : problem with integrins (CD18)
What would a child's lab be if they had delayed separation of the umbilicus?
LAD -1 : would have neutrophilia (b/c would have problem with tight binding, so all the PMN's would be in the blood)
What is a x-linked defect that has progressive deletion of B and T cells? What are the symptoms?
wiskott-aldrich syndrome
TIE
Thrombocytopenic purpura, infections, ecxema
What are the labs with someone with wiskott-aldrich?
increased IgE and IgA decreased IgM
What is the condition that is autosomal recessive; and a defect in microtubular function with decreased phagocytosis
Chediack-Higashi syndrome (lazy neutrophil)
What would the presentation be of someone with Chediack-Higashi syndrome?
recurrent pyogenic infections by staph and strep, partial albinism, peripheral neruopathy
What disease is caused by a lack of NADPH oxidase?
Chronic graulomatous disease
What is the problem in CGD?
lack of NADPH oxidase - decreased reactive oxygen species (superoxide) and absent respiratory burst in neutrophils
What is LAD 1?
Defect in LFA-1 integrin (CD18) protein on phagocytes (neutrophils)
What is the presentation of someone with CGD?
recurrent infections with catalase positive organisms (S. aureus, E.coli, Aspergillus) - b/c they inhibit the phagocyte from using their superoxide
If a child has recurrent bacterial infections, absent pus formation and delayed separation of the umbilicus what do they have?
LAD-1 : problem with integrins (CD18)
What would a child's lab be if they had delayed separation of the umbilicus?
LAD -1 : would have neutrophilia (b/c would have problem with tight binding, so all the PMN's would be in the blood)
What is a x-linked defect that has progressive deletion of B and T cells? What are the symptoms?
wiskott-aldrich syndrome
TIE
Thrombocytopenic purpura, infections, ecxema
What are the labs with someone with wiskott-aldrich?
increased IgE and IgA decreased IgM
What is the condition that is autosomal recessive; and a defect in microtubular function with decreased phagocytosis
Chediack-Higashi syndrome (lazy neutrophil)
What would the presentation be of someone with Chediack-Higashi syndrome?
recurrent pyogenic infections by staph and strep, partial albinism, peripheral neruopathy
What disease is caused by a lack of NADPH oxidase?
Chronic graulomatous disease
What is the problem in CGD?
lack of NADPH oxidase - decreased reactive oxygen species (superoxide) and absent respiratory burst in neutrophils
What is the presentation of someone with CGD?
recurrent infections with catalase positive organisms (S. aureus, E.coli, Aspergillus) - b/c they inhibit the phagocyte from using their superoxide
Someone with a negative nitroblue tetraxolium dye reduction test has what condition?
CGD - normal individuals have a normal test