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15 Cards in this Set
- Front
- Back
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SLE
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women of childbearing age
connective tissue disorder affecting Blood, Joints, Skin, Kidneys, Heart, and Lungs Blood - autoimmune hemolytic anemia, leukopenia, thrombocytopenia Lymphatic - painful lymphadenopathy, splenomegaly Joints - small joint inflammation (hands) without deformity Skin - immunocomplexes along basement membrane - Malar butterfly rash Kidney - diffuse proliferative glomerulonephritis Cardiac - Fibrinous pericarditis with effusion, Libman-Sacks endocarditis (sterile vegetations on mitral valve) Respiratory - Interstital fibrosis, pleural effusion with friction rub can cause complete heart block (anti-rho) and spontaneous abortion (antiphospholipid syndrome) |
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Systemic Scleroderma
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women childbearing age
Excessive production of collagen SKIN GI - dysphagia SOLIDS + Liquids (lower 2/3) - Loss of villi MALABSORPTION - wide mouthed diverticuli LUNGS - interstitial fibrosis RESPIRATORY FAILURE (most common cause of death) RENAL - vasculitis involving arterioloes (hyperplastic arteriosclerosis) - infarction + malignant hypertension ANTI - TOPOISOMERASE |
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Dermatomyositis + Polymyositis
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(skin involvment) - DM
Women 40 - 60, increased risk for malg. cancer esp. lung Antibody mediated damage (DM) T cell mediated damage (PM) Muscle pain + atrophy (shoulders) Raccoon eyes (purple discoloration) Increased Creatine Kinase |
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MCTD
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like others but renal involvment uncommon
Anti - RNP |
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General characteristics for B Cell, T cell deficiency
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T (T-IN) - intracellular infections (fungi, parasites, viruses)
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B Cell deficiencys
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cant deal with encapsulated bacteria (s. pneumo)
1) Bruton's agammaglobulinemia 2) IgA deficiency 3) Common variable immunodeficiency |
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Bruton's agammaglobulinemia
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-X-linked Recessive
-Tyrosine kinase mutation -FAILURE OF MATURATION FROM PRE-B to Mature B Cells -recurrent sinus/pulmonary infections -appears after 6 months (maternal ABs) - overall decreased immunoglobulins |
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IgA deficiency
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FAILURE IGA B CELLS -> mature PLASMA CELLS
Sinus/pulm. infections - GIARDIASIS -ANAPHYLAXIS IF EXPOSED TO BLOOD PRODUCTS THAT CONTAIN IgA decreased IgA and secretory IgA |
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Common variable immunodeficiency
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defect in B cell --> plasma cell maturation
Adult immunodef. disorder Sinopulm, GI infxn (GIARDIA), pneumonia, Autoimmune disease decrease overall immunoglobulin |
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T Cell deficiency
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-recurrent intracellular (viruses, fungi, protozoa)
1) DiGeorge's Syndrome |
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DiGeorge's
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Failure of 3rd and 4th pharyngeal puches to develop - NO THYMUS OR PARATHYROIDS
hypoparathyroidism = TETANTY no thymus shadow Pneumocystis jarici infxn increase risk for GVH |
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Combined B + T Cell Disorders
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1) SCID
2) Wiskott - Aldrich syndrome 3) Ataxia- telangiectasia |
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SCID
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Severe combined immunodeficiency
Autosomal recessive buildup of adenine that is toxc to B + T cells due to ADENOSINE DEAMINASE deficiency, decreased deoxynucleoside triphosphate precurosrs for DNA synthesis fucked up CMI decreasd immunoglobulins |
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Wiskott- Aldrich
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X-linked recessive
Progessive loss of T + B cells Triad: 1) eczema 2) thrombocytopenia 3) sinopulmonary infxns inc risk for malignant lymphoma decreased IgM normal IgG Increased IgA + IgE |
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Ataxia - telangectasia
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Autosomal recessive
Mutations in dna repair + thymic hyoplasia cerebella ataxia, telangtasia of eyes and skin risk of lymphoma + leukemia increase A-Fetalprotein |
