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19 Cards in this Set
- Front
- Back
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Clinical features of SLE
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Type II and III hypersensitivity reactions. Pancytopenia, arthritis, butterfly rash, diffuse proliferative glomerulonephritis, Libman-Sacks endocarditis. ANA, anti-DNA, anti-Sm, anti-histone (drug-induced lupus). Rx.: steroids
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Sjogren syndrome
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Autoantibodies against lacrimal and salivary glands. Keratoconjunctivitis, corneal ulcers (dry eyes), xerostomia (dry mouth). Anti-Ro (SS-A), anti-La (SS-B). 15% of rheumatoid arthritis patients have Sjogren.
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Mikulicz syndrome
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Enlargement of the salivary and lacrimal glands associated with Sjogren syndrome
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Diffuse scleroderma
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Fibroblast stimulation and deposition of collagen in the skin and internal organs. Anti-DNA topoisomarease I (Scl-70) (helicase). Skin involvement, dysphagia, malabsorption, pulmonary fibrosis (dyspnea), cardiac fibrosis (arrhythmias), kidney fibrosis (renal failure)
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Localized scleroderma (CREST)
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Fibroblast stimulation and deposition of collagen. Anti-centromere antibodies. Calcinosis, Raynaud, esophageal dysmoility, sclerodactyly, telangiectasia.
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Bruton's agammaglobulinemia
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X-linked recessive. No B cells, No Igs. Recurrent staph, haemophilus and strep infections after 6 months. Increased pre-B cells. Mutation of B-cell Bruton tyrosine kinase (btK).
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Common variable immunodeficiency
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B-cell maturation defect. Hypogammaglobulinemia, recurrent bacterial infections, giardia lamblia.
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DiGeorge syndrome
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Failure to develop 3rd and 4th pharyngeal puches results in absence of parathyroid and thymus glands, hypocalcemia, tetany, T-cell deficiency, recurrent viral infections, heart defects, chronic candidiasis
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SCID
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B and T cell deficiency due to mutation of IL-2 receptor (x-linked), adenosine deaminase deficiency (AR) or failure to make MHC II. Recurrent infections and susceptibility to candida, CMV and p. carinii
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Wiskot-Aldrich syndrome
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X-linked recessive. "WIPE": recurrent infections, thrombocytopenic purpura, eczema, risk of lymphomas, low IgM
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Ataxia-Telangiectasia
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Ataxia, spider angiomas, low IgA, defect of DNA repair enzyme
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Chronic granulomatous disease
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Low NADPH oxidase. Recurrent catalase+ infections, negative nitroblue tetrazolium test.
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Leukocyte adhesion deficiency
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Defect of CD-18 (LFA-1 beta chain), no pus formation, failure of umbilical cord to detach
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Chediak-Higashi
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Defect in microtubulus with no phagocytosis by lysosome. Partial albinism, peripheral neuropahty, recurrent infections
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Hereditary angioedema
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Edema at mucosal surfaces. Defect of C1-INH (esterase inhibitor). Decreased C1, C2, C4
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Hyper IgM
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Defect of CD-40L on T-lymphocytes. No isotope switching, increased IgM
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MHC-I deficiency
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Normal CD4, no CD8. Failure of TAP-1 to transport peptides to MHC-I groove
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Amyloid stains
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Stains red with Congo-red stain then apple green birefringence under polarized light
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Signs and symptoms of amyloidosis
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Nephrotic syndrome, renal failure, arrythmias, CHF, hepatosplenomegaly, macroglosia
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