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Pathology 6: 197-226
Robbins and Cotran
74
Pathology
Graduate
03/16/2011

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Term
Hypersensitivity Type I Description
Definition
Immediate, anaphylactic
Term
Hypersensitivity Type II Description
Definition
Antibody-mediated, autoimmune
Term
Hypersensitivity Type III Description
Definition
Immune complex-mediated, SLE
Term
Hypersensitivity Type IV Description
Definition
Cell-mediated, contact dermatitis, MS
Term
Hypersensitivity Type I Mechanism
Definition
IgE antibody, Th2, Mast cells
Term
Hypersensitivity Type II Mechanism
Definition
IgG, IgM, Phagocytosis, lysis
Term
Hypersensitivity Type III Mechanism
Definition
Antigen-antibody complex deposition, inflammation, necrosis
Term
Hypersensitivity Type IV Mechanism
Definition
Perivascular cellular infiltrates, activated T lymphocytes, cytokines, cytotoxicity, Th1, Th17, CTLs
Term
Type I hypersensitivity
Definition
Allergy, Activates complement 3a/5a. IL-8, mellitin, codeine, adenosine. Th2 cells are vital
Term
Type I initial reaction
Definition
vasodilation, vascular leakage, spasm, secretions
Term
Type I late phase
Definition
2-24 hours. Infiltration, tissue destruction
Term
Th2 cell cytokines for development
Definition
IL-4, IL-5, IL-13
Term
IL-4
Definition
B cell stimulation to IgE
Term
IL-5
Definition
development and activation of eosinophils. Most potent known
Term
IL-13
Definition
enhances IgE production and stimulates epithelial mucus secretion
Term
FceRI
Definition
avidly binds IgE antibodies on mast cells and basophils, sensitizing them to the antibody's target
Term
Leukotriene C4 and D4
Definition
Most potent vasoactive and spasmogenic agents known
Term
Leukotriene B4
Definition
highly chemotactic for neutrophils, eosinophils and monocytes
Term
Prostaglandin D4
Definition
intense bronchospasm as well as increased mucus secretion
Term
PAF
Definition
Platelet activating factor
Term
Platelet Activating Factor
Definition
causes platelet aggregation, release of histamine, bronchospasm, increased vascular permeability, vasodilation
Term
Non-atopic allergy
Definition
Type I reaction to non-chemical stimuli such as temperature extremes and exercise
Term
Type II hypersensitivity
Definition
antibodies react on cell surfaces or in the extracellular matrix. Complement and Fc receptor dependent inflammation. Myasthenia gravis, graves disease
Term
ADCC
Definition
Antibody-dependent cellular cytotoxicity
Term
antibody-dependent cellular cytotoxicity
Definition
cells coated with antibody are killed by a variety of effector cells. Includes transfusion reactions, erythroblastosis fetalis, autoimmune hemolytic anemia, agranulocytosis, thrombocytopenia, hapten drugs
Term
Type III hypersensitivity
Definition
acute necrotizing vasculitis, fibrinoid necrosis, smudgy eosinophilic deposit, SLE. Includes acute serum sickness
Term
Type III hypersensitivity phase I
Definition
Formation of Immune complexes, about a week after injection of the protein
Term
Type III hypersensitivity phase II
Definition
Deposition of Immune Complexes. major influences are characteristics of complexes and local vascular alterations. ogten glomeruli and joints
Term
Type III hypersensitivity phase III
Definition
Tissue Injury. Acute inflammatory reaction. 10 days after antigen administration. fever, urticaria, arthralgias, lymph node enlargement, proteinuria. vasculitis, glomerulonephritis, arthritis. Serum C3 decreased.
Term
Arthus Reaction
Definition
Local Immune complex disease. intracutaneous injection of antigen. Complexes precipitate on vessel walls with superimposed thrombosis
Term
Type IV Hypersensitivity
Definition
antigen-avtivated (sensitized) T lymphocytes including CD4+/8+. Includes DTH. Activated macrophages transform into epithelioid cells, granulomatous inflammation
Term
DTH
Definition
Delayed-type hypersensitivity
Term
Delayed-type hypersensitivity
Definition
includes tuberculin reaction and contact dermatitis
Term
IL-2
Definition
autocrine growth factor of antigen-responsive T cells
Term
IL-1
Definition
Inflammatory cytokine. Secreted by IFN-gamma activated magrophages. Pyrogenic
Term
IL-12
Definition
induces differentiation of CD4+ T-cells into the Th1 subset. Produced by activated macrophages.
Term
IFN-gamma
Definition
Promotes Th1 development. Activates macrophages. DTH response
Term
IL-17, IL-22
Definition
cytokines that recruit neutrophils and monocytes. Produced by Th17 cells
Term
IL-21
Definition
Produced by activated Th17 cells. Amplifies Th17 response.
Term
IL-23
Definition
Close relative of IL-12. Inflammatory cytokine
Term
Th17 differentiation promoters
Definition
IL-1, IL-6, IL-21, IL-23, TGF-beta
Term
Central Tolerance
Definition
self-reactive cells are killed in lymphoid organs. Receptor editing occurs often.
Term
AIRE
Definition
autoimmune regulator. stimulates expression of peripheral tissue restricted self-antigens in the thymus.
Term
Anergy
Definition
prolonged and irreversible functional inactivation of lymphocytes
Term
Peripheral Tolerance
Definition
Anergy when costimulators are not available with antigens. Suppression by regulatory T cells. Deletion by activation-induced cell death
Term
Regulatory T-cells
Definition
CD25+, develop in the thymus, Mutations in Foxp3 break.
Term
IPEX
Definition
Immune dysrelugation, polyendocrinopathy, enteropathy, X-linked. Mutations in the Foxp3 gene that breaks regulatory T-cells.
Term
Deletion
Definition
self-reactive cells receive apoptotic signal through Fas-Fas ligand system and Bim.
Term
Autoimmune lymphoproliferative disease
Definition
Mutations in the FAS gene because deletion cannot occur
Term
Autoimmune susceptibility genes
Definition
PTPN-22, NOD-2, IL-2 receptor (CD25), IL-7 receptor chains. AIRE, CTLA4, PD1, FAS, FASL
Term
Epitope spreading
Definition
As an autoimmune disease matures it will recognize wider versions of it's target.
Term
Th17 response
Definition
More neutrophils with the monocytes than seen in Th1 response.
Term
SLE
Definition
Systemic Lupus Erythromatosus
Term
Systemic Lupus Erythromatosus
Definition
Type III, LE cells, Kidneys, blood, arthritis, HLA-DQ. Butterfly rash, spenomegaly, relapsing fevers, photosensitivity, pleuritic chest pain, hypocomplementemia
Term
Mesangial lupus glomerulonephritis
Definition
Most mild form. mesangial cell proliferation
Term
Focal proliferative glomerulonephritis
Definition
second type, may progress to diffuse
Term
Diffuse proliferative glomerulonephritis
Definition
Most severe. cellular crescents, hematuria as well as proteinuria
Term
Membranous glomerulonephritis
Definition
diffuse thickening of capillary walls. nephrotic syndrome. wire loop lesion
Term
Chronic Discoid Lupus Erythromatosis
Definition
Skin manifestations only. malar rash, urticaria, bullae, maculopapular lesions. Only rare dsDNA antibodies.
Term
Subacute Cutaneous Lupus Erythromatosis
Definition
skin involvement. widespread, superficial, nonscarring. SS-A antigen with HLA-DR3 common.
Term
Homogeneous/diffuse nuclear staining
Definition
chromatin, histone, maybe dsDNA antibodies
Term
Rim/peripheral staining
Definition
dsDNA antibodies
Term
Speckled pattern of staining
Definition
most commonly .non-DNA antibodies. SM, ribonucleoprotein, SS-A/B antibodies
Term
Nucleolar Pattern of Staining
Definition
RNA antibodies
Term
Antibodies diagnostic of SLE
Definition
dsDNA and Sm
Term
Sjogren Syndrome
Definition
keratoconjunctivitys sicca and xerostomia from immunologic destruction of glands. SS-A (ro) and SS-B (la) in many patients. Rh factor in 75%. HRHTLTV1. Appearance of a lymphoma. Previously Mikulicz disease. Lip biopsy diagnositic.
Term
Keratoconjunctivitis
Definition
Blurring of vision, burning, itching
Term
Xerostomia
Definition
dysphagia, cracks/fissures in mouth, parotid gland enlargement.
Term
Scleroderma
Definition
Systemic Sclerosis. Chronic inflammation, microvascular damage, perivascular fibrosis. Autoimmune. ANAs, nailfold capillary loops distorted. sclerodactyly, rubber-hose esophagus, GERD, malabsorption, malignant hypertension. pulmonary hypertension, pericarditis. Raynaud's, dysphagia, CREST
Term
CREST
Definition
calcinosis, reynaud, esophageal dysfunction, sclerodactyly, telangiectasia
Term
diffuse scleroderma
Definition
skin involvement at onset, rapid progression, early visceral involvement.
Term
limited scleroderma
Definition
confined to fingers, forearms, face. late visceral involvement.
Term
Scleroderma ANAs
Definition
DNA topoisomerase I (Anti-Scl 70) is highly specific with more pulmonary fibrosis. Anticentromere antibody is less common and related to CREST
Term
Polyarteritis Nodosa
Definition
necrotizing inflammation of the walls of blood vessels
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