Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pathology > Immunopathology > Flashcards

Immunopathology Flashcards

1
Q

Type I Hypersensitivity Rxn

A

Immediate hypersensitivity, injury caused by TH2 cells, IgE antibodies, mast cells, & other leukocytes in response to allergen
Ex: Anaphylaxis, bronchial asthma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Type II Hypersensitivity Rxn

A

Antibody-mediated disorders/complement activation, secreted IgG & IgM antibodies injure cells by promoting phagocytosis or lysis & injure tissues by inducing inflammation
Antibody can stimulate receptor (like TSH - Graves) or inhibit (like ACh - Myastheria gravis))

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Type III Hypersensitivity Rxn

A

Immune complex-mediated disorders, IgG & IgM antibodies bind antigens usually in the circulation, & complex deposit in tissues and induce inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Type IV Hypersensitivity Rxn

A

Cell-mediated immune disorders, sensitized T lymphocytes (TH1 & TH17 cells and CTLs) cause tissue injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Type III Hypersensitivity Rxn

A

Immune complex-mediated disorders, IgG & IgM antibodies bind antigens usually in the circulation, & complex deposit in tissues and induce inflammation (ex: lupus)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Type IV Hypersensitivity Rxn

A

Cell-mediated immune disorders, sensitized T lymphocytes (TH1 & TH17 cells and CTLs) cause tissue injury
(Ex: Rheumatoid arthritis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Th1 activates ____; Th17 activates ____; cytokines secreted by _____

A

IFN-gamma; IL 17, 22; CD4 T cell activated by class II MHC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

CD8 T cell activated by class I MHC causes tissue damage through

A

Direct cytotoxic T cell tissue damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Autograft

A

your own tissues

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Isograft

A

identical twin, same genetic background

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Allograft

A

same species, different genetic background

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Xenograft

A

different species

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Transplant rejection

A

Immune damage resulting from recipient response to allograft HLA antigens
Type IV hypersensitivity rxn

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Direct pathway of transplant rejection

A

Antigen presenting cells in graft (donor antigen presenting cell) present to CD8 & CD4 T-cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Indirect pathway of transplant rejection

A

Own self cells get a hold of one of allogenic peptides & present to immune system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Humoral mechanism of transplant rejection

A

Ab bind to HLA (self) molecules in graft & activate complement
Causes acute inflammation & Type II hypersensitivity
Ag-Ab complexes form in circulation & cause Type III hypersensitivity - necrotizing, immune complex vasculitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Hyperacute rejection

A 
Happens minutes to hours after transplantation
Preformed Ab (from prior transplant, transfusion, etc.) react against Ag in allograft
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Hyperacute rejection causes

A

Type III hypersensitivity (immune complex formation)

Vasculitis w/ fibrinoid necrosis, thrombosis, ischemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Acute cellular rejection

A

Rapid progression after initiation

Occurs days to months after transplant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Acute cellular rejection causes

A

Tubular damage & endothelitis
Extensive interstitial inflammation
Lymphocytic infiltrates & tubular necrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Acute humoral rejection

A 
Necrotizing vasculitis
Intimal thickening (valve smaller) due to accumulation of fibroblasts, foamy macrophages, myocytes, smooth muscle proliferation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Chronic rejection

A

Months to years after transplant
Humoral injury - proliferative vascular lesions
Cellular injury - cytokine induced proliferation of vascular smooth muscle & production of collagen in ECM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Chronic rejection causes

A

Vascular changes, interstitial fibrosis, tubular atrophy, chronic inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Chronic rejection causes

A

Vascular changes, interstitial fibrosis, tubular atrophy (flat epithelium), chronic inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Acute liver rejection

A

Mixed inflammatory cell infiltrates w/ eosinophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Acute liver rejection triad of features

A

Portal tract inflammation
Bile duct epithelial damage
Endothelitis of portal vein & hepatic artery branches

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Chronic liver rejection

A

Progressive disappearance of bile ducts due to direct immunologic destruction or loss of blood supply
Obliterative arteritis from proliferation of intimal layer
Results in portal & hepatic fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Graft arteriopathy

A

Intimal thickening occurs in long lengths of donor vessels

Areas of vessels to narrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Cyclosporine

A

Block nuclear factor of activated T cells (NFAT) necessary for IL-2 stimulation of T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Steroids

A

Suppress macrophage activity & inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Azathioprine

A

Inhibits DNA synthesis of lymphocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Hematopoietic cell transplant

A

Stem cells harvested from donor bone marrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Complications of hematopoietic cell transplant

A

Graft vs. host disease, infection, immunodeficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Graft vs Host Disease (GVHD)

A

Cell mediated rxn
Donor T cells recognize Host HLA antigens as foreign & mount Type IV reaction against graft elements and tissues
Destroy recipient cells (skin, GIT, liver, lungs)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Acute GVHD

A

days to weeks
Donor cytotoxic T cells or cytokines (from helper T cells) destroy epithelial cells
Skin: rash, exfoliation
GIT: ulcerative gastroenteritis
Hepatic: bile duct necrosis
Immunosuppression - more likely to get infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Exfoliation

A

skin starts to peel off

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Chronic GVHD

A

May follow acute or appear without acute phase
Autoreactive T cells derived from donor stem cells
Mimics systemic scerosis - generalized fibrosis of dermis & skin appendages, GI mucosa (strictures), bile ducts (jaundice)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Autoimmune disease criteria

A

Immunological reaction to a self-Ag or native tissue
Reaction primary to pathogenesis, not secondary to tissue damage
No other well-defined cause (exclude other diseases)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Autoimmune disease mechanism

A

Failure of tolerance/immune regulation (failure of T cell anergy, apoptosis of self-reactive cells, or regulatory CD4 T cell; molecular mimicry, polyclonal lymphocyte activation, exposure of cryptic antigenic determinants, emergence of sequestered Ag)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Hyper- or hypothyroidism self antigen

A

TSH receptor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Hyper or Hypoglycemia self antigen

A

Insulin receptor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Myasthenia gravis self antigen

A

Acetylcholine receptor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Blistering skin diseases self antigen

A

Epidermal cell adhesion molecules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Systemic lupus erythematosus (SLE) self antigen

A

Double-stranded DNA

Histones

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Diffuse scleroderma self antigen

A

Topoisomerase I

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Limited scleroderma self antigen

A

Centromere proteins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Thyroiditis self antigen

A

Thyroid peroxidase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Systemic Lupus Erythematosus

A

Multisystem disorder
Clinically acute w/ flares & remissions, often febrile
Affects more females than males (9:1) & more black females
Usually starts between 20 & 40

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

SLE diagnostic criteria

A

Malar rash, Discoid rash, photosensitivity (UV light causes rash), painless oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, antinuclear antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Malar rash

A

fixed erythema, butterfly rash, flat or raised

Spares nasolabial folds usually

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Discoid rash

A

erythematous raised patches with adherent keratotic scaling & follicular plugging; scarring may occur

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

SLE gene locus

A

HLA-DQ locus

Creates B & T cells specific for self nuclear antigens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

SLE environmental triggers

A

UV radiation, viruses, drugs, hormones

Results in defective clearance of apoptotic bodies & increased burden of nuclear antigens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

SLE pathogenesis

A

Antinuclear antibody complexes with self nuclear antigens
TLR stimulation of B cells & dendritic cells to complex - release IFN for further stimulation
High level of anti-nuclear IgG antibody production

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

SLE most common clinical manifestations

A

Hematologic (anemia or leukopenia, lymphopenia, thrombocytopenia; every pt), arthritis, skin rashes, fever, fatigue, weight loss, renal (proteinuria)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Hallmark of Lupus

A

Autoantibodies - antinuclear antibodies (ANAs)

directed against nuclear Ag (DNA, histones, proteins bound to RNA, & nucleolar Ag)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

ANA testing

A

Uses human tissue cell culture nuclei (Hep-2) as substrate
ANAs in serum bind substrate & fluorescent anti-IgG bind Ab
Can visualize amount & pattern of nuclei/Ab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Peripheral (rim) ANA staining pattern

A

Ab to ds-DNA

Seen w/ lupus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Homogeneous (diffuse) ANA staining pattern

A

Ab to chromatin, ds-DNA, histones

Seen w/ lupus & rheumatoid arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

Speckled ANA staining pattern

A

Ab to histones, ribonucleoproteins

Seen w/ lupus, Sjogren syndrome, Systemic Scleroderma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Nucleolar ANA staining

A

Ab to nucleolar RNA

Seen w/ lupus & scleroderma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

SLE common antibody system

A 
Native DNA (anti-ds-DNA), Histones (antihistone)
Less common - Anti-Sm, nuclear RNP, SS-A(Ro), SS-B(La)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Drug-Induced LE common antibody system

A

Antihistone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

Systemic Sclerosis - diffuse common antibody system

A

DNA topoisomerase I (Scl-70), Nuclear RNP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

Limited Scleroderma - CREST common antibody system

A

Centromeric proteins (anticentromere)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

Sjogren Syndrome common antibody system

A

RNP - ribonucleoprotein (SS-A(Ro) & SS-B(La))

67
Q

Homogeneous (diffuse) ANA staining pattern

A

Ab to chromatin, ds-DNA, histones

Seen w/ drug induced lupus & rheumatoid arthritis

68
Q

Speckled ANA staining pattern

A

Ab to histones, ribonucleoproteins - least specific

Seen w/ lupus, Sjogren syndrome, Systemic Scleroderma

69
Q

Nucleolar ANA staining

A

Ab to nucleolar RNA

Seen w/ lupus & systemic sclerosis - CREST

70
Q

SLE common antibody system

A 
Native DNA (anti-ds-DNA), Histones (antihistone)
Less common - Anti-Smith Ab, nuclear RNP
71
Q

Drug-Induced SLE or RA common antibody system

A

Antihistone

72
Q

Centromere ANA staining pattern

A

Ab to centromere

Systemic sclerosis - CREST

73
Q

SLE most common clinical manifestations

A

Hematologic (anemia or leukopenia, lymphopenia, thrombocytopenia; every pt), arthritis (hands, knees, ankles; painful inflammation w/o deformity), skin rashes, fever, fatigue, weight loss, renal (proteinuria)

74
Q

SLE common antibody system

A 
Native DNA (anti-ds-DNA), Histones (antihistone)
Less common - Anti-Smith Ab (ribonucleoprotein), nuclear RNP
75
Q

Lupus vascular changes

A

Circulating immune complexes deposit in vascular beds, activate complement & cause inflammatory (Type III) rxn
Acute: vasculitis w/ fibrinoid necrosis of arteries/arterioles in any tissue
Chronic: layered fibrous thickening (collagen onion skin)

76
Q

Lupus nephritis

A

Immune complex glomerulonephritis
Major cause of death in lupus pts
Immune complex deposition in glomeruli, basement membranes, larger blood vessels
Causes tubulointerstitial changes, thickening of capillary loops, proteinuria

77
Q

Lupus serositis

A

Effusions usually involve pleura & pericardium
Acute: exudation of fibrin
Chronic: proliferation of fibrous tissue, thickened membranes, adhesions

78
Q

Lupus effects on the heart

A

Frequent pericarditis, occasional myocarditis
Usually get nonbacterial endocarditis affecting any valve - appears on both sides of valve (typically AV valves)
Accelerated CAD d/t steroid therapy or immune damage
Common cause of death in lupus pts along with kidney damage

79
Q

Lupus effects on the heart

A

Frequent pericarditis, occasional myocarditis
Usually get nonbacterial endocarditis affecting any valve - appears on both sides of valve (typically AV valves, called Libman-Sacks endocarditis)
Accelerated CAD d/t steroid therapy or immune damage
Common cause of death in lupus pts along with kidney damage

80
Q

Lupus effects on lungs

A

Pleuritis
Acute: pneumonitis w/ alveolar damage, edema, hemorrhage
Chronic: interstitial & vascular fibrosis, pulmonary fibrosis & pulmonary hypertension

81
Q

Lupus effects on the heart

A

Frequent pericarditis, occasional myocarditis
Usually get nonbacterial endocarditis affecting any valve - appears on both sides of valve (typically AV valves, called Libman-Sacks endocarditis)
Accelerated CAD d/t steroid therapy or immune damage
Common cause of death in lupus pts along with kidney damage

82
Q

Lupus effects on CNS

A

involvement common
Focal neurologic defects, seizures, neuropsychiatric symptoms
Small vessel thickening, ischemia, microinfarcts

83
Q

Cause of death with lupus (typically)

A

Renal failure, infection, CAD

84
Q

Severity of lupus symptoms range from

A

mild dermatologic & joint symptoms to life threatening organ failure & cytopenias
Higher titers, more likely to have worse symptoms

85
Q

Chronic discoid Lupus

A

Variety of skin lesions (plaques, erythema, scaling, atrophy) without systemic features
5-10% of cases develop into systemic lupus

86
Q

Subacute cutaneous lupus

A

Diffuse, superficial, nonscarring, photosensitive lesions; mild systemic disease; some consider it an intermediate phase before full systemic lupus

87
Q

Drug Induced Lupus

A

Lupus like syndrome with multiple organ involvement
Rash, fever, arthralgias, serositis; no renal or CNS pathology
Positive ANAs
Drug cessation can cause remission

88
Q

Drug Induced Lupus

A

Lupus like syndrome with multiple organ involvement
Rash, fever, arthralgias, serositis; no renal or CNS pathology
Positive ANAs
Can be caused by drugs used to treat TB
Drug cessation can cause remission

89
Q

Sjogren’s Syndrome

A

Systemic disease, less severe than lupus
Autoimmune destruction of exocrine glands, primarily lacrimal & salivary glands
Mostly affects females in middle age

90
Q

Sjogren’s Syndrome pathogenesis

A

CD4 T cells against glandular epithelial self-Ag initiate disorder
Could be induced by viral infections
Systemic B cell hyperactivity

91
Q

Sjogren’s Syndrome pathogenesis

A

CD4 T cells against glandular epithelial self-Ag initiate disorder
Could be induced by viral infections
Systemic B cell hyperactivity lead to ANAs (auto-antibodies)
ANAs to ribonucleoproteins SS-A & SS-B

92
Q

Sjogren’s Syndrome morphology

A

T cell, B cell, plasma cells infiltrate ducts & vessels in glands
Follicle formation w/ germinal centers
Ductal epithelial hyperplasia causes obstruction
Acinar atrophy, fibrosis, fat replacement of parenchyma

93
Q

Sjogren’s Syndrome clinical course

A

Drying of mucous membranes leads to xerostomia, keratocunjunctivitis, nasal septal erosion, and perforation, dysphagia, dypareunia
Lymph nodes massively hyperplastic
Increased risk of B cell lymphoma
Systemic vasculitis w/ kidney (nephritis), lungs (pulmonary fibrosis), skin, CNS (peripheral neuropathy), & muscle occur 25% of time (associated SS-A Ab)

94
Q

Systemic Sclerosis

A 
Autoimmune disorder
Chronic inflammation, destruction of small vessels, & progressive tissue fibrosis
Rigidity, loss of specialized cells & function in skin & connective tissue
Mostly female (3:1) & 50-60 yr
95
Q

Systemic Sclerosis most common cause of death

A

GIT, kidneys, heart, lungs, musculoskeletal system

96
Q

Systemic Sclerosis pathology

A

Trigger/External stimuli causes endothelial cell injury & Immune response
Injury to blood vessel walls causes narrowing & thickening leading to ischemia/repair
Activation of immune response (T & B cells) causes release of autoantibodies & pro-fibrotic cytokines (TFG-B esp. & PDGF, IL-1, 13) - activate fibroblasts
Fibroblasts increase ECM protein synthesis & cause Fibrosis in skin & parenchymal organs
Result = Fibrosis & autoantibodies

97
Q

What key cytokine leads to fibrosis in Systemic Sclerosis

A

TFG beta

98
Q

Limited Sclerosis (CREST syndrome)

A

Limited skin involvement of face, forearms, & fingers
Late visceral involvement (relatively benign)
Anti-centromere Ab

99
Q

CREST stands for:

A

C: calcinosis (calcium in skin)
R: Raynaud’s phenomenon
E: esophageal dysfunction (acid reflux & decrease in motility)
S: sclerodactyly (thickening & tightening of skin on fingers/hands)
T: telangiectasias (dilation of capillaries - red marks on skin)

100
Q

Raynaud’s phenomenon

A

Spasm of blood vessels in response to cold or stress - tips of fingers pale
Can result in gangrenous necrosis (blackened fingertips, edema, dusky color)
CREST sclerosis symptom

101
Q

Diffuse Sclerosis

A

Widespread skin involvement at onset
Early visceral involvement - multiple organ systems
Rapid progression - more severe
Ab to DNA topoisomerase I (anti-Scl-70)

102
Q

Diffuse Sclerosis skin changes

A

Early: edema, lymphocyte infiltrates
Late: epidermal thinning, dermal/appendage fibrosis, subcutaneous calcifications, contractures (claw fingers, mask facies)
Vascular endothelial damage & fibrosis causes ischemia

103
Q

Diffuse Sclerosis

A

Widespread skin involvement at onset
Early visceral involvement - multiple organ systems (GI, Renal, Lungs, heart, muscle - fibrosis & narrowing)
Rapid progression - more severe
Ab to DNA topoisomerase I (anti-Scl-70)

104
Q

Systemic Sclerosis skin changes

A

Early: edema, lymphocyte infiltrates
Late: epidermal thinning, dermal/appendage fibrosis, subcutaneous calcifications, contractures (claw fingers, mask facies)
Vascular endothelial damage & fibrosis causes ischemia

105
Q

Systemic Sclerosis GI changes

A

Esophagus - collagenization & fibrosis (dysmotility & reflux)
Small bowel - mucosal thinning, loss of villi/microvilli, fibrosis (malabsorption)

106
Q

Systemic Sclerosis renal changes

A

2/3 of cases
Thickening of interlobular arteries, proliferation of intimal cells, collagen deposition, hyaline change
May cause hypertension

107
Q

Systemic Sclerosis cause of death

A

Progressive, survival rate depends on severity (organs involved)
Renal, cardiac (restrictive cardiomyopathy), pulmonary (respiratory insufficiency & pulmonary hypertension), GI (reflux) dysfunction or failure

108
Q

Rheumatoid arthritis

A

Systemic, autoimmune inflammatory disorder
Joints, skin, vessels, heart, lungs, soft tissue - very painful & deforming
More likely in women (3-5:1), any age (esp 40-70)

109
Q

RA pathogenesis

A

unknown trigger
host susceptibility genes HLA-DRB1, PTPN22 (tyrosine phosphatase)
Enzymatic modification of self protein & failure of tolerance = T & B cell responses to self antigens
Production of rheumatoid factor (IgM auto-Ab to Fc portion of IgG) - complex deposition & joint injury
Cause Pannus formation; destruction of bone, cartilage; fibrosis; ankylosis

110
Q

Citrullinated peptides (CCP)

A

Anti-CCP or T cell response to CCP contribute to chronic RA

111
Q

RA morphology

A

Destructive joint inflammation
Edema, synovial cell hyperplasia, stromal & perivascular infiltrates
Fibrin deposition on synovial surface & exudation into joint space - loss of joint spaces
Pannus formation
Articular cartilage erosion, osteoclastic destruction of subchondral bone (causing ankylosis)

112
Q

Pannus formation

A

granulation tissue, synovial & inflammatory cells, fibrous CT
Present in RA

113
Q

Rheumatoid nodules

A

Skin at pressure points (elbow, occiput, lumbosacrum) & viscera (lungs, spleen, heart) develop nodules in 25% cases
Necrotic tissue, palisading macrophages, & fibrosis make up nodule

114
Q

RA clinical course

A

Mild discomfort to progressive disability

Malaise, fatigue, pain, swelling, stiffness, deformity in small & large joints

115
Q

COD in RA

A

disease complications of amyloidosis & vasculitis

Drug therapy complications of bleeding & infection

116
Q

Juvenile Idiopathic Arthritis

A
117
Q

Still’s disease variant

A

Form of Juvenile idiopathic arthritis

Febrile illness with hepatosplenomegaly, rash, & high WBC count

118
Q

Mixed Connective Tissue Disease (MCTD)

A

Clinical syndrome w/ overlapping features of SLE & Systemic Sclerosis
Ab to ribonucleoprotein
Minimal renal disease & good response to steroid therapy
May evolve into classic SLE or SS

119
Q

Immunodeficiency disorder clinically manifest as

A

infection (frequent, severe, resistant)

120
Q

Primary Immunodeficiencies

A

Genetically determined defects of B & T lymphocytes
6 mo - 2 yrs
Repeated infection
Failure to thrive

121
Q

X-Linked Agammaglobulinemia of Bruton

A

Mutation in tyrosine kinase gene (BTK gene) required to mature B cells from pre-B cell into immature B cell
Causes absence of mature B cells & no antibody production (agammaglobulinemia)
X-linked recessive (seen in males)

122
Q

Infections usually seen w/ X-Linked Agammaglobulinemia of Bruton

A

Recurrent sinus, oropharyngeal, & respiratory inf.
D/t Staph, Strep, & H. influenza (all usually opsonized by Ab to be cleared by phagocytosis), enteric viruses & protozoa

123
Q

Tx of X-Linked Agammaglobulinemia of Bruton

A

Parenteral immunoglobulin replacement for life

124
Q

Common Variable Immunodeficiency

A

Hypogammaglobulinemia (usually all Ab classes, but sometimes only IgG)
Occurs 20-30 yr old
Intrinsic B cell defect (in differentiation) or Abnormal T cell signaling to B cells (increased suppressor of decreased helper)
Results in inability of B cells to become plasma cells

125
Q

Infection caused by common variable immunodeficiency

A

Recurrent bacterial infections of sinuses & respiratory tract
Lack of IgA (increased enteroviral infections & protozoa)

126
Q

Selective IgA deficiency

A

Most common, mild
Familial or acquired w/ measles
Serum IgA absent or low because IgA + B cells fail to mature
Other immunoglobulin types normal & cellular immunity intact

127
Q

Infection caused by selective IgA deficiency

A

GI, respiratory, GU infections (secretion related)

Increased risk to develop AI disorders & anti-IgA Ab

128
Q

Hyper IgM syndrome

A

Failure of T cells (CD40 ligand) to induce B cell isotype switching from IgM to IgG, IgA, & IgE & to activate macrophages to remove intracellular microbes
IgM normal or up & all other isotypes absent

129
Q

Infection by Hyper IgM syndrome

A 
Recurrent pyogenic (lack of opsoniziation)
Intracellular organisms (lack of phagocytic activation)
Autoimmune lysis (IgM rxn against blood cells)
GIT lymphoid hyperplastic accumulations of IgM B cells
130
Q

DiGeorge Syndrome

A

Interruption of 3rd & 4th pharyngeal pouch development causes aplasia or hypoplasia of thymus & parathyroids
T cell defect (as well as hypocalcemia, cardiac abnormalities, & defects of face (cleft palate) & aortic arch)
Chromosome 22q11 deletion common
Normal Ig levels

131
Q

DiGeorge Syndrome

A

Interruption of 3rd & 4th pharyngeal pouch development causes aplasia or hypoplasia of thymus & parathyroids
T cell defect (as well as hypocalcemia, cardiac abnormalities (VSD) , & defects of face (cleft palate) & aortic arch)
Chromosome 22q11 deletion common
Normal Ig levels - infection of virus & fungi

132
Q

Severe Combined Immunodeficiency (SCID)

A

Deficient cellular & humoral immune responses

Combined T & B form rare, often just severe T cell defect

133
Q

X-linked pattern of SCID

A

Mutation of common gamma-chain subunit of cytokine receptors

Affects interleukins causing impaired lymphocyte development, proliferation, & function

134
Q

Autosomal recessive pattern of SCID

A 
Adenosine deaminase deficiency causes lympotoxic metabolites (deoxyadenosine, deoxy-ATP)
Failure of class II MHC expression leads to impaired T cell recognition of Ag
135
Q

SCID symptoms

A

Early onset - thrush, diaper rash, failure to thrive
Recurrent infections from all microbes (bacteria, virus, fungi, protozoa)
Requires bone marrow transplantation

136
Q

Wiskott-Aldrich Syndrome

A

Immunodeficiency, thrombocytopenia, & eczema (scaly red rash)
Genetic defect - progressive depletion of T & B cells
Ab levels normal or elevated, IgM is low
Poor or lack of Ab response to protein Ag, & polysaccharide Ag
X linked male

137
Q

Wiskott-Aldrich syndrome symptoms

A

Hemorrhagic diathesis
Recurrent respiratory infections
Pyogenic bacteria, viruses, fungi
Early death (w/o bone marrow transplant)

138
Q

Secondary Immunodeficiencies

A

Immune impairment in previously healthy person
Caused by diseases & physiologic states
Potentially reversible

139
Q

AIDS occurs due to

A

HIV-1 retroviral infection

140
Q

HIV enters cell by using

A

gp120 to bind to CD4 & gp41 to penetrate membrane

141
Q

HIV destroys CD4 T cells through

A
  1. Viral replication in infected T cell = death (cytopathic effect of virus)
  2. Activation of uninfected T cells = activation-induced apoptosis
  3. Expression of HIV peptides on infected T cell = killing of infected cells by virus-specific CTLs
142
Q

HIV causes

A

CD4: Decreased response to antigens & decreased lymphokine secretion
CD8: decreased specific cytotoxicity
NK: decreased killing of tumor cells
B: depressed Ig production in response to new antigens (however have B cell activation with high Ig)
Macrophage: decreased cytotoxic ability, decreased chemotaxis, decreased IL-1 secretion & Ag presentation

143
Q

Early acute phase of AIDS

A

Viral replication, viremia, viral seeding of lymphoid tissues
Fever, sore throat, myalgias

144
Q

Middle, chronic phase of AIDS

A

Replication in lymphoid tissue persistant

Lymphadenopathy, weight loss, night sweats, fatigue, fever, rash

145
Q

Final, crisis phase of AIDS

A

Marked viral replication
Depletion of T cells = profound immune suppression
Fever, fatigue, weight loss, opportunistic diseases, neoplasms

146
Q

AIDS defining neoplasms

A

Kaposi’s sarcoma, B cell lymphomas, primary lymphoma of brain, invasive carcinoma of uterine cervix & anus

147
Q

AIDS neoplasms can occur though

A
  1. Increase in follicular T cell signal B cell hyperplasia (& thus hypermutation) - leads to B cell lymphomas
  2. T cell depletion leads to unchecked infections (like EBV & KSHV) causing virus associated B cell lymphomas
148
Q

Pneumocytis pneumonia

A

Complication of AIDS
Alveoli fill w/ foamy exudate, thickened interstitium
Bronchoalveolar lavage - coffee bean like w/ silver stain

149
Q

Kaposi’s Sarcoma

A

Associated w/ AIDS & immunosuppressed
Caused by KSHV (Kaposi sarcoma-associated herpes virus) due to herpes type 8 (HHV8)
Painful purple nodules on skin - often of hands or feet
Highly vascular

150
Q

Fusion/entry inhibitors

A

Block co-receptors CXCR4 & CCR5 that bind to HIV to let it into cell

151
Q

Reverse transcriptase inhibitors

A 
First class of HIV drugs
Prevents reverse transcriptase of RNA into DNA
152
Q

Integrase inhibitors

A

Prevents HIV DNA from being integrated into host DNA

153
Q

Protease inhibitors

A

Blocks viral maturation by not allowing viral protease to activate viral proteins

154
Q

Amyloidosis

A

Group of disorders - extracellular deposition & accumulation of abnormal, misfolded protein material (amyloids)
Cannot be degraded
Can occur systemically or in one organ/tissue

155
Q

Amyloid stained w/

A

Starch-like staining rxn w/ iodine

Seen with Congo red & green fluorescence under polarized light

156
Q

Amyloid protein fibrils

A

95%
Fold into beta-pleated sheet
Binding sites for Congo red

157
Q

Pentagonal component of amyloid

A

5%

Glyco-protein related to normal serum protein from which it is derived

158
Q

Amyloid derived from

A

conversion of soluble circulating protein precursors into insoluble fibrillar forms

159
Q

Amyloid Light chain (AL)

A

Ig light chains derived from abnormal clones of B cells
Commonly primary (some secondarly to multiple myeloma)
Tends to involve heart, GIT, PNS, skin, & tongue

160
Q

Amyloid associated (AA)

A

Secondary or reactive (after inflammatory or infectious states)
Fibrils related to non-immunoglobulin AA protein & serum precursor (SAA) - from hepatic cells stimulated by IL-1/6
Occurs w/ RA, IBD, drug abuse, renal cell CA, Hodgkin’s lymphoma
Tend to involve kidneys, liver, spleen, lymph nodes, adrenals

161
Q

ATTR amyloid

A

Mutant form of transthyretin (transports thyroxin & retinol) in PNS
Polyneuropathy

162
Q

Beta amyloid protein

A

Deposited in cerebral blood vessels & plaques of pts w/ senile cerebral amyloidosis & Alzheimer’s disease
Severe atrophy of cerebral cortex

163
Q

Cardiac amyloid

A

Builds up between myofibers (subendocardial, interstitial)

Causes stiffness of ventricles

164
Q

Renal amyloid

A

In glomeruli, interstitial, vascular

Kidneys pale & enlarged w/ smooth surface

Pathology (6 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions