Immune Disorders Flashcards
(132 cards)
1
Q
Crystallized eosinophil granules
A
Charcot-Leyden crystals
2
Q
This can occur when immune complexes deposit in vessels in type III hypersensitivity
A
Vasculitis
Often produces fibrinoid necrosis of vascular wall (fibrin-like pink material)
3
Q
This method can be used to see “granular” deposits of immune complexes with the use of labeled antisera against IgG, IgA, IgM, and complement components
A
Direct immunofluorescence
4
Q
Granuloma formation and apoptotic cells may be seen in this type of hypersensitivity
A
Type IV
from macrophage activation and CTL response
5
Q
Low levels of this product that is made in the liver cause edema
A
Albumin
6
Q
Immunoglobulin which precipitates in cold temperatures
A
Cryoglobulin
7
Q
Cryoglobulinemia vasculitis is highly associated with this
A
Hep C infection
8
Q
This immune-complex mediated condition is highly associated with Hep C infection
A
Cryoglobulinemia vasculitis
9
Q
Mixed cryoglobulinemia has both of these types of Ig
These precipitate in tissues and cause damage
A
Polyclonal IgG (against Hep C)
Monoclonal IgM (with Rheumatoid Factor activity)
10
Q
Macrophages with some giant cells and “loose” granulomas are characteristic of this type of hypersensitivity
A
Type IV
11
Q
Antibodies directly on cell causing damage, such as those against ABO blood groups, is this type of hypersensitivity
A
Type II Hypersensitivity
12
Q
Autoimmune disorders occur due to defective this
A
Self tolerance
13
Q
2 characteristics of autoimmune disorders
A
Inherent susceptibility (family hx, HLA, female more common)
Environmental triggers (molecular mimicry, polyclonal activation, tissue damage)
14
Q
Tissue damage can lead to exposure of these, which can cause autoimmunity
A
Autoantigens
15
Q
Inflammation can alter these, leading to autoimmunity
A
Self antigens
16
Q
Antibodies against various (nonspecific) nuclear antigens
Often used as initial screen test for autoimmune diseases
A
Antinuclear antibodies
17
Q
Antinuclear antibodies are originally detected on cultured cells (HEp2 usually) by this
A
Indirect immunofluorescence
18
Q
Antinuclear antibodies are more commonly detected with this testing
A
ELISA/EIA
19
Q
Antibody (usually IgM) against IgG Fc receptor
May be monoclonal or polyclonal
May result from immune activation of various causes; seen in healthy individuals (greater chance with age) and in many autoimmune diseases
A
Rheumatoid Factor
20
Q
What is Rheumatoid Factor?
A
Antibody (usually IgM) against IgG Fc receptor
21
Q
Is Rheumatoid Factor specific?
A
No
Screening for autoimmune disorders
22
Q
Is Rheumatoid Factor monoclonal or polyclonal?
A
May be either
23
Q
Is Rheumatoid Factor seen in healthy individuals?
A
Yes
Higher chance with age
24
Q
High titer Rheumatoid Factor usually seen in this two conditions
A
Rheumatoid arthritis and Systemic Sclerosis
25
Typical onset of Systemic Lupus Erythematosus
Mostly young adults 20-30's, but any age
26
2 very specific autoantibodies associated with Systemic Lupus Erythematosus
Anti-Smith
Anti-dsDNA
27
What is Systemic Lupus Erythematosus mediated by?
Immune complexes and direct cell destruction
28
What organs/tissues does Systemic Lupus Erythematosus affect?
"Any"
Lesions of kidney, serosal membranes, skin, joints most common
29
4 effects of renal lesions in Systemic Lupus Erythematosus
Hematuria
Proteinuria
Increased creatinine (indicates renal failure)
Decreased albumin (causes edema)
30
Basement membrane is normally thin and uniform, however one that is thick with big dark deposits of Ig complexes is characteristic of this condition
Systemic Lupus Erythematosus
31
Malar rash is characteristic of this condition
Systemic Lupus Erythematosus
32
This type of rash is characteristic of Systemic Lupus Erythematosus
Malar rash
33
Rash, often after sun exposure, occurs in this condition
Systemic Lupus Erythematosus
34
Subacute cutaneous lupus erythematosus or Discoid lupus:
Annular photosensitive rash
Scaly with erythematous rim
Subacute cutaneous lupus erythematosus
35
Subacute cutaneous lupus erythematosus or Discoid lupus:
Tend to be transient with no scarring
Subacute cutaneous lupus erythematosus
36
Subacute cutaneous lupus erythematosus or Discoid lupus:
Typically oval rash with raised plaque and erythematous rim
Discoid lupus
37
Subacute cutaneous lupus erythematosus or Discoid lupus:
Deep inflammation
Discoid lupus
38
Subacute cutaneous lupus erythematosus or Discoid lupus:
Produces atrophy, pigment loss, hair loss, scarring
Discoid lupus
39
Condition involving:
Hemolytic anemia (due to autoimmune hemolysis)
Anemia of chronic disease (due to chronic inflammation)
Leukopenia
Immune thrombocytopenia
Systemic Lupus Erythematosus
40
Level of creatinine in Systemic Lupus Erythematosus
High
41
Result of Coomb's test in Systemic Lupus Erythematosus
Positive
Autoimmune hemolysis common
42
Renal lesions in Systemic Lupus Erythematosus are mostly this type
Glomerular
But also affects tubules, vessels
43
Anti-cardiolipin antibodies indicate this condition
Systemic Lupus Erythematosus
44
Condition that most commonly affects kidneys, serosal membranes, skin, joints, lungs and heme
Systemic Lupus Erythematosus
45
Arthritis in Systemic Lupus Erythematosus involves these types of joints
Small joints (hands, fingers)
46
Autoimmune destruction of salivary and lacrimal glands
Sjogren Syndrome
47
Sjogren Syndrome typically involves this age and gender
50-60 year old woman
48
Is Sjogren Syndrome primary or secondary?
May be either
49
Sjogren Syndrome may be secondary, most commonly to this
Rheumatoid arthritis
50
Sjogren Syndrome may result in this symptom complex involving glandular enlargement and inflammation, usually with decreased secretions
Mikulicz Syndrome
51
Mikulicz Syndrome is a symptom complex involving glandular enlargement and inflammation, usually with decreased secretions, and results from this condition
Sjogren Syndrome
52
Sjogren Syndrome involves autoimmune destruction of these
Salivary and lacrimal glands
53
Clinical symptom of Sjogren Syndrome involving dry, irritated eyes, and photobia
Xerophthalmia
54
Clinical symptom of Sjogren Syndrome involving dry, cracked lips and decreased taste, dysphagia to solids
Xerostomia
55
Xerophthalmia and Xerostomia are characteristic of this condition
Sjogren Syndrome
56
In Sjogren Syndrome, these types of cells infiltrate glands
Mostly periductal
CD4+ T cells and B cells
57
Lymphoepithelial lesions are present in this condition
Sjogren Syndrome
58
Keratitis, Ulceration and infection, Oral ulcers, caries and candidiasis, and MALT lymphoma are complications of this condition
Sjogren Syndrome
59
Progressive interstitial fibrosis of skin and other organs
Combination of vascular damage and tissue fibrosis
Always involves vasculature and skin
Variable involvement of other organs
Systemic Sclerosis
60
Other name for Systemic sclerosis
Scleroderma
61
Systemic Sclerosis is a combination of these two things
Vascular damage and tissue fibrosis
(always involves vasculature and skin; variable involvement of other organs)
62
In Systemic Sclerosis, these produce pro-fibrotic cytokines (TGF-beta, PDGF, IL-13)
CD4+ T cells
63
In Systemic Sclerosis, CD4+ T cells produce these 3 pro-fibrotic cytokines
Activate fibroblasts and myofibroblasts to produce collagen
TGF-beta, PDGF, IL-13
64
Type of autoantibodies associated with limited scleroderma
Anti-centromere ANA
65
Type of autoantibodies associated with diffuse scleroderma
Anti-RNA pol III
66
Type of autoantibodies associated with both limited and diffuse scleroderma
Anti-Scl-70 (topoisomerase I)
67
Anti-centromere ANA autoantibodies are associated with this type of scleroderma
Limited
68
Anti-RNA pol III autoantibodies are associated with this type of scleroderma
Diffuse
69
Anti-Scl-70 (topoisomerase I) autoantibodies are associated with this type of scleroderma
Both limited and diffuse
70
Dystrophic calcium deposition is characteristic of this condition
Systemic Sclerosis
71
Symptom of Systemic Sclerosis involving tapered fingers, joint contractures, ischemic digits, nail dystrophy
Sclerodactyly
72
Sclerodactyly is characteristic of this condition
Systemic Sclerosis
73
Facial telangiectasias and tight perioral skin with furrowed lips are characteristic of this condition
Systemic Sclerosis
74
Arteriolar intimal thickening and narrowing is characteristic of this condition
Systemic Sclerosis
75
Raynaud's phenomenon and telangiectasias are characteristic of this condition
Systemic Sclerosis
76
Vascular narrowing and interstitial fibrosis that may decrease renal function and accelerate hypertension is characteristic of this condition
Systemic Sclerosis
77
Mural fibrosis in the GI tract (in esophagus leads to poor peristalsis and in intestine causes poor absorption and dysmotility) is characteristic of this condition
Systemic Sclerosis
78
Vascular ectasias is characteristic of this condition
Systemic Sclerosis
Leads to chronic blood loss and iron deficiency
79
This is the most common cause of death in untreated Systemic Sclerosis
Renal hypertension
80
Tight, thickened skin and atrophic adnexa are characteristic of this condition
Systemic Sclerosis
81
Type of Systemic Sclerosis that mainly involves skin, especially distal fingers and face
Raynauds as a long preclude to skin findings
Limited scleroderma
82
Type of Systemic Sclerosis that involves late systemic findings
Limited scleroderma
83
Type of Systemic Sclerosis that is also known as "CREST syndrome"
(Calcinosis, Raynauds, Esophageal dysmotility, Sclerodactyly, Telangiectasias)
Limited scleroderma
84
Type of Systemic Sclerosis that involves a short prelude of Raynauds
Diffuse scleroderma
85
Type of Systemic Sclerosis that involves earlier and more progressive organ involvement
Diffuse scleroderma
86
Type of Systemic Sclerosis with rapid development and more frequent fatality (most often due to renal or lung disease)
Diffuse scleroderma
87
Anti-centromere autoantibodies are most common in this type of Systemic Sclerosis
Limited scleroderma
88
Overlap syndrome with features of SLE, Scleroderma, Polymyositis
May evolve into single defined syndrome
Clinical – usually a milder course than others
(Constitutional syndrome, Raynauds, Arthralgias, Myalgias)
Mixed connective tissue disease
89
Speckled ANA and anti-U1 RNP are found in this condition
Mixed connective tissue disease
90
Deposition of fibrillar proteins with Beta-pleated sheet structure
Amyloidosis
91
In amyloidosis, there is a deposition of fibrillar proteins with this structure
Beta-pleated sheet
92
This stains with Congo red stain --> Apple green birefriingence
Amyloid
93
In amyloidosis, this stain is used
Congo Red stain
94
Type of Amyloidosis that is the most common form in the US
AL: Light chain amyloidosis
95
Type of Amyloidosis that is seen in plasma cell neoplasms, results in systemic amyloidosis
AL: Light chain amyloidosis
96
Type of Amyloidosis that is most common in the developing world
AA: amyloid associated protein (AAP)
97
Type of Amyloidosis that involves acute phase protein (seen in chronic inflammatory condition), results in systemic amyloidosis
AA: amyloid associated protein (AAP)
98
Carrier molecule for thyroid hormone and retinol
Transthyretin
99
Problems with this protein is seen in Senile cardiac amyloidosis
Transthyretin
100
Problems with this protein is seen in Familial amyloid polyneuropathy
Transthyretin
101
Problems with this protein are seen in Alzheimer's plaques and cerebral amyloid angiopathy
Amyloid precursor protein
102
Type of Amyloidosis due to misfolded Ig light chain deposition
AL amyloidosis
103
AL amyloidosis is caused by this
Misfolded Ig light chain deposition
104
Type of amyloidosis involving neoplastic plasma cells (--> monoclonal immunoglobulin)
AL amyloidosis
105
In AL amyloidosis, light chains (usually lambda) deposit as these
Beta-pleated sheets
106
In AL amyloidosis, these deposit as beta-pleated sheets
Light chains (usually lambda)
107
Type of amyloidosis due to misfolded AAP
AA amyloidosis
108
AA amyloidosis is due to this
Misfolded AAP (amyloid precursor protein)
109
Type of amyloidosis involving a chronic inflammatory state
AA amyloidosis
110
APP amyloidosis is due to this
Misfolded APP
111
In APP amyloidosis, there are deposits in these 2 places
Blood vessels and Neuritic Plaques
112
Type of amyloidosis involving elderly state or increased production
APP amyloidosis
113
Macroglossia is characteristic of this condition
Amyloidosis
114
Impaired glomerular function, mild hepatomegaly, interstitial deposition in heart (decreased myocardial compliance) and tongue (macroglossia), CNS plaques and amyloid angiopathy, and PNS vascular and neural deposition (compression and ischemic neuropathy) are clinical symptoms of this condition
Amyloidosis
115
Typical patient with amyloidosis
70 year old man with multiple myeloma
116
Centromere pattern in indirect immunofluorescence is specific for this
Limited cutaneous systemic sclerosis
117
Anti-Smith autoantibodies are seen in this condition
Systemic lupus erythematosus
118
Anti-dsDNA autoantibodies are seen in this condition
Systemic lupus erythematosus
119
Anti-SS-A, anti-SS-B and anti-RF autoantibodies are seen in this condition
Systemic lupus erythematosus
120
Autoantibodies seen in Systemic lupus erythematosus
Anti-Smith
Anti-dsDNA
Anti-cardiolipin
Anti-SS-A (Ro), SS-B (La), RF
121
Autoimmune disease mediated by immune complexes and direct cell destruction
Systemic lupus erythematosus
122
Are acute phase reactants seen in Systemic lupus erythematosus?
Yes
123
CD4+ T cells produce pro-fibrotic cytokines (TGF-beta, PDGF, IL13) in this condition
Activated fibroblasts and myofibroblasts to produce collagen
Systemic sclerosis
124
Raynaud's phenomenon is seen in these 2 autoimmune conditions
Systemic sclerosis
Mixed connective tissue disease
125
Systemic sclerosis effects on renal vasculature may do this
Accelerate hypertension
126
Drawn, tight facial mask appearance is characteristic of this autoimmune disorder
Systemic sclerosis
127
Most common cause of death in treated systemic sclerosis
Pulmonary disease
(Dyspnea)
128
Dysphagia, gastroesophageal reflux, malabsorption, abdominal pain, renal hypertension, and dyspnea are seen in this autoimmune disorder
Systemic sclerosis
129
What does CREST stand for when used to describe limited scleroderma?
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telangiectasias
130
Most systemic sclerosis patients are positive for this laboratory test
ANA +
131
2 autoantibodies seen in mixed connective tissue disease
Speckled ANA
Anti-U1 RNP
132
Type of amyloidosis characterized by a chronic inflammatory state
AA amyloidosis
