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General Pathology -- Roecker > Gen Path Exam 3 Section 4: Diseases of the Immune System > Flashcards

Gen Path Exam 3 Section 4: Diseases of the Immune System Flashcards

1
Q

What is a systemic autoimmune condition that affects multiple organ systems, called the “great imitator”?

A

Systemic Lupus Erythematosus (SLE)

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2
Q

Lupus (SLE) injures host tissue via what type of hypersensitivity?

A

Type II and Type III hypersentitivites

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3
Q

What is a diagnosis tool for Lupus (SLE)?

A

Anti-nuclear antibodies (ANAs) Test

- ass. with autoimmune conditions and form in response to nuclear components

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4
Q

What is the cause of Lupus (SLE)?

A

idiopathic (cause unknown)

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5
Q

List some of the criteria that someone needs to have at least 4 of to come Lupus?

A
  • malar rash
  • discoid skin rash
  • photosensitivity
  • painless oral ulcers
  • nonerosive arthritis
  • serositis
  • renal disorders
  • neurologic disorders
  • blood cell disorders
  • immunological disorders
  • antinuclear antibodies (ANA)
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6
Q

What individuals are typically affected to Lupus (SLE)?

A

MC to affect reproductive age females
9:1 female-to-male ratio
African-American females have highest risk

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7
Q

What is the familial association with Lupus (SLE)?

A

20% increased risk if family member with it
25% concordance rate in monozygotic twins
3% concordance rate in dizygotic twins

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8
Q

What genes increase your risk of Lupus (SLE)?

A

if have HLA-DR2 or HLA-DR3 have 3-fold increased risk

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9
Q

Photosensitivity

A

increase reactivity of skin to UV light, sunlight;
trigger apoptosis and inadequate clearance of apoptosis bodies thought to contribute to inadequate controlled immune reaction

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10
Q

What are the MC affected tissues in Lupus (SLE)?

A

skin, joints, kidneys, and serosal membranes

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11
Q

How does Lupus affect the kidneys?

A
  • everyone with Lupus has autoimmune reaction occurring in kidney
  • 50% lupus patients have renal damage –> lupus nephritis or glomerulonephritis
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12
Q

What is the MC cause of death in someone with Lupus (SLE)?

A

renal failure

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13
Q

Lupus and Cardiac Tissue

A

~1/2 all cases affect cardiac tissue

  • Pericarditis
  • Myocarditis/Endocarditis/Carditis
  • Libman-Sacks endocarditis
  • Vasculitis (of coronary arteries)
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14
Q

What is an autoimmune condition that produces dry eyes and dry mouth due to CD4+ T cells infitrating lacrimal and salivary glands?

A

Sjogren Syndrome

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15
Q

Keratoconjunctivitis sicca

A

dry eyes

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16
Q

Xerostomia

A

dry mouth; ass. with salivary glands inflammation; enlarged parotid gland is common

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17
Q

What tissues are involved in Sjogren Syndrome?

A
  1. Eyes–> lack of tear production, drying of corneal epithelia, tissue inflammation and may cause ulcers
  2. Mouth –> fissuring of tongue and oral ulcerations
  3. Respiratory tract and vagina = LESS frequently affected
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18
Q

What is the cause of Sjogren syndrome?

A

pathogenesis = poorly understood
Hypothesized: viral infection of salivary glands may increase risk

80% Sjogren patients test positive for ANAs

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19
Q

What individuals are most commonly affected with Sjogren Syndrome?

A

1-4% of population affected
Women ages 30-60 years (does NOT seem to target younger reproductive-age females)
9x more likely in women

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20
Q

Sicca Syndrome

A

when Sjogren Syndrome occurs as an isolated disorder

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21
Q

Secondary Sjogren Syndrome

A

when Sjogren Syndrome develops after onset of another autoimmune condition
60% of all cases (more common that Sicca Syndrome)

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22
Q

Secondary Sjogren Syndrome is most likely to develop in an individual that already has what other disease?

A

Rheumatoid Arthritis

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23
Q

What autoimmune condition causes chronic inflammation in multiple soft tissues and results in fibrosis and hardening of tissues due to excessive collagen deposition?

A

Systemic Sclerosis

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24
Q

What cells are involved in Systemic Sclerosis?

A

CD4+ T-cells responding to idiopathic stimuli

- once rxn starts, cytokines recruit fibroblasts that deposit scar tissue

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25
What individuals are most likely affected by Systemic Sclerosis?
3:1 ratio of female-to-males affected | More common among older adults ~40-60 years old
26
What tissues are commonly involved in Systemic Sclerosis?
soft tissue fibrosis --> damages body's vasculature 95 % cases affect skin 90% alimentary tract 65% Kidneys 50% lungs 30% heart ~10% skeletal muscle and synovial tissues
27
Diffuse Systemic Sclerosis
aggressive and severe; widespread very early in clinical course; involves many areas of skin and many visceral tissues; worse prognosis and do NOT live as long as inds. with Limited Systemic Sclerosis
28
Limited Systemic Sclerosis
less severe form of the disease, involved relatively small areas of skin, confined to fingers, forearms, and face; some may never involve visceral involvement and those who do it is a late onset "CREST"
29
What is the common acronym to describe tissue change in Limited Systemic Sclerosis?
``` CREST Syndrome C- calcinosis of subcutaneous tissues, esp. hands R - raynaud's phenomenon E- esophageal dysmotility S - sclerodactyly T - telangiectasia (AKA "spinder veins") ```
30
Primary Immunodeficiency
inherited, frequently detected b/w 6 months and 2 years of age; due to susceptibility of recurrent infections; "congenital" Ex: Bruton's Disease and SCID
31
Bruton's Disease
Primary Immunodeficiency diease; X-linked agammaglobulinemia (XLA); mutation on X-chromosome; ONLY males affected; mutation on BTK gene; apparent ~ 6 months old-once mothers anitbodies are depleted
32
Bruton's Tyrosine Kinase (BTK) gene mutation causes what?
halts differentiation of pre B-cells into mature and full functioning B Cells; therefore unable to transition into Plasma cells and produce antibodies when needed; results in Agammaglobulinemia
33
Agammaglobulinemia
= an absence of gamma globulins (antibodies) in the blood
34
What is a confirmatory test for Bruton's Disease?
evaluate blood for lack of antibodies or lack of mature B-Cells and genetic testing to look at BTK mutation
35
Why does Bruton's Disease become apparent at ~6 months old?
maternal antibodies depleting; and get recurrent bacterial and viral infections occur - Bacterial Inf. --> usually respiratory tract - Bacterial/viral inf. of middle ear (otitis media) - Viral inf. of GI tract
36
SCID (Severe Combined Immunodeficiency)
Primary Immunodeficiency disorder; group of different disorders grouped together because manifest similar; all patients have impaired development of T-cells/B-cells; most have atrophy and hypoplasia of lymphatic tissues
37
What individuals are most commonly affected with SCID?
MC in Native Americans --> 1 in 65,000 | Other groups --> 1 in 100,000
38
What type of microbial infections frequently appear in 1st year of life in inds with SCID?
- Oral Candadiosis ("thrush") - Candadiasis - Failure to Thrive - Severe diaper rash - cytomegaloviral infection of upper respiratory tract - fungal infections of lungs - varicella zoster viral inf.
39
Secondary Immunodeficiency Disorders
``` acquired; occur late in life; may be result of various traumatic or destructive processes Causes: - cancer mets to bone - bone marrow cancer (leukemia) - HIV inf./AIDS - ionizing radiation therapy - immunosuppressive medications - chemotherapy - sever malnutrition - advanced diabetes - chronic inf. - removal of spleen (splenectomy) ```
40
Aquired Immunodeficiency Syndrome (AIDS)
blood-borne secondary immunodeficiency disease; results from infection of HIV (human immunodeficiency virus); transmitted via bodily fluids that contain virus
41
What are 3 methods for Transmitting HIV?
1. Sexual Transmission (MC ~75%) 2. Parenteral Transmission 3. Mather-to-Infant Transmission (MC reason child may get it)
42
Sexual Transmission of HIV
MC ~75% of infections U.S. MC is men who have sex with men Other Areas MC is male-to-female sex - blood and semen can transmit HIV via contact with oral, anal, or vaginal abrasions - STD's that have ulcerations, increase transmission of HIV during sex
43
Parenteral Transmission of HIV
contact with blood that has HIV, largely limited to drug abusers sharing needles 0.3% accidental needle stick (rare)
44
Mother-to-Infant Transmission of HIV
MC reason why child may get HIV; 2% all cases of AIDS; can cross placenta, breast milk, vagina secretions - Antiretrovial medications have largely eliminated maternal-to-infant HIV transmission in US
45
Does does HIV target in the body?
immune system and CNS | - infects CD4+ T-cells --> when levels reach fewer than 200 cells/micro-liter, ind. considered to have AIDS
46
What is the process of HIV affecting CD4+ T cells?
HIV binds to surface protein gp120 with CD4 protein that is on surface of CD4+ T cells; once bound it interacts with T-cells CCR5 protein and undergoes change and integrated viral RNA into infected T-cell
47
What mutations would make and individual resistant to AIDS?
mutations to CCR5 protein because HIV cannot integrate viral RNA into T cell
48
3 Step Process of HIV Infeciton
1. Acute Phase 2. Chronic Phase 3. Crisis Phase (AIDS)
49
Acute Phase of HIV infection
Stage 1 - death of many CD4+ Tcells and spike in HIV viremia - infects mucosal tissues and begins to spread into local lymph nodes - 3-6 weeks; develop reaction to viral spread, "Acute HIV syndrome" - "flu-like" symptoms, sore throat, myalgia, fever, fatigue, vomiting, diarrhea
50
Chronic Phase of HIV infeciton
Stage 2 - HIV replication within lymphatic tissues (lymph nodes, spleen, and thymus gland - last 2-10 years - steady decline in CD4+ T-cells and gradual increase in HIV viremia - manifest with generalized lymphadenopathy and may be ass. with occasional infections that are mild
51
Crisis Phase of HIV infeciton
Stage 3 - official AIDS severe immune dysfunction; ass. with prominent infections and other disease processes - decline in CD4+ T-cells below level of 200 cells/micro-liter
52
Three Clinical Features of AIDS
1. Opportunistic infections 2. Tumors 3. CNS disorders
53
What do people with AIDS most commonly die from?
~80% of people with AIDS die from lethal infections
54
What are some characteristic infections associated with AIDS?
Pneumocystis Pneumonia Candidiasis Cytomegalovirus (CMV) Tuberculosis (TB)
55
What types of Tumors (secondary neoplasms) usually proliferate with someone with AIDS?
1. Kaposi Sarcoma | 2. Lymphoma
56
Kaposi Sarcoma
vascular tumor; "AIDS-defining" cancer; develops following Kaposi Sarcoma Herpesvrisus (KSHV) inf.--> reemerges from dormancy upon getting AIDS - causes purple spots on skin
57
Lymphoma (and AIDS)
develops in ~5% all patients with AIDS; inds. with history of Epstein-Barr viral infection most likely to manifest with AIDS-related lymphomas
58
CNS disorders and relation to AIDS
~50% all individuals with AIDS manifest with some form of CNS related neurological dysfunction ~90% have evidence of CNS damage upon autopsy
59
Amyloidosis
occurs when extracellular fibrillar proteins (amyloid proteins) are deposited and cause tissue damage and dysfunction
60
What are the 4 forms of Amyloid Proteins?
1. AL (amyloid light) proteins 2. AA (amyloid associated) proteins 3. beta-amyloid (Abeta) 4. Transthyretin (TTR)
61
AL (amyloid light) proteins
- composed of immunoglobulin light chains - characteristically ass. w/ mulitple myeloma (plasma cell caner) - most cases of primary amyloidosis involve AL proteins
62
AA (amyloid associated) proteins
- more likely deposited in inds. w/ chronic inflammation conditions - most cases of secondary amyloidosis involve AA proteins
63
Conditions characteristic to produce AA amyloidosis?
TB, osteomyelitis, rheumatoid arthritis, Crohn disease, or ulcerative colitis
64
Beta-Amyloids
- associated with alzheimer disease | - A-beta plaque are neurotoxic and cause neurodegeneration that results in dementia of Alzheimers disease
65
Transthyretin (TTR) Amyloid Proteins
- a normal protein that accumulates in excessive amounts as a form of amyloidosis - older inds. most likely to have TTR accumulation following mutations for misfolding of this protein - accumulates in walls of heart in patients with senile cardiac amyloidosis
66
Senile systemic amyloidosis
= when TTR proteins associated in many organ systems
67
What are the two patterns of Amyloid Deposition?
1. Systemic | 2. Localized
68
Systemic Pattern of Amyloid Deposition
- involves multiple organ systems - MC cause when AL proteins accumulate following multiple myeloma - poorer prognosis than localized amyloid deposition
69
Localized Pattern of Amyloid Deposition
- singular (isolated) region of body | - common areas = heart, lungs, brain, skin, liver, kidney, spleen, ligaments of wrist, tongue, larynx
70
Diagnosis of Amyloidosis
- requires histological evaluation | - Congo Red dye combined with polarized light produces characteristic Apple-Green Biregringence
71
What is the most common causes of death related to Amyloidosis?
Renal Failure and Heart Failure

General Pathology -- Roecker (7 decks)

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