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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
Q

What individuals are most likely affected by Systemic Sclerosis?

A

3:1 ratio of female-to-males affected

More common among older adults ~40-60 years old

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26
Q

What tissues are commonly involved in Systemic Sclerosis?

A

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

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27
Q

Diffuse Systemic Sclerosis

A

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

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28
Q

Limited Systemic Sclerosis

A

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”

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29
Q

What is the common acronym to describe tissue change in Limited Systemic Sclerosis?

A 
CREST Syndrome
C- calcinosis of subcutaneous tissues, esp. hands
R - raynaud's phenomenon
E- esophageal dysmotility
S - sclerodactyly 
T - telangiectasia (AKA "spinder veins")
30
Q

Primary Immunodeficiency

A

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
Q

Bruton’s Disease

A

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
Q

Bruton’s Tyrosine Kinase (BTK) gene mutation causes what?

A

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
Q

Agammaglobulinemia

A

= an absence of gamma globulins (antibodies) in the blood

34
Q

What is a confirmatory test for Bruton’s Disease?

A

evaluate blood for lack of antibodies or lack of mature B-Cells and genetic testing to look at BTK mutation

35
Q

Why does Bruton’s Disease become apparent at ~6 months old?

A

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
Q

SCID (Severe Combined Immunodeficiency)

A

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
Q

What individuals are most commonly affected with SCID?

A

MC in Native Americans –> 1 in 65,000

Other groups –> 1 in 100,000

38
Q

What type of microbial infections frequently appear in 1st year of life in inds with SCID?

A
  • 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
Q

Secondary Immunodeficiency Disorders

A 
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
Q

Aquired Immunodeficiency Syndrome (AIDS)

A

blood-borne secondary immunodeficiency disease; results from infection of HIV (human immunodeficiency virus); transmitted via bodily fluids that contain virus

41
Q

What are 3 methods for Transmitting HIV?

A
  1. Sexual Transmission (MC ~75%)
  2. Parenteral Transmission
  3. Mather-to-Infant Transmission (MC reason child may get it)
42
Q

Sexual Transmission of HIV

A

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
Q

Parenteral Transmission of HIV

A

contact with blood that has HIV, largely limited to drug abusers sharing needles
0.3% accidental needle stick (rare)

44
Q

Mother-to-Infant Transmission of HIV

A

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
Q

Does does HIV target in the body?

A

immune system and CNS

- infects CD4+ T-cells –> when levels reach fewer than 200 cells/micro-liter, ind. considered to have AIDS

46
Q

What is the process of HIV affecting CD4+ T cells?

A

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
Q

What mutations would make and individual resistant to AIDS?

A

mutations to CCR5 protein because HIV cannot integrate viral RNA into T cell

48
Q

3 Step Process of HIV Infeciton

A
  1. Acute Phase
  2. Chronic Phase
  3. Crisis Phase (AIDS)
49
Q

Acute Phase of HIV infection

A

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
Q

Chronic Phase of HIV infeciton

A

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
Q

Crisis Phase of HIV infeciton

A

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
Q

Three Clinical Features of AIDS

A
  1. Opportunistic infections
  2. Tumors
  3. CNS disorders
53
Q

What do people with AIDS most commonly die from?

A

~80% of people with AIDS die from lethal infections

54
Q

What are some characteristic infections associated with AIDS?

A

Pneumocystis Pneumonia
Candidiasis
Cytomegalovirus (CMV)
Tuberculosis (TB)

55
Q

What types of Tumors (secondary neoplasms) usually proliferate with someone with AIDS?

A
  1. Kaposi Sarcoma

2. Lymphoma

56
Q

Kaposi Sarcoma

A

vascular tumor; “AIDS-defining” cancer; develops following Kaposi Sarcoma Herpesvrisus (KSHV) inf.–> reemerges from dormancy upon getting AIDS
- causes purple spots on skin

57
Q

Lymphoma (and AIDS)

A

develops in ~5% all patients with AIDS; inds. with history of Epstein-Barr viral infection most likely to manifest with AIDS-related lymphomas

58
Q

CNS disorders and relation to AIDS

A

~50% all individuals with AIDS manifest with some form of CNS related neurological dysfunction
~90% have evidence of CNS damage upon autopsy

59
Q

Amyloidosis

A

occurs when extracellular fibrillar proteins (amyloid proteins) are deposited and cause tissue damage and dysfunction

60
Q

What are the 4 forms of Amyloid Proteins?

A
  1. AL (amyloid light) proteins
  2. AA (amyloid associated) proteins
  3. beta-amyloid (Abeta)
  4. Transthyretin (TTR)
61
Q

AL (amyloid light) proteins

A
  • composed of immunoglobulin light chains
  • characteristically ass. w/ mulitple myeloma (plasma cell caner)
  • most cases of primary amyloidosis involve AL proteins
62
Q

AA (amyloid associated) proteins

A
  • more likely deposited in inds. w/ chronic inflammation conditions
  • most cases of secondary amyloidosis involve AA proteins
63
Q

Conditions characteristic to produce AA amyloidosis?

A

TB, osteomyelitis, rheumatoid arthritis, Crohn disease, or ulcerative colitis

64
Q

Beta-Amyloids

A
  • associated with alzheimer disease

- A-beta plaque are neurotoxic and cause neurodegeneration that results in dementia of Alzheimers disease

65
Q

Transthyretin (TTR) Amyloid Proteins

A
  • 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
Q

Senile systemic amyloidosis

A

= when TTR proteins associated in many organ systems

67
Q

What are the two patterns of Amyloid Deposition?

A
  1. Systemic

2. Localized

68
Q

Systemic Pattern of Amyloid Deposition

A
  • involves multiple organ systems
  • MC cause when AL proteins accumulate following multiple myeloma
  • poorer prognosis than localized amyloid deposition
69
Q

Localized Pattern of Amyloid Deposition

A
  • singular (isolated) region of body

- common areas = heart, lungs, brain, skin, liver, kidney, spleen, ligaments of wrist, tongue, larynx

70
Q

Diagnosis of Amyloidosis

A
  • requires histological evaluation

- Congo Red dye combined with polarized light produces characteristic Apple-Green Biregringence

71
Q

What is the most common causes of death related to Amyloidosis?

A

Renal Failure and Heart Failure

General Pathology -- Roecker (7 decks)

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