Chapter 4: Diseases of the Immune System Flashcards Preview

General Pathology > Chapter 4: Diseases of the Immune System > Flashcards

Flashcards in Chapter 4: Diseases of the Immune System Deck (47)
Loading flashcards...
1
Q

These types of reactions are injurious immune reactions that are inappropriately targeted or inadequately controlled and can result in secondary tissue injury.

A

Hypersensitivity reactions

2
Q

This general type of hypersensitivity reaction is characterized by a reaction to self antigens.

A

Autoimmunity hypersensitivity reactions

3
Q

This general type of hypersensitivity reaction is characterized by an excessive reaction or persistent infection (viral hepatitis, tuberculosis).

A

Microbial hypersensitivity reactions

4
Q

This general type of hypersensitivity reaction is characterized by reactions to harmless stimuli (allergies).

A

Environmental hypersensitivity reactions

5
Q

This type of hypersensitivity is known as an “allergy” and is immediate. It is characterized by the activation of T helper cells, an increase in IgE antibody production, and mast cell degranulation.

A

Type I hypersensitivity

6
Q

This type of hypersensitivity is “cytotoxic” and antibody-mediated (IgG, IgM). It is characterized by opsonization, inflammation, and various non-injurious forms of dysfunction.

A

Type II hypersensitivity

7
Q

This type of hypersensitivity is known as an “immune complex”. It is characterized by antigen exposure leading to antibody production, antigen-antibody complexes, and inflammation (complement activation).

A

Type III hypersensitivity

8
Q

This type of hypersensitivity is T cell-mediated (“delayed,” antibody independent). It is cytokine-mediated (CD4+ T cells) and has direct cytotoxicity (CD8+ T cells attack antigenic cells).

A

Type IV hypersensitivity

9
Q

This type of disorder is characterized by an inappropriate immune reaction due to a failed tolerance of the body’s own cells. Tissue damage can be localized or multisystem.

A

Autoimmune disorders

10
Q

What demographic is the most likely to develop autoimmune conditions?

A

Childbearing aged females

11
Q

This autoimmune condition is characterized by a failed self-tolerance leading to massive antibody formation (anti-nuclear antibodies (ANA), IgG). It is extremely variable, has an insidious onset, and can occur in any organ.

A

Systemic lupus erythematosus (lupus)

12
Q

What types of hypersensitivities are involved in lupus?

A

Type III (immune complex deposition) and Type II (autoantibodies against various cells)

13
Q

What are some possible symptoms of lupus?

A
  • Vessel injury
  • Renal failure
  • Fever of unknown origins
  • Photosensitivity
  • Serositis
  • Hair loss
  • Libman-Sacks endocarditis (mitral valve)
  • Oral ulcers
  • Arthritis
  • CNS (seizures, psychosis)
  • Fatigue, myalgia, pulmonary and liver fibrosis
  • Pancytopenia (reduced number of blood cells)
  • Anti-nuclear antibodies
14
Q

This is a systemic autoimmune disease characterized by exocrine gland (lacrimal and salivary) destruction and enlargement. It is also positive for ANA and a type IV hypersensitivity.

A

Sjogren syndome (SS)

15
Q

This condition is a part of Sjogren syndrome and characterized by dry eyes.

A

Keratoconjunctivitis sicca

16
Q

This condition is a part of Sjogren syndrome and characterized by dry mouth.

A

Xerostomia

17
Q

If Sjogren syndrome is affecting the body alone, what is called?

A

Sicca syndrome

18
Q

Which other autoimmune conditions are patients with Sjogren syndrome most likely to have?

A

Rheumatoid arthritis, lupus, or systemic sclerosis

19
Q

This systemic autoimmune disease is characterized by extensive fibrosis in multiple tissues and vascular destruction in small arteries. It most commonly occurs in the skin.

A

Systemic sclerosis (scleroderma)

20
Q

What are some common features of systemic sclerosis?

A
  • Raynaud phenomenon
  • Malabsorption
  • Dysphagia
  • Renovascular hypertension
  • Renal failure
  • Pulmonary hypertension
  • Dyspnea
  • Myalgia
  • Arthritis
21
Q

This type of systemic sclerosis has mild skin involvement (fingers, face, late visceral involvement) and is also called CREST syndrome.

A

Limited systemic sclerosis

22
Q

What is CREST syndrome?

A
  • Calcinosis
  • Raynaud phenomenon
  • Esophageal dysmotility (fibrosis of the lower 2/3)
  • Sclerodactyly (hard/ eroded skin)
  • Telangiectasia (spider veins)
23
Q

This type of systemic sclerosis has rapid/widespread skin involvement, early visceral involvement, and is aggressive.

A

Diffuse systemic sclerosis

24
Q

This general category of immune deficiency disorders are rare, genetic, and have an early onset (6-24 months).

A

Primary or congenital immune deficiency disorders

25
Q

This primary immune deficiency disorders has an onset of 6 months and often occurs in males. It is caused by failed B cell maturation resulting in the inability to produce antibodies. It is treated by a good prognosis and IgG infusions.

A

X-linked agammaglobulinemia (Bruton disease)

26
Q

This primary immune deficiency disorder is due to a decreased antibody response to infection. Individuals have normal B cells but no plasma cells. This results in an increase in autoimmunity.

A

Common variable immunodeficiency

27
Q

This primary immune deficiency disorder is due to a decreased IgA production. Most cases are asymptomatic or very mild and characterized by upper GI and respiratory infections.

A

Isolated IgA deficiency (most common)

28
Q

This primary immune deficiency disorder is due to an increase in IgM and decrease in IgG, IgA, and IgE. Most are X-linked mutations and are characterized by recurrent pyogenic bacterial infections.

A

Hyper-IgM syndrome

29
Q

This primary immune deficiency disorder is known as the “bubble boy” disorder and is characterized by lymphopenia (no T or B cells) and lymphoid atrophy.

A

Severe combined immunodeficiency (SCID)

30
Q

This primary immune deficiency disorder is due to a defective TH17 resultin in chronic mucocutaneous fungal infections.

A

Defects in lymphocyte activation

31
Q

This primary immune deficiency disorder is characterized by defective complements, phagocytes, TLRs, and can result in multiple infections.

A

Defects in innate immunity

32
Q

This general category of immune deficiency disorders are more common than primary ones and are the result of infections, malnutrition, aging, cancer, and therapy-induced immunosuppresion (most common). It is characterized by suppression of marrow and lymphocyte dysfunction.

A

Secondary (acquired) immune deficiency disorders

33
Q

This secondary immune deficiency disorder is a result of an HIV infection through various bodily fluids (Blood, semen, vaginal fluid, breast milk). It is characterized by a decrease in CD4+ cells and macrophages and paves the way for opportunistic infections, tumors, and CNS defects.

A

Acquired immunodeficiency syndrome (AIDS)

34
Q

What types of tests can be used to screen for HIV?

A
  • ELISA test (gp120)

- Western blot

35
Q

This stage of AIDS occurs after 3-6 weeks after infection and is characterized by pyrexia, pharyngitis, myalgia, and viremia.

A

Acute phase

36
Q

This stage of AIDS occurs 2-10 years after infection and is characterized by generalized lymphadenopathy, a steady decrease of CD4+ cells, and gradually increase viremia. Other features may include a reemergence of shingles, extended pyrexia, fatigue, and candidiasis.

A

Chronic (latent) phase

37
Q

This stage of AIDS occurs when you have 1 month), weight loss and CNS defects.

A

Crisis phase

38
Q

80% of deaths from AIDS are a result of what?

A

Opportunistic infections

39
Q

This condition is due to misfolded proteins being produced and accumulating in a variety of tissues. This can result in damage to tissues and a disruption of function.

A

Amyloidosis

40
Q

This type of amyloid protein is strongly associated with abnormal B cells, which produce irregular plasma cells. Bence-Jones proteins are an example (multiple myeloma)

A

Amyloid light (AL)

41
Q

This type of amyloid protein is associated with chronic inflammation (TB, rheumatoid arthritis, osteomyelitis) and serum-associated amyloid plasma proteins.

A

Amyloid-associated (AA)

42
Q

This type of amyloid protein is associated with amyloid precursor protein leading to the production of cerebral plaques (Alzheimer’s disease). They are also associated with Down syndrome.

A

Beta-Amyloid

43
Q

This type of amyloidosis normally affects multiple organ systems.

A

Systemic amyloidosis

44
Q

What are the three types of systemic amyloidosis?

A
  • Primary (most common): due to malignant plasma cells (AL proteins)
  • Secondary: due to chronic inflammation (AA proteins)
  • Aging
45
Q

This type of amyloidosis normally affects a single organ/tissue and has a large amount of AL proteins.

A

Localized amyloidosis

46
Q

This type of amyloidosis is rare, familial, and geographic. There are a large amount of AA proteins present and most often caused by an inflammasome mutation.

A

Hereditary amyloidosis (Mediterranean fever)

47
Q

What is the most common tissue affected by amyloidosis?

A

Kidneys