Immunodeficiency management Flashcards

1
Q

What is the role of the immune system?

A

Protect from infection

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2
Q

What 3 conditions can present when the immune system goes wrong?

A

Immunodeficiency
Allergy
Autoimmunity

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3
Q

What 2 functions of the immune system should always stay balanced?

A

The ability to protect from infection - recognise and eliminate pathogens, destroy infected cells
AND
The ability to tolerate self-antigens - tolerate commensal organisms and tolerate healthy self-antigens

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4
Q

If an individual has immunodeficiency, in what way are the functions of the immune system not balanced?

A

Less able to recognise and eliminate pathogens and destroy infected cells

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5
Q

If an individual has allergy/autoimmunity, in heat way are the function of the immune systems not balanced?

A

Less able to tolerate commensal and self-antigens

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6
Q

What type of immune system is constant, the 1st level of defence and does not change in individuals?

A

Innate immune system

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7
Q

What are the first physical barriers to infection? What type of immune system is this a part of?

A

Skin, endothelium

Innate

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8
Q

Name some chemical barriers to infection?

What type of immune system is this a part of?

A

Enzymes (lysozyme), low pH in stomach, complement

Innate

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9
Q

What cells are involved in the innate immune system?

A

Phagocytes - link with adaptive immune system by activating lymphocytes

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10
Q

Name the 3 pathways that activate complement?

A

Classical, lectin, alternate

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11
Q

What are the 3 functions of complement?

A

Opsonisation, activation of inflammatory cells, lysis

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12
Q

What organ are involved in the adaptive immune system?

A

Lymphoid organs

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13
Q

How are lymphoid organs classified and what are the functions of each?

A

Primary lymphoid organs - where lymphocytes are produced (bone marrow, thymus)

secondary lymphoid organs - where adaptive responses start (apendoids, tonsils, lymph nodes, spleen, Peters patch)

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14
Q

What is the main function of the adaptive immune system?

A

Formation of specific antibodies

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15
Q

What is the first type of immunoglobulins produced?

A

IgM not very specific

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16
Q

What cells produce antibodies?

A

B cells

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17
Q

How are B cells activated?

A

TFH cells (T follicular helper cells)

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18
Q

What is the function fo CD4+ T helper cells?

A

Help other cells by producing activating cytokines to help recognise antigen presented by DCs

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19
Q

What is the function of CD8+ cytotoxic T cells?

A

Recognise and kill infected cells

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20
Q

What is immunodeficiency?

A

When one or more components fo the immune system is defective

21
Q

How can immunodeficiency be classified?

A

Primary (inherited) and secondary (acquired)

22
Q

Consequences of immunodeficiency?

A

Infection, increased autoimmune disease, malignancy

23
Q

What are the symptoms of immunodeficiency? (SPUR)

A

Serious infection
Persistence of infections
Unusual sites or organisms
Recurrent infections

24
Q

Signs of immunodeficiency?

A
Acute infection
Chronic infection
Damage from infections (bronchiectasis)
Autoimmune features (endocrine disease)
Specific findings e.g. eczema, thrombocytopenia
25
Q

Warning signs of immunodeficiency?

A

8 or more new ear infections in 1 yea
2 or more serious sinus infections in 1 year
2 or more pneumonias in 1 year
Recurrent, deep, skin or organ abscesses
2 or more deep-seated infection such as osteomyelitis, cellulitis
Antibiotics for 2 months without effect
Persistent thrush in mouth or else where after age 1 year
Family history

26
Q

How can primary immuodeficienies be classified?

A
By the type of cell it effects e.g.
Antibodies (B cells)
T cells
Neutrophils
Complement 
Innate
27
Q

How will an immunodeficiency caused by a B cell defect present?

A

Increased bacterial infections especially capsulated bacteria e.g. streptococci, staphylococci

28
Q

How will an immunodeficiency caused by a T cell defect present?

A

Increased fungal and viral infections

e.g. candida, herpes etc

29
Q

What kind of immunodeficiency is likely to get abscesses forming?

a) B cell defect
b) T cell defect

A

B cell defect

30
Q

How will an immunodeficiency caused by a phagocytic defect present?

A

Abscesses
Bacterial infection
Fungal infection
Viral infections uncommon

31
Q

How will an immunodeficiency caused by a complement defect present?
This depends on the pathway

A

Defect in classical pathway will cause recurrent bacterial infections (meningitis)
Viral and fungal infections are rare
If the alternate or lectin pathway are defected it will present as meningitis but due to neisserial infection

32
Q

What is SCID?

A

Severe combined immunodeficiency

33
Q

What is the cause of SCID?

A

Multiple genes effected causing absence of T cells and B cells

34
Q

How will patient with SCID present?

A

Lots of recurrent infections of viral, fungal and bacterial origin

35
Q

At what age will SCID usually present?

A

From birth

36
Q

What does X-linked agammaglobulinaemia result in? (cause)

A

Lack of B cells and reduced immunoglobulins due to mutation in a gene that makes tyrosine kinase which is involved in B cell maturation

37
Q

How will patients with X-linked agammaglobulinaemia present?

A

Recurrent bacterial infections, absent tonsils

38
Q

What are the causes and effects of common variable immunodeficiency?

A

Multiple gene defects causing ow but not absent antibodies

39
Q

How does a patient with common variable immunodeficiency present?

A

Recurrent bacterial infections in sinus, lungs, ears and eyes
high risk of lymphoma

40
Q

What is the cause of chronic granulomatous disease?

A

Genetic defects causing neutrophils that do not produce oxidative bursts, so they cannot kill invading organisms

41
Q

How does a patient with chronic granulomatous disease present?

A

Recurrent abscesses

  • deep-seated
  • in the liver, soft tissues
  • fungal origin
  • high risk of gingivitis
42
Q

What are the causes and effects of chronic mucocutaneous candidiasis?

A

Cytokine abnormalities causing failure to clear candida

43
Q

What cell is reduced in DiGeorge syndrome?

A

Thymus doesn’t develop so no/reduced T cells

44
Q

How does DIGeorge syndrome present?

A

Viral, fungal and bacterial infections, learning difficulties

45
Q

What causes causes secondary immunodeficiency?

A
Malnutrition
Medication
Infection
Radiotherpay
Splenectomy
46
Q

How can drugs cause immunodeficiency?

A

Cytotoxins can reduce T and B cells and neutrophils

Immunosuppressive drugs

47
Q

How can immunodeficiency affect a dental history taking?

A

Look for recurrent infections, family history, drugs they are taking

48
Q

What oral manifestations are common in immunodeficney patients?

A

Candidaisis, oral hairy leukoplakia, severe gingivitis, ulceration

49
Q

What can cause oral candida?

A

Antibiotic therapy, dentures, steroid inhalers, secondary immunodeficiency, primary immunodeficiencies