Immunodeficiency management

Question 1

What is the role of the immune system?

Answer 1

Protect from infection

Question 2

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

Answer 2

Immunodeficiency
Allergy
Autoimmunity

Question 3

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

Answer 3

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

Question 4

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

Answer 4

Less able to recognise and eliminate pathogens and destroy infected cells

Question 5

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

Answer 5

Less able to tolerate commensal and self-antigens

Question 6

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

Answer 6

Innate immune system

Question 7

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

Answer 7

Skin, endothelium

Innate

Question 8

Name some chemical barriers to infection?

What type of immune system is this a part of?

Answer 8

Enzymes (lysozyme), low pH in stomach, complement

Innate

Question 9

What cells are involved in the innate immune system?

Answer 9

Phagocytes - link with adaptive immune system by activating lymphocytes

Question 10

Name the 3 pathways that activate complement?

Answer 10

Classical, lectin, alternate

Question 11

What are the 3 functions of complement?

Answer 11

Opsonisation, activation of inflammatory cells, lysis

Question 12

What organ are involved in the adaptive immune system?

Answer 12

Lymphoid organs

Question 13

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

Answer 13

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

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

Question 14

What is the main function of the adaptive immune system?

Answer 14

Formation of specific antibodies

Question 15

What is the first type of immunoglobulins produced?

Answer 15

IgM not very specific

Question 16

What cells produce antibodies?

Answer 16

B cells

Question 17

How are B cells activated?

Answer 17

TFH cells (T follicular helper cells)

Question 18

What is the function fo CD4+ T helper cells?

Answer 18

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

Question 19

What is the function of CD8+ cytotoxic T cells?

Answer 19

Recognise and kill infected cells

Question 20

What is immunodeficiency?

Answer 20

When one or more components fo the immune system is defective

Question 21

How can immunodeficiency be classified?

Answer 21

Primary (inherited) and secondary (acquired)

Question 22

Consequences of immunodeficiency?

Answer 22

Infection, increased autoimmune disease, malignancy

Question 23

What are the symptoms of immunodeficiency? (SPUR)

Answer 23

Serious infection
Persistence of infections
Unusual sites or organisms
Recurrent infections

Question 24

Signs of immunodeficiency?

Answer 24

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

Question 25

Warning signs of immunodeficiency?

Answer 25

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

Question 26

How can primary immuodeficienies be classified?

Answer 26

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

Question 27

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

Answer 27

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

Question 28

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

Answer 28

Increased fungal and viral infections | e.g. candida, herpes etc

Question 29

What kind of immunodeficiency is likely to get abscesses forming? a) B cell defect b) T cell defect

Answer 29

B cell defect

Question 30

How will an immunodeficiency caused by a phagocytic defect present?

Answer 30

Abscesses Bacterial infection Fungal infection Viral infections uncommon

Question 31

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

Answer 31

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

Question 32

What is SCID?

Answer 32

Severe combined immunodeficiency

Question 33

What is the cause of SCID?

Answer 33

Multiple genes effected causing absence of T cells and B cells

Question 34

How will patient with SCID present?

Answer 34

Lots of recurrent infections of viral, fungal and bacterial origin

Question 35

At what age will SCID usually present?

Answer 35

From birth

Question 36

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

Answer 36

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

Question 37

How will patients with X-linked agammaglobulinaemia present?

Answer 37

Recurrent bacterial infections, absent tonsils

Question 38

What are the causes and effects of common variable immunodeficiency?

Answer 38

Multiple gene defects causing ow but not absent antibodies

Question 39

How does a patient with common variable immunodeficiency present?

Answer 39

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

Question 40

What is the cause of chronic granulomatous disease?

Answer 40

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

Question 41

How does a patient with chronic granulomatous disease present?

Answer 41

Recurrent abscesses - deep-seated - in the liver, soft tissues - fungal origin - high risk of gingivitis

Question 42

What are the causes and effects of chronic mucocutaneous candidiasis?

Answer 42

Cytokine abnormalities causing failure to clear candida

Question 43

What cell is reduced in DiGeorge syndrome?

Answer 43

Thymus doesn't develop so no/reduced T cells

Question 44

How does DIGeorge syndrome present?

Answer 44

Viral, fungal and bacterial infections, learning difficulties

Question 45

What causes causes secondary immunodeficiency?

Answer 45

``` Malnutrition Medication Infection Radiotherpay Splenectomy ```

Question 46

How can drugs cause immunodeficiency?

Answer 46

Cytotoxins can reduce T and B cells and neutrophils | Immunosuppressive drugs

Question 47

How can immunodeficiency affect a dental history taking?

Answer 47

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

Question 48

What oral manifestations are common in immunodeficney patients?

Answer 48

Candidaisis, oral hairy leukoplakia, severe gingivitis, ulceration

Question 49

What can cause oral candida?

Answer 49

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