Immunology Flashcards

1
Q

What is atopy?

A

A predisposition of having hypersensitivity reactions to allergens:
- eczema
- asthma
- allergic rhinitis

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2
Q

What is the importance of establishing and excluding allergies?

A

Food allergies can have a large psycho-social burden, particularly in those who have anaphylaxis.

It is not uncommon for symptoms and histories of ‘allergy’ to actually be a somatisation disorder rather than a true allergy. Allergy testing can play a role in reassuring patients they do not have a true allergy to certain foods.

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3
Q

What is the skin sensitisation theory of allergy?

A

There are two main contributors to a child developing an allergy to food:

  1. There is a break in the infant’s skin (ie. eczema, skin infection) that allows allergens from the environment to cross the skin and react with the immune system.
  2. The child does not have contact with that allergen from the gastrointestinal tract, and there is an absence of GI exposure to the allergen.

The theory is that allergens entering through the skin are recognised by the immune system as being foreign and harmful, leading to sensitisation.

When a baby is weaned at around 6 months, if they are regularly eating foods that contain the allergen, their GI tract is regularly exposed to the protein. Therefore, regular exposure to an allergen through food and preventing exposure to the allergen through the skin barrier can help prevent food allergies developing.

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4
Q

What is Type 1 hypersensitivity reaction?

A

IgE antibodies react to an allergen, triggering mast cells and basophils to release histamines and cytokines.

This causes an immediate reaction.

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5
Q

What is a Type 2 hypersensitivity reaction?

A

IgG and IgM antibodies react to an allergen, activating the complement system and leading to direct damage to the local cells.

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6
Q

What is a Type 3 hypersensitivity reaction?

A

Immune complexes accumulate and cause damage to local tissues - often autoimmune.

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7
Q

What is a Type 4 hypersensitivity reaction?

A

Cell mediated hypersensitivity reactions caused by inappropriate activation of T-lymphocytes, leading to inflammation and damage to local tissues.

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8
Q

Give an example of hypersensitivity reactions:

a) Type 1

b) Type 2

c) Type 3

d) Type 4

A

a) food allergy reactions; anaphylaxis; asthma

b) haemolytic disease of the newborn; transfusion reactions

c) SLE; rheumatoid arthritis; HSP

d) organ transplant rejection; contact dermatitis

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9
Q

What are the main ways of testing for an allergy?

A
  • skin prick testing (?sensitisation)
  • RAST testing (?sensitisation)
  • food challenge testing (?allergy)
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10
Q

What is the role of skin prick testing in allergy diagnosis?

A

Allergen solutions are selected and placed at marked points along the skin, alongside a water control and a histamine control.

A fresh needle is used to make a break in the skin at the site of each allergen.

At 15 minutes, the size of the wheals to each allergen are assessed and compared to the controls.

The issue with skin prick testing is it only tests for sensitisation; they often come back showing the patient is sensitised to many things you have tested for, and it doesn’t mean the patient has an allergy.

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11
Q

What is the role of RAST testing in allergy diagnosis?

A

Measures the total and allergen specific IgE quantities in the patients blood sample.

In patients with atopic conditions, the results will often come back positive for everything you test.

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12
Q

What is the role of food challenge testing in allergy diagnosis?

A

GOLD STANDARD

Food challenge is performed in a specialised unit with close monitoring. Patient is given increasing quantities of allergen to assess the reaction.

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13
Q

Management of allergy.

A
  • establish correct allergen
  • avoidance of allergen
  • avoid foods that trigger reactions
  • regular hoovering and changing sheets and pillows
  • stay indoors when pollen count is high
  • prophylactic antihistamines
  • adrenaline auto-injector

In some cases, specialist centres may initiate immunotherapy. The patient is exposed to allergens over months, with the aim of reducing their reaction to certain foods or allergens.

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14
Q

Treatment of allergic reactions following exposure.

A
  • antihistamines
  • steroids
  • intramuscular adrenaline

Antihistamines and adrenaline work by dampening the immune response to allergens.

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15
Q

What type of hypersensitivity reaction is anaphylaxis?

A

Type 1 hypersensitivity reaction - IgE stimulates mast cells to rapidly release histamine and other pro-inflammatory chemicals.

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16
Q

Presentation of anaphylaxis.

A
  • urticaria
  • itching
  • angio-oedema
  • abdominal pain
  • shortness of breath
  • wheeze
  • stridor
  • tachycardia
  • collapse
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17
Q

Management of anaphylaxis.

A

ABCDE approach.

Intramuscular adrenalin - repeat after 5 mins if required.

Antihistamines.

Steroids (IV hydrocortisone).

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18
Q

Blood test to confirm anaphylaxis.

A

Mast cell tryptase within 6 hours of an anaphylactic reaction - this is released during degranulation and stays in the blood for 6 hours.

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19
Q

Indications for adrenalin auto-injectors.

A

Anaphylaxis.

Generalised allergic reactions with risk factors:
- asthma
- poor access to medical treatment (e.g. rural locations)
- adolescents
- nut or insect sting allergies
- significant co-morbidities

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20
Q

What type of hypersensitivity reaction is allergic rhinitis?

A

Type 1 hypersensitivity - IgE mediated response to environmental allergens, causing an inflammatory response in the nasal mucosa.

21
Q

Causes of allergic rhinitis:

a) seasonal

b) perennial

c) occupational

A

a) hay fever

b) house dust mite allergy

c) associated with the school or work environment

22
Q

Presentation of allergic rhinitis.

A
  • runny, blocked and itchy nose
  • sneezing
  • itchy, red and swollen eyes

Allergic rhinitis is associated with a personal or family history of atopy.

23
Q

Triggers for allergic rhinitis.

A
  • tree pollen
  • grass allergy
  • house dust mites
  • pets
  • mould
24
Q

Management of allergic rhinitis.

A
  • trigger avoidance
  • oral antihistamines (non-sedating vs sedating)
  • nasal corticosteroid sprays
  • nasal antihistamines
25
Q

Role of oral antihistamines in allergic rhinitis.

A

Taken prior to exposure to reduce allergic symptoms.

Non-sedating (cetirizine, fexofenadine).

Sedating (chlorphenamine, promethazine).

26
Q

Role of nasal corticosteroid sprays in allergic rhinitis.

A

Fluticasone and mometasone can be taken to suppress local allergic symptoms.

Hold the spray with the left hand when spraying into the right nostril and vice versa.

Aim to spray slightly outward, away from the nasal septum. Do not sniff at the same time as spraying, as this sends the mist straight to the back of the throat.

27
Q

Presentation of Cow’s milk protein allergy.

A

Presentation before 1 year of age:
- bloating and wind
- abdominal pain
- diarrhoea
- vomiting
- urticarial rash
- angio-oedema
- cough or wheeze
- sneezing
- eczema

28
Q

Management of Cow’s milk protein allergy.

A

Avoiding cow’s milk should fully resolve symptoms:
- breast feeding mothers should avoid dairy products
- hydrolysed formulas

29
Q

What are hydrolysed formulas?

A

Milk formula where the proteins have been broken down so they no longer trigger an immune response, but still contains cow’s milk.

30
Q

Prognosis of Cow’s milk protein allergy.

A

Most children will outgrow cow’s milk protein allergy by the age of 3.

Every 6 months, infants can be tried on the first step of the milk ladder and progress up the ladder until they develop symptoms. Over time, they should gradually be able to progress towards a normal diet containing milk.

31
Q

Cow’s milk intolerance vs Cow’s milk allergy.

A

Cow’s milk intolerance presents with gastrointestinal symptoms, but no allergic features (e.g. angio-oedema, rash, sneezing, coughing).

32
Q

Normal number of respiratory infections in a healthy child.

A

4 to 8 respiratory infections per year is normal.

33
Q

Which features associated with recurrent infections should make you consider investigating further for immunodeficiency.

A
  • chronic diarrhoea
  • failure to thrive
  • appearing unusually well despite severe infection
  • more infections than expected
  • atypical infective organisms
34
Q

How should recurrent infections in children be investigated?

A
  • FBC (?neutropenia, lymphopenia)
  • immunoglobulins (?b cell disorder)
  • complement proteins (?complement disorder)
  • HIV test
  • CXR
  • sweat teat (?cystic fibrosis)
  • CT chest (?bronchiectasis)
35
Q

What is severe combined immunodeficiency (SCID)?

A

A number of genetic disorders that result in absent or dysfunctioning T and B cells - causing almost no immunity to infections.

36
Q

Presentation of SCID.

A
  • persistent severe diarrhoea
  • failure to thrive
  • opportunistic infections
  • unwell after live vaccines
  • Omenn syndrome
37
Q

Causes of SCID.

A

Mutations in common gamma chain on the X chromosome - X-linked recessive.

There are many other mutations:
- JAC3 gene mutations
- adenosine deaminase deficiency

38
Q

What is Omenn syndrome?

A

A rare cause of SCID resulting from a genetic mutation that codes for proteins in T and B cells.

It causes abnormally functioning and deregulated T cells that attack the tissues in the fetus or neonate:
- red, scaly, dry rash
- hair loss
- diarrhoea
- failure to thrive
- lymphadenopathy
- hepatosplenomegaly

39
Q

Management of SCID.

A

Fatal unless successfully treated - management should be in an immunology centre:
- immunoglobulin therapy
- sterile environment
- avoid live vaccines
- haematopoietic stem cell transplantation

40
Q

What is selective immunoglobulin A deficiency?

A

Patients have low IgA and normal IgG / IgM.

This causes a milk immunodeficiency, with most patients asymptomatic and never diagnosed.

Patients have a tendency to recurrent mucous membrane infections, such as lower respiratory tract infections, and autoimmune conditions.

41
Q

What is common variable immunodeficiency?

A

Genetic mutation in the genes coding for components of B cells results in a deficiency in IgG and IgA, without deficiency in IgM.

This leads to recurrent respiratory tract infections, leading to chronic lung disease over time.

Patients are unable to develop immunity to infections or vaccinations, and are prone to immune disorders (e.g. rheumatoid arthritis, non-Hodgkin’s lymphoma).

42
Q

What is X-linked agammaglobulinaemia?

A

X-linked recessive conditions resulting in abnormal B cell development, and deficiency in all classes of immunoglobulins. It causes similar issues to common variable immunodeficiency.

43
Q

What is DiGeorge Syndrome?

A

Genetic mutation that causes an incomplete development of thymus gland, resulting in an inability to create functional T cells.

44
Q

Features of DiGeorge Syndrome.

A

CATCH-22 mnemonic:
- Congenital heart disease
- Abnormal facies
- Thymus gland incompletely developed
- Cleft palate
- Hypoparathyroidism / Hypocalcaemia
- 22nd chromosome affected

45
Q

What is ataxic telangiectasia?

A

Autosomal recessive mutation that affects the protein important in DNA coding. This causes:
- low number of T cells and immunoglobulins
- ataxia (cerebellar impairment)
- telangiectasia
- predisposition to cancers
- slow growth and delayed puberty
- accelerated ageing
- liver failure

46
Q

What are complement disorders?

A

Complement proteins are affected, which usually are important in dealing with encapsulated organisms:
- Haemophilus influenza B
- Streptococcus pneumoniae
- Neisseria meningitidis

47
Q

What is a complement deficiency?

A

Deficiencies in complement proteins leads to vulnerability to infective organisms, particularly respiratory tract, ears and throat.

C2 deficiency is the most common complement deficiency.

48
Q

What is hereditary angioedema?

A

Absence of C1 esterase causes intermittent angioedema in response to minor triggers, such as viral infections or stress.

Patients can be treated with IV C1 esterase inhibitor as prophylaxis before dental or surgical procedures.