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CC2 - exam preparation > Immunodeficiencies and autoimmunity > Flashcards

Immunodeficiencies and autoimmunity Flashcards

1
Q

What are some functions the immune system?

A

Immunosurveillance against faulty cells

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2
Q

What cells are part of the acquired immune system?

A

B cells

T cells

NTK cells (both)

Gamma Delta T cells (both)

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3
Q

What is C1 inhibitor deficiency known as?

A

Hereditary angioedema

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4
Q

How many primary immunodeficiency diseases are there?

A

320

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5
Q

What might make you think someone has an immunodeficiency?

A

Recurrent infections

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6
Q

What are the main primary immunodefiencies?

A

ADA or X linked SCID (The one every knows)

XLA (the one associated with BTK, sinusitis, brochniectasis, otitis media)

Common variable immunodeficiency (CVID). (the adult version of XLA)

Hyper-IgM. (the forgotten one)

Neutropenia and neutrophil conditions (chronic granulomatous disease NADPH oxidase deficiency, severe cogneital neutropenia (ELASE deficiency) and adhesion (CD18 leukocyte adhesion deficiency) (the one with severe lympahdenopathy and recurrent infections)

Complement deficiences (the intriguing one) - causes meningitis risk increase to be very high

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7
Q

What is SCID? Types

How does it present?

What is the treatments

A

About: Results in negative T cells, B cells and K cells as all are from a common lymphoid precursor. Adenosine deaminase deficiency (ADA) is one cause due to toxicity and metabolism, whereas X-linked is seen in boys and was made famous by the ‘boy in the bubble’.

Presentation: Recurrent infections L. Very young children. <2 x 10^9 cells/L.

Prophylaxis: avoid all live vaccines such as BCG (BGCosis), Yellow fever and MMR.
Treatment: Enzyme replacement? Bone marrow transplant?

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8
Q

What are the main live vaccines avoided in SCID?

A

MMR, BCG (BCGosis) and yellow fever

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9
Q

XLA, hyper-IGM and CVID are all antibody linked conditions. What are the roles of antibodies?

A

Opsonisation

Agglutination

Neutralisation

Particularly helpful against coagulase positive staph and catalase strep

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10
Q

What is XLA?

What causes XLA?

How does it present?

How can XLA be managed?

A

About: XLA is a recessive condition affecting males and causes a decrease in antibody numbers. Antibodies needed for opsonisation, neutralisation and agglutination predisposing more risk to s.aureus, strep, haemophilis influenza and enteric infections.

Cause: Mutation in BTK.

Presentation: Recurrent infections L. Bronchiectasis. Sinusitis. Otitis media. Enteritis. Young children. Adults more likely to have CVID. Presents from 6 months due to mother’s fall in placental IgG.

Prophylaxis: antibodies and self administration of IVIG/ SCIG (intravenous or subcutaneous). This causes problems with adolescence due to the difficult transition. Patients should be under an immunology and respiratory consultant.

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11
Q

How would an XLA present?

A

Recurrent otitis media, sinusitis, brochiectasis after 6 months due to low number of antibodies

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12
Q

Why does XLA become more apparent at 6 months?

A

The mother’s antibodies IgG via placenta decrease

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13
Q

Who is affected by XLA?

A

Males

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14
Q

What is hyper IgM?

A

Faulty CD40 deficiency or mutation that causes aberrant class switching. As a consequence there would be far more IgM than IgG and IgA causing recurrent infections. Presentation: Recurrent infections L. Affected mucous membranes.

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15
Q

What is CVID?

A

About: Common variable immunodeficiency (CVID) is an immune disorder characterized by recurrent infections and low antibody levels, specifically in immunoglobulin (Ig) types IgG, IgM and IgA.

Presentation: Generally, symptoms include high susceptibility to foreign invaders, chronic lung disease, and inflammation and infection of the gastrointestinal tract. Seen in adults.

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16
Q

What are three common neutropenia or neutrophil disorders?

What are some key characteristics?

A

Severe congenital neutropenia

CD18 leukocyte adhesion disease

Chronic granulmoatous disease

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17
Q

Why do lymph nodes enlarge in neutrophil conditions? Name one

A

Chemotaxis affected in CD18 adhesion leukocyte deficiency

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18
Q

What is chronic granulomatous disease?

A

NADPH oxidase

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19
Q

What is a complement deficiency likely to cause?

A

Neisseria meningtidis (may present later)

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20
Q

What does CVID stand for?

A

Common variable immunodeficiency

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21
Q

What are the most common effects of agammaglobulinemias?

A

The most common bacterial infections that occur in people with X-linked agammaglobulinemia are lung infections (pneumonia and bronchitis), ear infections (otitis), pink eye (conjunctivitis), and sinus infections (sinusitis).

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22
Q

Which virus is particularly harmful in the absence of NK cells?

A

The viruses that seem to be best targeted by NK cell mediated defenses are those of the Herpesvirus family. There are 2 major types of NK cell deficiencies {NKD}. Classical NKD {CNKD} is defined as an absence of NK cells and their function among peripheral blood lymphocytes.

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23
Q

What does a T cell deficiency cause risk of?

A

Fungal disease

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24
Q

What are the symptoms of Neisseria?

A

Severe headache

Neck stiffness

Photophobia

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25
Q

Suggest is the difference between primary and secondary autoimmunity.

A

Secondary immunodeficiencies are far more common than primary immunodeficiencies, which are, by definition, caused by genetic defects affecting cells of the immune system.1 Secondary immunodeficiencies result from a variety of factors that can affect a host with an intrinsically normal immune system, including infectious agents, drugs, metabolic diseases, and environmental conditions.

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26
Q

Outline the main causes of secondary autoimmunity and explain.

A
  1. Medications like PPI increase c difficile and anticholinergics reduce salvia increasing dental carries
  2. Morbid obesity causes cytokine release/ pro-inflammatory and also increases aspiration pneumonia due to breathing problems
  3. Haematological malignancy like CLL and multiple myeloma increase risk of infection
  4. Surgeries like splenectomy
  5. Chemotherapy leads to neutropenic sepsis
  6. Pre-existing disease like Still’s disease affects GM-CSF
  7. Biologics like rituxamab target CD20, inflicimab and enteracept anti-TNFalpha, tocilizumab Il-6, eculizumab targets complement
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27
Q

What diseases are linked with HLA-DR4?

A

RA, MS, T1DM

5-7% of population

More scandinavia

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28
Q

What are three classes of drug for autoimmunity?

A

Steroids (risk of bone fractures and skin atrophy)

Antimetabolites like methotraxate for dihydrofolate reductase, azathioprine for 6-MP and mycophenolate/ MPP for ionosine monophosphate dehydrgenase

Calcineurin inhibitors like tacromilus

29
Q

What does AZA/ azathioprine target?

A

azathioprine for 6-MP

30
Q

What does methotraxtae target?

A

Antimetabolites like methotraxate for dihydrofolate reductase,

31
Q

What does mycophenylate target?

A

ionosine monophosphate dehydrgenase

32
Q

What is IPEX?

A

Immunodysregulation polyendocrinopathy enteropathy X-linked (or IPEX) syndrome is a rare disease linked to the dysfunction of the gene encoding transcription factor forkhead box P3 (FOXP3),

IPEX is a genetic disease of immune dysregulation in which patients can present early in life with diarrhea , diabetes and eczema. It is extremely rare, affecting 1 in every 1.6 million people.

33
Q

What is pre-test probability?

A

The likelihood of symptoms informing you what the diagnosis is e.g. MI is easier to diagnose than tiredness for SLE. MI has higher pre-test probability

34
Q

What are the markers of RA?

A

anti-CCP

35
Q

What is anti-RO and LA?

A

(anti–Sjögren’s-syndrome-related antigen A autoantibodies, also called anti-Ro, or similar names including anti-SSA/Ro, anti-Ro/SSA, anti–SS-A/Ro, and anti-Ro/SS-A)

36
Q

What is anti-JO and MI?

A

Anti-Jo-1 antibody is a myositis specific autoantibody most commonly found in patients with idiopathic inflammatory myopathies (IIM). Myopathies (Jo is in pain)

37
Q

How is ANA testing done?

A

Antibodies taken from sufferer or plasma and added to HEP cells that bind to markers in nucleus

38
Q

What is a marker of allergy?

A

IgE

Tryptase from mast cells

39
Q

What are aeroallergens?

A

Aeroallergens are various airborne substances or inhalants, such as pollens, spores, and other biological or non-biological airborne particles that can cause allergic disorders

40
Q

What is rhinorrhea?

A

Rhinorrhea or rhinorrhoea is the free discharge of a thin nasal mucus fluid. The condition, commonly known as a runny nose, occurs relatively frequently. Rhinorrhea is a common symptom of allergies (hay fever) or certain viral infections, such as the common cold

41
Q

What is needed for an allergy to occur?

A

Exposure –> sensistisation –> specific IgE –> re-exposure and acute response.

Everyone allergic or not is sensistised

42
Q

What is the difference between type 1 and 4 sensitivity?

A

Type 4 has a response that can take >4 hours to occur or a day or 2 after and last a while

More likely to cause rash than brochoconstriction

Rash, itch, arthralgia, myalgia

Type 1 <1 hour, vomiting low blood pressure, high heart rate

43
Q

What is the receptor on mast cells for IgE called?

A

Fce1r

44
Q

What do mast cells release?

A

PAF! Platelet activating factor

Prostaglandin

Leukotrienes

Histamine

Tryptase

45
Q

When checking for hayfever what should you check?

A

When the hayfever occurs. If it is seasonal t could be trees in the spring i.e. birch, plane, ash or it could be grasses like rye and meadow in the summer.

All year long suggests dustmites.

46
Q

What pollen is high in the summer?

A

Rye, meadow, nettle, cladosporium (mould)

47
Q

What is cladosporium?

A

Indoor mould

48
Q

What is non-allergic hayfever?

A

Vasomotor symptoms

49
Q

Is specific IgE a useful test for allergy sufferers?

A

Not really in atopy because IgE is high. Check total IgE too

50
Q

What are treatments for type 1 hypersensitivity?

A

Anti-histamines

Steroids

Avoidance

Type 4: avoidance

51
Q

What is the difference between food allergy and food intolerance?

A

Usually the type of reaction like anaphylaxis

52
Q

What is sodium chromoglycate?

A

A mast cell stabiliser

53
Q

What % of patients claim to have a penicillin allergy?

A

10%

54
Q

What are the four types of drug reaction?

A

Immediate

Delayed

Idiosyncratic

Side effects

55
Q

What is the difference between immediate and delayed?

A

<1 h vs > 1 h

Urticaria, angioedema, vomit, BP vs rash, myalgia, arthralgia

56
Q

What are examples of idiosyncratic drug reactons?

A

SJS, TEN

57
Q

What is a de-label?

A

Where the patients risk to penicillin may be re-evaluated based on the history

58
Q

What % of fatal drug reactions are caused by penicillin?

A

26%

59
Q

What should you check?

A

When was the drug take? What reaction occured? Were other drugs used at the time?

60
Q

What can be given to patients with food allergy?

A

Auto-injectors

61
Q

What dose of adrenaline must be given in anaphylaxis?

A

1 in 1000 (0.5ml)

(asystole is much higher at 1:10000)

62
Q

What is anaphylaxis?

A

A serious life-threatening allergic reaction which usually occurs within few seconds or minutes of exposure to allergic substances. This involves hives, swelling and sudden drop in the blood pressure and sometimes shock.

63
Q

What is scombroid poisoning?

A

Symptoms of scombroid poisoning generally begin quickly, about 30 minutes to 1 hour after ingestion of the poison and include:

nausea,

vomiting,

flushing,

abdominal cramps,

diarrhea, and

headache.

64
Q

What is carcinoid syndrome?

A
65
Q

Describe steps to manage anaphylaxis?

A

A-E

66
Q

What position should the patient be in when giving pencillin?

A

Lying flat

67
Q

What is the biphasic reaction in anaphylaxis?

A

A biphasic reaction is the recurrence of anaphylaxis symptoms within 72 hours of the initial anaphylactic event, without re-exposure to the trigger. Biphasic reactions are uncommon and unpredictable. Prior studies suggest that wheezing and delayed treatment with epinephrine may be associated risk factors.

You must monitor the patient

68
Q

What two things are critical following treating a patient with anaphylaxis?

A

Monitoring for biphasic reaction

and documenting for future references

69
Q

What are the main differences between anaphylaxis and delayed reaction?

A

Anaphylaxis involves drop in BP/ increase heart rate, vomiting, angioedema, urticaria, vs type 4 with myalgia and rash

CC2 - exam preparation (76 decks)

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