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Respiratory > Primary Immunodeficiency Disease > Flashcards

Primary Immunodeficiency Disease Flashcards

1
Q

Name pneumonic for remembering the hallmarks of immune deficiency and what it stands for?

A

Serious infections
Persistent infections
Unusual Infections
Recurrent infections

SPUR

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2
Q

if unresponsive to oral antibiotics what type of infection is it?

A

serious

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3
Q

chronic infections are what type of infection?

A

persistent

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4
Q

name some other features of immune deficiency apart from spur

A 
Weight loss or failure to thrive
Severe skin rash (eczema)
Chronic diarrhoea
Mouth ulceration
Unusual autoimmune disease
Lymphoproliferative disorders
Cancer
Family history
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5
Q

when does an immune disorder become secondary?

A

when it involves more than one component of the immune system

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6
Q

name the conditions associated with secondary immune deficiency- physiological immune deficiency

A

extremes of life

eg ageing, prematurity

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7
Q

name the conditions associated with secondary immune deficiency- infections

A

HIV
Measles
compromised immune system cos its fighting something else

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8
Q

why does ageing cause secondary immunodeficient disease?

A

thymus shrinks with age, cant produce T cells

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9
Q

why does prematurity cause secondary immunodeficient disease?

A

doesn’t have all the maternal AB

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10
Q

name the conditions associated with secondary immune deficiency- treatment interventions

A

immunosuppressive therapy
anti-cancer drugs
corticosteroids

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11
Q

name the conditions associated with secondary immune deficiency

A 
physiological deficiency
infection
treatment interventions
malignancy
biochemical and nutritional disorders
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12
Q

describe the features of primary immunodeficiency disorders

A

Immune dysregulation,
Autoinflammatory disorders
Defects in innate and adaptive immunity

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13
Q

what is the first warning sign of PID?

A

recurrent respiratory infections

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14
Q

name 3 upper respiratory complications or PIDs

A

sinusitis
otitis media
laryngeal angioedema

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15
Q

name 3 lower respiratory complications or PIDs

A

malignancies
interstitial lung diseases
pneumonia
bronchitis, bronchiectasis

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16
Q

name 3 Examples of Primary immunodeficiency disorders with significant respiratory tract complications

A

PAD primary antibody deficiency eg. sinusitis and otitis
complement system disorders
pneumonia

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17
Q

What are the microorganisms Involved in Infectious Pulmonary Complications of Primary Immunodeficiency?

A

Streptococcus pneumoniae, Haemophilus influenza

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18
Q

what is PAD?

A

primary antibody deficiency (PAD):

Selective IgA deficiency

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19
Q

d: angioedema

A

by localized swelling that is generally asymmetric owing to increased vascular permeability with leakage of plasma

20
Q

when may a PID be a complement system one?

A 
considered in patients with laryngeal angioedema:
Hereditary angioedema (HAE)
21
Q

d: neutropenia

A

is an abnormally low concentration of neutrophils in the blood

22
Q

name the ways the neutrophil life cycle can go wrong

A

Defects in Neutrophil development
Defects in Neutrophil trans-endothelial migration
Defects in Neutrophil killing

23
Q

what syndrome is severe congenital neutropenia?

A

Kostmann syndrome

24
Q

what is Kostmann syndrome?

A

Failure to produce neutrophils

e.g. Specific failure of neutrophil maturation

25
Q

How can Kotsmann syndrome be treated?

A

with granulocyte colony-stimulating factor (G-CSF), which increases the neutrophil count and decreases the severity and frequency of infections

26
Q

What would you expect to happen if a patient’s phagocytes were unable to bind to endothelial adhesion molecules?

A

recurrent bacterial and fungal infections
v. high neutrophil blood count
deep tissues infected with no pus formation

27
Q

What is Leukocyte adhesion deficiency? LAD

A

autosomal recessive primary immunodeficiency

Results in failure of neutrophil adhesion and migration

28
Q

Clinical presentation of LAD?

A

leucocytosis
localised bacterial infections
infected umbilical stump

29
Q

What is Chronic granulomatous disease, CGD and what is there a problem with?

A

Deficiency of the intracellular killing mechanism of phagocytes - absent respiratory burst

Excessive inflammation
→ failure to degrade chemoattractants and antigens
→ persistent accumulation of neutrophils, activated macrophages and lymphocytes leads to granuloma formation

30
Q

clinical features of CGD?

A 
Recurrent deep bacterial infections
Especially Staphylococcus, Aspergillus, pseudomonas cepacia
Mycobacteria, atypical mycobacteria
Recurrent fungal infections
Failure to thrive
Lymphadenopathy and hepatosplenomegaly
Granuloma formation
31
Q

name some treatments of phagocyte deficiencies

A

immunoglobulin replacement therapy (IVIg)
aggressive management of infection ( antibiotics and anti-fungals)
Definitive Therapy eg stem cell transplant

32
Q

Name 3 important PIDs associated with respiratory complications in children

A

transient hypogammaglobulinemia of infancy
severe combined immunodeficiency
X-linked agammaglobulinemia

33
Q

what is SCID?

A

severe combined immunodeficiency

failure to produce normal B+T cells

34
Q

symptoms of SCIDs?

A 
Unwell by 3 months of age
Persistent diarrhoea
Failure to thrive
Infections of all types
Common infections – more severe than usual
Unusual & opportunistic infections
Vaccine associated diseases
Unusual skin disease
Graft versus host disease
Colonisation of infant’s “empty” bone marrow by maternal lymphocytes 
Family history of early infant death
35
Q

name some potential causes of SCIDs?

A

Deficiency of cytokine receptors
Deficiency of signalling molecules
Metabolic defects
Defective receptor rearrangements

36
Q

what is the commonest form of severe combined immunodeficiency and what is it?

A

X-linked SCID

Mutation of a component of the IL-2 Receptor

37
Q

d: prophylactic

A

intended to prevent disease

38
Q

name some prophylactic treatments

A

Avoid infections
Prophylactic antibiotics
Prophylactic antifungals
No live attenuated vaccines
Aggressive treatment of existing infections
Antibody replacement - Intravenous immunoglobulin

39
Q

name some definitive treatments

A

Stem cell transplant from HLA identical sibling if possible

Stem cell transplant from other sibling or parent, or from matched unrelated donor

40
Q

How does gene therapy work for SCID?

A

Lymphoid cells are dysfunctional
Stem cells can be treated ex vivo to express the missing component
These cells have a survival advantage in vivo

41
Q

what is Bruton’s X-linked hypogammaglobulinaemia?

A

No circulating B cells
No plasma cells
No circulating antibody after first 6 months

BTK* gene is essential for B cell development
(* Bruton’s Tryosine Kinase)

42
Q

name the disorders of T cell effector function

A

cytokine production
cytotoxicity
T/B cell communication

43
Q

what is the function of the IL-12, INFgamma network?

A

defence against intracellular mycobacteria

44
Q

describe how the IL-12 INFgamma network works?

A

infected macrophages produce IL-12
stimulates NK cells and Th1 cells to secrete IFN gamma
which feeds back to macrophages and neutrophils
stimulates the production of TNFalpha
activating NADPH oxidase complex

45
Q

what diseases is the IL-12 INFgamma network linked to?

A

TB
Atypical mycobacteria
BCG infection after vaccination
Deep fungal infections eg aspergillus

Respiratory (27 decks)

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