3. Autoimmunity Flashcards

1
Q

what drives autoimmune diseases?

A

Presence of auto antibodies or auto reactive T cells resulting in organ fibrosis

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2
Q

What factors influence the development of autoimmune diseases?

A

Genetic factors
Environmental factors
Hormonal factors - female after puberty
Infectious microbe exposure

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3
Q

Why do some infections trigger autoimmune diseases?

A

Some microbes have a similar epitope to one present within the body - mimicry

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4
Q

What is autoimmunity?

A

Immune response against the host due to the break in immunological tolerance for self-antigen(s).

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5
Q

What is an autoimmune disease?

A

Disease caused by tissue damage or disturbed physiological responses due to an auto-immune response

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6
Q

What 2 groups can autoimmune diseases be split into?

A

Organ specific

Non-organ specific

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7
Q

What is the difference between organ and non-organ specific autoimmune diseases?

A

Organ specific : auto antigen only present in one organ resulting in organ/tissue specific damage

Non-organ specific : autoantigen found in multiple sites resulting in damage throughout the body. Usually type III hypersensitivity

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8
Q

What investigation results would confirm presence of an autoimmune disease?

A

Serology or biopsy showing the presence of auto reactive T cells / auto antibodies at a level of which correlates with disease severity and activity

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9
Q

What are the most common autoimmune diseases in the UK?

A

Systemic lupus erythematosus

Sjögren’s syndrome

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10
Q

What autoimmune diseases have primary autoantibodies driving the disease?

A
Graves’ disease (anti-TSHR)
Myasthenia gravis (anti-acetylcholine receptor )
Lambert-Eaton myasthenia syndrome (anti-voltage-gated Ca2+ channel)
Goodpastures syndrome (anti-anti-glomerular basement membrane)
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11
Q

What autoimmune diseases are driven by secondary autoantibodies?

A
SLE  (anti-nuclear)
Pernicious anaemia (anti-gastric parietal cell)
Hashimoto thyroiditis (anti-thyroid peroxidase)
Rheumatoid arthritis (anti-rheumatoid factor)
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12
Q

Why might babies of mothers with autoimmune diseases experience symptoms?

A

Autoimmune disease can be transferred to neonates as maternal autoantibodies can be transferred but this effect diminishes by 6 months when maternal IgE/IgG fades.

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13
Q

What are the treatment options for autoimmune diseases?

A

Plasma exchange to remove auto antibodies (plasmapheresis)
Immunosuppressive drugs to suppress auto reactive T cells
Anti-inflammatory drugs (corticosteroids) to treat the tissue damage
Replacement therapy surgery to treat organ dysfunction

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14
Q

What causes autoimmune rheumatic diseases?

A

Result from a break in immune tolerance by producing pathogenic antibodies which result in a heterogenous group of diseases which affect multiple systems.

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15
Q

How is autoimmune rheumatic disease diagnosed?

A

Investigations showing presence of autoantibodies
Clinical features
Patient history

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16
Q

On clinical examination, how will a patient with autoimmune rheumatic disease present?

A

Ulcers
Muscle weakness
Alopecia
Rash

17
Q

What investigations should be done in a patient with suspected autoimmune rheumatic disease?

A
Clinical examination
FBC
Urea
Electrolytes
Creatinine
Liver enzymes
CRP
Plasma viscosity 
ESR  
Antibodies
18
Q

What are the risk factors for SLE?

A

Female
Afro-Caribbean
South Asian

19
Q

What is the treatment for SLE?

A

Lifestyles modifications
DMARDs (disease-modifying antirheumatic drugs)
Steroids
IV cyclophosphamide (in severe cases)

20
Q

What are the risk factors of rheumatoid arthritis?

A

Female

CVS disease

21
Q

What is used in the diagnosis of rheumatoid arthritis?

A

S factor diagnosis - stiffness, swelling, squeezing

22
Q

How is rheumatoid arthritis treated?

A

DMARDs
Steroids
Biological ( in severe cases)

23
Q

Name some organ specific auto immune diseases

A

Hashimoto’s thyroiditis (Thyroid peroxidase and thyroglobulin)
Type 1 diabetes mellitus (Pancreatic islet cells)
Multiple sclerosis (Myelin sheath)
Goodpasture’s disease (Glomerular/alveolar basement membrane) Addison’s disease (Steroid-21 hydroxylase - adrenal cortex)
Graves’ disease (Thyroid-stimulating hormone receptor)
Myasthenia gravis (Acetylcholine receptor)
Pernicious anaemia (Intrinsic factor - terminal ileum)

24
Q

Name some non-organ specific autoimmune diseases

A

Autoimmune haemolytic anaemia (RBC antigens)
Rheumatoid arthritis (rheumatoid factor - Fc portion of IgG)
SLE - (dsDNA + histones)
Sjogren’s syndrome (nuclear antigens)

25
Q

What autoimmune diseases are caused by a type IV hypersensitivity reaction? What common presentation do they share?

A
Type 4 causes destruction of the tissue resulting in a loss of function 
Hashimoto’s thyroiditis 
T1DM
Multiple sclerosis 
Goodpastures disease
Addison’s disease 

RA
Sjögren’s syndrome

26
Q

What autoimmune diseases can be cause by a type II hypersensitivity reaction?

A

Graves’ disease
Myasthenia gravis
Pernicious anaemia
Autoimmune haemolytic anaemia

27
Q

What type of hypersensitivity reaction occurs in systemic lupus erythematosus?

A

Type III

28
Q

How does autoimmunity develop to autoimmune disease?

A
  1. Presence of autoantibodies/autoreactive T cells
  2. Levels of autoantibodies correlate with disease severity and activity
  3. Autoantibodies/autoreactive T cells found at the site of tissue damage
29
Q

What is meant by sensitivity?

A

The % of individuals with a condition that the test identifies

30
Q

What is meant by specificity?

A

The % of individuals who do not have condition that the test excludes

31
Q

What techniques can be used in detecting autoimmune diseases?

A
Infiltration of T cells into specific tissue 
Indirect immunofluorescence 
Immunofluorescence
Radioimmunoassay 
Coombs test (ANA)
Agglutination (RA)
32
Q

What diseases can be induced in the neonate due to autoimmune disease in pregnancy?

A
Thrombocytopenia
Haemolytic anaemia
Neonatal Graves’ disease
Neonatal myasthenia gravis 
Neonatal SLE
33
Q

What is the edrophonium test?

A

Patient is given a IV anti cholinesterase drug. This inactivated the enzyme cholinesterase that breaks down acetylcholine. If there is an improvement in the patients symptoms the most likely diagnosis would be myasthenia gravis.

34
Q

What environmental factors trigger autoimmunity?

A

Hormones
Infections
Drugs

35
Q

What is the next promising therapeutic alternative for autoimmune diseases?

A

Monoclonal antibodies

36
Q

Why is it important to test for autoantibodies in autoimmune rheumatic diseases?

A
  • aid diagnosis
  • associated with specific clinical features
  • disease prognosis
  • to stratify therapy
37
Q

What symptoms do we look out for when taking a history for autoimmune rheumatoid arthritis?

A
  • Current symptoms (Pain/Morning Stiffness/ Swelling/ Pattern of joint involvement - small joints)
  • Evolution (Acute or chronic/ Associated events /Response to treatment/Family history)
  • Involvement of other systems (Skin, eye, lung/ Malaise, weight loss, fevers, night sweats)
  • Impact on patient’s lifestyle
  • Constitutional symptoms (Fever, fatigue, weight loss, night sweats, Poor appetite)
  • “Glove and sweater” approach
38
Q

What does the glove and sweater approach mean when taking a history for autoimmune rheumatoid disease?

A

Gloves = raynauds, joint pains and swelling(synovitis), hand rash

Sweater = proximal muscle weakness > myalgia, hair loss, eye and mouth dryness, nose bleeds, mouth ulcers, pleuritic chest pain, pericardial pain, truncal rash/photosensitivity, limb weakness

39
Q

What is the lupus mneumonic?

A

A rash points medical diagnosis

ANA positive
Renal abnormalities
Arthralgia/arthritis
Serositis
Haematological abnormalities
Photosensitivity 
Oral ulcers
Immunological abnormalities
Neurological abnormalities
Malar rash
Discoid rash