Autoimmunity

Question 1

The presence of what 2 cells/ structures are responsible for driving autoimmune disease?

Answer 1

• Autoantibodies
• Autoreactive T cells

These lead to organ/ tissue fibrosis

Question 2

Define Autoimmunity and Autoimmune Disease

Answer 2

Autoimmunity;
- Immune response against the host due to the break in immunological tolerance for self-antigens

Autoimmune Disease;
- Disease caused by Tissue Damage or Disturbed Physiological Responses due to an auto-immune response

Question 3

Autoimmune diseases can be Organ Specific or Non-Organ Specific.

Compare these

Answer 3

Organ specific;
- Autoantigen is only present in 1 organ-> Organ/ tissue specific damage

Non-organ specific;
- Autoantigen is found in multiple sites-> Damage throughout body (often Type III Hypersensitivity)

Question 4

The severity of the autoimmune disease and the clinical outcome depend on the organ affected and the type of hypersensitivity reaction.

List the 2 most common autoimmune diseases in the UK

Answer 4

• Systemic Lupus Erythematosus

- Sjogren’s Syndrome

Question 5

Compare Primary and Secondary Autoantibodies

Answer 5

Primary (more rare);
- Drive the disease

Secondary;
- Do not drive the disease, occur later in the course of the disease

Question 6

List 4 secondary antibodies and their associated autoimmune disease

Answer 6

• Anti-nuclear ABs in SLE
• Anti-gastric parietal cell ABs in Pernicious Anaemia
• Anti-thyroid peroxidase ABs in Hashimoto’s
• Anti-rheumatoid factor ABs in RA

Question 7

List 4 primary antibodies and their associated autoimmune disease

Answer 7

• Anti-TSH receptor ABs in Graves’
• Anti-ACh receptor ABs in Myasthenia Gravis
• Anti-VGCC ABs in Lambert-Eaton Myasthenia Syndrome
• Anti-glomerular BM ABs in Goodpasture’s Syndrome

Question 8

Describe the transfer of autoimmune diseases from mother to child

Answer 8

Can be transferred via maternal ABs, but effect diminshes by 6 months when maternal IgE/ IgG fades

Question 9

List the diagnostic criteria for an autoimmune disease

Answer 9

• Presence of Autoantibodies/ Autoreactive T cells at site of damage
• Levels of ABs correlate with disease severity
• Transfer of cells to healthy host induces autoimmune response
• Family history present
• Clinical benefit provided by Immunomodulatory therapy

Question 10

List 2 triggers of Autoimmunity

Answer 10

• Environmental factors (hormones, infection, drugs)

- Genetic factors (8x risk with affected sibling, 30x with affected identical twin, MHC variation)

Question 11

List 4 treatment options for autoimmune disease

Answer 11

• Plasma exchange to remove Autoantibodies
• Immunosuppressive drugs to suppress Autoreactive T cells
• Anti-inflammatory drugs to treat tissue damage
• Replacement therapy surgery to treat organ dysfunction

(Recent development of Targeted Monoclonal Antibodies)

Question 12

Describe the epidemiology of Systemic Lupus Erythematosus/ SLE

Answer 12

• 9:1 female:male
• Genetic + environmental factors (UV light, Smoking)
• Race prevalence: Afro-Caribbean> South Asian> White

Question 13

List 2 aspects of taking a history for Autoimmune Rheumatic Disease (ARD)

Answer 13

• Rule out Constitutional symptoms (fever, lethargy, weight loss, poor appetite, night sweats etc)
• ‘Glove and sweater’ approach

Question 14

What 3 things do you ask about, in reference to the ‘Glove’ of the ‘Glove and Sweater’ approach?

Answer 14

• Raynaud’s
• Hand rash
• Joint pain + swelling

Question 15

What is Raynaud’s?

How does it present?

Answer 15

• An exaggerated response to cold temperature

- Changes in skin colour (E.g Triphasic: Pale/ White-> Blue-> Red)

Question 16

Suggest 8 things to you ask about, in reference to the ‘Sweater’ of the ‘Glove and Sweater’ approach?

Answer 16

• Hair loss
• Dry eyes, mouth
• Nose bleeds
• Proximal muscle weakness-> Myalgia
• Limb weakness
• Pleuritic chest pain
• Truncal rash/ Photosensitivity
• Pericardial pain

Question 17

List 3 things on the head and hands you would look for on clinical examination if suspecting an ARD

Answer 17

Head;

• Hair loss
• Facial rash
• Mouth ulcers

Hands;

• Raynaud’s
• Rash
• Synovitis

Question 18

How do we treat SLE/ Lupus?

Answer 18

• Patient education (Lifestyle changes, use sunscreen SPF 50 at least)
• DMARDs (Azathioprine, Mycophenolate Mofetil, Hydroxychloroquine)
• Steroids (Prednisolone, Methylprednisolone)
• If severe, IV Cyclophosphamide or Rituximab

Question 19

State and explain the mnemonic used to diagnose SLE/ Lupus

Answer 19

• A RASH POINts Medical Diagnosis, 4/11 definitely means Lupus

A - ANA positive  
R - Renal abnormalities 
A - Arthralgia/ arthritis 
S - Serositis (pleuritic pain)
H - Haematological abnormalities 
P - Photosensitivity (Malar rash)
O - Oral ulcers
I - Immunological abnormalities 
N - Neurological abnormalities 
M/D - Malar/ Discoid rash

Question 20

Describe the epidemiology of RA

Answer 20

• 3:1 female:male
• No race predilection
• Genetic/ environmental factors
• High incidence of CVD

Question 21

Describe the S Factor diagnosis of RA

Answer 21

• Stiffness (Early morning, lasts >30mins)
• Swelling (Persistent, 1/ more joints, especially hand)
• Squeezing (Painful to squeeze joints)

Question 22

How is RA treated?

Answer 22

• Steroid bridging therapy
• DMARDs (Methotrexate, Sulfasalazine, Hydroxychloroquine)
• If severe, Biologicals

Question 23

Briefly describe the pathogenesis of SLE

Answer 23

• After cell death, cellular remnants appear on cell surface as ‘Blebs’. These carry self antigens
• In SLE removal of the Blebs by phagocytes is inefficient, so they are transferred to lymphoid tissues and taken up by APCs.
• Self antigens are presented to T cells, which stimulate B cells to produce ABs against these antigens