1. Autoimmunity Quiz Emily Matthews (e.k.matthews@warwick.ac.uk)e.k.matthews@warwick.ac.uk Chris Lancaster (c.j.lancaster@warwick.ac.uk)c.j.lancaster@warwick.ac.uk Jake Turner (jake.turner@warwick.ac.uk)jake.turner@warwick.ac.uk

2. Question 1 Define autoimmunity ( 4 marks) 1) WHAT? An immune response to self antigens 2) WHY? Due to a failure of immunological tolerance 3) IT CAUSES? Leading to immune-mediated damage to specific tissues 4) CAUSED BY? Usually due to a combination of genetic & environmental factors

3. Question 2 What is tolerance? (1 mark) Name TWO types of tolerance (think: location) (2 marks) Give TWO mechanisms of central tolerance (2 marks) Unresponsiveness of the immune system to an antigen Central Peripheral Clonal deletion (by apoptosis) Clonal anergy (inactivated by regulatory T lymphocytes)

4. Question 3 Environmental causes of autoimmune conditions: Explain how the following could lead to autoimmune disease: (a)Streptococcal infection → Rheumatic fever (2 marks) (b)Trauma to eye (2 marks) (b) Trauma causes antigens to be revealed which are normally sequestered from immune system. Body has NOT developed tolerance to these self-antigens, so autoantibodies react (a)Cross-reaction of antibodies against streptococci with myocardial antigen/heart valve antigen causing heart valve damage. An example of molecular/antigen mimicry

5. Question 4a Name the following signs of autoimmune conditions A B C 1 mark 3 marks i ii iii

6. Autoimmunity Quiz Question 4b Identify the likely autoimmune condition from the sign A B C 1 mark

7. Autoimmunity Quiz Question 4c Identify an autoantibody raised in this condition A B C 1 mark

8. Question 4 : Answers A B C Sign: Exopthalmos Disease: Grave’s Disease AA: TSHR autoantibodies Sign: Butterfly rash (note nasolabial groove sparing) Disease: Systemic Lupus Erythematosus AA: ANA e.g. anti-dsDNA, anti-cardiolipin Signs: (i) Boutonnière deformity (ii) Ulnar deviation of MCP (iii) Swan-neck deformity Disease: Rheumatoid arthritis AA: RhF, anti-CCP

9. A ‘fun’ list Autoimmune condition Rheumatoid arthritis Systemic Lupus Erythematosus Sjögren’s / Sicca syndrome Myasthenia Gravis Grave’s Disease Hashimoto’s thyroiditis Coeliac Disease Elevated antibodies RhF (rheumatoid factor), anti-CCP (anti cyclic citrullinated protein) ANA (anti-nuclear antibody) e.g. anti-dsDNA (anti double-stranded DNA), anti-cardiolipin RhF, ANA e.g. anti-Ro, anti-La nAChR (nicotinic acetylcholine receptor) antibodies TSHR (thyroid stimulating hormone recepetor) antibodies Anti-thyroid perioxidase Antigliadin N.B. I’ve used acronyms: remember in an exam WRITE EVERYTHING OUT IN FULL!

10. Question 5 Describe the joint position of a finger in: (a)Swan neck deformity (b) Boutonnière deformity

11. Question 6a A patient with Rheumatoid arthritis presents with: -Dry mouth -Scratchy, dry eyes -Rashes Sjögren’s Syndrome / Sicca Syndrome Bonus Marks List TWO diagnostic tests Schirmer’s test Slit lamp examination Saliva flow test Autoantibodies (anti-La = most specific) Ultrasound of salivary glands ( hypoechoic lesions & loss of tissue) Biopsy of lip or salivary gland (lymphocytic infiltration tissue damage & loss) Which secondary autoimmune condition should be suspected? Xerostomia Xerophthalmia N.B. Sjorgren’s / Sicca can also present on its own, i.e. as a primary condition N.B. Be able to describe these (just a sentence) Medical terms

12. Autoimmunity Quiz Question 6b Sicca / Sjögren’s Syndrome : State THREE treatments/management CONSERVATIVE MEDICAL SURGICAL Artificial tears, regular sips of fluid, artificial saliva, (awareness of lymphoma risk) DMDs: Topical cyclosporin Lacrimal plugs

13. Autoimmunity Quiz Question 7 Grave’s Disease: State THREE treatments/management CONSERVATIVE MEDICAL SURGICAL N/A 1.Thioamides: Carbimazole or propylthiouracil (inhibt thyroid peroxidase enzyme required to convert iodine → thyroid hormone) 2.Radioiodine Partial or total thyroidectomy

14. Autoimmunity Quiz Clarifications Exopthalmos = “appearance of protruding eyes” caused by proptosis (eye protrudes beyond orbit) and lid retraction Thyrotoxicosis = excess thyroid hormone Usually due to hyperthyroidism Most common cause is Grave’s disease