week 9

Question 1

what are innate defences and purpose

Answer 1

surface barriers
internal defences eg phagocytes, inflammation, fever
• Prevent foreign substances
from entering the body
• Immobilise and eliminate invaders

Question 2

what are adaptive defences and purpose

Answer 2

humeral immunity (B cells)
cellular immunity (T cells)
T and B lymphocytes and
protein products
• Tolerant to “self” but react against “non-self”
• Target and destroy specific substances (“antigens”)

Question 3

what do cytotoxic T cells do

Answer 3

target infected cells, but also affects cells around

Question 4

what happens in an autoimmune disease

Answer 4

adaptive defences: 
attack normal body cells
antigen cannot be cleared
sustained response
 tissue and organ damage
autoimmune disease

Question 5

define systemic disease

Answer 5

affects multiple organs/system

Question 6

define organ specific disease

Answer 6

affects one organ/tissue

Question 7

mechanisms of autoimmune disease

Answer 7

combination of genetic and evironemtnal factors

Question 8

whats genetic factors autoimmune disease

Answer 8

type of “self proteins” a person has (HLA proteins)

• Women (75% of patients)àrole for estrogen? • Ethnicity

Question 9

whats environemental factors autoimmune disease

Answer 9

Chemicals

• Viral and bacterial infections (e.g. EBV, streptococcus)

Question 10

how does breakdown of self tolerance occur

Answer 10

• failure to destroy self reactive T and B lymphocytes during development
• failure of regulatory T cells to control immune response
• antigen mimicry (foreign antigens resemble self antigens)

Question 11

how does autoimmune disease cause tissue damage 3 ways

Answer 11

mmune complex mediated inflammation
cell mediated cytotxicity
antibody reactions

Question 12

immune complex mediated inflammation tissue damage

Answer 12

immune complex mediated inflammation eg rheumatoid arthritis:

• autoantibodies are common marker of autoimmune disease eg rheumatoid factor.
• immune complexes go into the blood which attract phagocyte which realease digestive enzymes into extracellular space which leads to tissue damage

Question 13

cell mediated cytotoxicity tissue damage

Answer 13

• Cytotoxic T cells lyse target cells, e.g. insulin-producing pancreatic b cells in Type I diabetes
• Phagocytesdamage/kill cells by releasing digestive TC cell
  enzymes, e.g. rheumatoid arthritis, multiple sclerosis
• T cells release enzymes that damage target cells membranes and degrade DNA

Question 14

antibody reactions tissue damange

Answer 14

against cell surface antigens (act as agonists) eg graves disease

Question 15

whats rheumatoid arthritis

Answer 15

systemic disease with prominent joint involvement. Immune-mediated chronic inflammationàjoint
destruction, i.e. autoimmune disease
• Inflammatory arthritis involving pain, swelling and stiffness of symmetrical joints
• Heart, lungs, skin, eyes, central nervous system may be affected/damaged by inflammatory reactions

Question 16

whats idiopathic means

Answer 16

cause unknown

Question 17

how are autoimmune disease treated

Answer 17

• relieve symptoms eg anti-inflammatory drugs (NSAID)
• replace vital substances the body can no longer make eg hormone replacement (insulin)
• suppress the immune system: control disease process and preserve organ function
• dietary manipulation eg no gluten in coeliac disease

Question 18

what is JIA

Answer 18

• onset prior to 16 years old
• idiopathic
• arthritis
• immune mediated chronic inflammation (autoimmune disease)
• affects girls more than boys

Question 19

genetic components of JIA

Answer 19

Markedly increased risk for close relatives
• Females > males
• Most common in Caucasians
• Emotionalissues/stressmayworsensymptoms
• Roleforsexhormones?

Question 20

what area of immune system affected JIA

Answer 20

Elevated cytokine levels (TNF, IL-1, IL-6) and auto- reactive antibodies
• Distinct from adult RA, despite many similarities

Question 21

JIA signs and symptoms

Answer 21

• joints pain and swelling (synovial inflammation, erosion of articular cartilage)
• joint stiffness, flexion contracture: leads to restricted movement and physical activity
• sleep disturbances, fatigue
• period of remission (symptom free) and flareups

Question 22

the stages the pain cycle

Answer 22

• pain
• tense muscles
• stress
• sleep difficulty
• fear, anger, frsutration
• depression
• fatigue

Question 23

three major factors that cause pain in JIA patients

Answer 23

inflammation
damage to joints
muscle tension

Question 24

extra articular manifestations JIA

Answer 24

acute phase response
serositis
uveitis
growth failure

Question 25

whats acute phase response

Answer 25

Low-gradefever,weakness,malaise,muscle and joint aches, increased BP, lack of appetite, anaemia

Question 26

whats serositis

Answer 26

autoantibodies lead to rollin pathogenesis via immune complex mediated inflammation leads to damage to: - pericardium= impaired function. pain - lung pleura= impaired fonction, pain - blood vessels= schema, necrosis in skin, heart and nerves - nerve pain (tingling or burning in hands and feet

Question 27

whats uveitis

Answer 27

inflammation of the urea membrane Easy to treat but hard to know if they have it - long term uveitis = permanent eye damage, blindness

Question 28

whats growth faliue

Answer 28

- overall rate of growth reduced - uneven limb/joint growth - low bone mineral density

Question 29

three major types of JIA

Answer 29

oligoarticular polyarticular systemic

Question 30

whats oligoartuclar JIA

Answer 30

- less than 5 joints - medium-large joints affected eg knees, ankle - asymmetric - non destructive arthritis - 20% uveitis

Question 31

what is polyarticular JIA

Answer 31

- more than 5 joints affected - usually smaller joints - symmetric - destructive arthritis - usually RF positive

Question 32

what is systemic JIA

Answer 32

- more than one joint - any join - symetric - destructive arthrisic - daily fever, rash

Question 33

what is enthesitis associated JIA

Answer 33

- typically knee, ankle - ethestis: inflation of tendons at muscle insertion ponts - increase risk of unites - more communion males

Question 34

whats psoriatic JIA

Answer 34

- any joint small and large - increase risk of uveitis - nail pitting and loss

Question 35

whats undifferentiated JIA

Answer 35

does not fit into any category or fit into 2 or more categories

Question 36

diagnosis of JIA

Answer 36

usually by exclusion of other paediatric disorders | • Involving blood tests, imaging studies, clinical presentation

Question 37

three goals for treatment of JIA

Answer 37

Control pain and prevent joint damage • Maintain normal growth, joint function and muscle strength • Control systemic complications

Question 38

pharmacological treatment of JIA

Answer 38

non steroidal anti-inflammatory drugs (NSAID): eg nurophen, aspirin. (1ST LINE) - reduce joint pain and swelling but DOES NOT prevent joint destruction or inhibit disease progression corticosteroids: reduce pain, inflammation and can prevent joint inflammation. (1st LINE) - short term treatment only disease modifying antirhumatic drugs (DMARDS): (2ND LINE) - long term use - imunosupressent, reduces inflammation, progressive joint destruction Biologics (when 1st and 2 nd line fail) - immune suppressants

Question 39

side affects of corticserotied treatment

Answer 39

immunosupression= increased risk of infetion grwoth retardation decreased bone mineral density weight gain

Question 40

non pharmacological treamtoer

Answer 40

- exercises, stretching and strengthening - restnng and functional splints PAIN MANAGMENT: - breathing and relaxation techniques - massage - heat cold application NUTRITION: - vitamin D and calcium supplements (bones) - Omega 3 (may help reduce inflammation/pain) SURGERY

Question 41

three types of surgery

Answer 41

Osteotomy – removing or inserting a wedge of bone to allow more normal joint alignment • Epiphysiodesis – removal/impairment of the growth plate to stop bone growth • Joint replacement – not usually done until growth has stopped

Question 42

prognosis for JIA

Answer 42

``` Oligoarticular 50% at 10 y post-diagnosis Polyarticular (RF-) 25% at 10 y Polyarticular (RF+) <10% at 10 y Systemic 10% if single episode (90% will have relapsing or chronic disease) Poor prognosis associated with: • Early hip or wrist involvement • Symmetrical disease, rheumatoid factor (i.e. like adult RA) • Persistent inflammation (> 5-10 years) and/or prolonged systemic disease ```

Question 43

side effects of non steroidal antjnflamtory drugs (NSAID)

Answer 43

Generally well tolerated in children • < 1% experience mild nauseaàloss of appetite, but minimised by taking with meals • Bruising more common

Question 44

side effects of disease modifying antirhumatic drugs

Answer 44

mmunosuppressantàincreased risk of infection • Generally well tolerated in children but ... • ~10% experience nausea and abdominal discomfort • ~ 3% liver damageàregular blood tests (no alcohol) • Teratogenàbirth defects

Question 45

side effects of biologics

Answer 45

Increased frequency and severity of infections e.g. UTI, pneumonia, tuberculosis, fungal infections • Patients cannot have any live vaccines, e.g. polio, chicken pox, MMR

Question 46

causes of fatigue in JIA

Answer 46

disease activity pain (affects sleep) muscle weakness/ wasting depression

Question 47

why is exercise good for JIA

Answer 47

Improve mobility and flexibility of joints, muscle strength, posture and balance Decrease pain, fatigue (tiredness), muscle tension and stress

Question 48

should clients exercise a painful or inflamed joint

Answer 48

No. You should stop exercising if it is causing you unusual pain or increases your pain beyond what is normal for you. Exercising through this type of pain may lead to injury or worsening of your arthritis symptoms.