SLE, Systemic manifestations and RA

Question 1

what is rheumatoid arthritis

Answer 1

Chronic joint inflammation (synovium) can result in joint damage
Associated with autoantibodies (RF and Anti-CCP)

Question 2

Which autoantibodies are associated with rheumatoid arthritis

Answer 2

Rheumatoid factor

Anti-CCP

Question 3

What is ankylosing spondylitis

Answer 3

Chronic spinal inflammation that can result in spinal fusion and deformity
Site of inflammation includes the enthesis

No autoantibodies (‘seronegative’)

Question 4

What are the consequences with ankylosing spondylitis

Answer 4

Spinal fusion

Question 5

How is spinal fusion evidently seen on a radiograph

Answer 5

Bamboo spine

Question 6

examples of seronegative spondyloarthopathies

Answer 6

Ankylosing spondylitis
Reactive arthritis (Reiter’s Syndrome)
Psoriatic arthritis
Arthritis associated with gastrointestinal inflammation (enterohepatic synovitis)

Question 7

main features of SLE

Answer 7

Chronic tissue inflammation in the presence of antibodies directed against self antigens
Multi-site inflammation but particularly the joints, skin and kidney

Question 8

key autoantibodies in SLE

Answer 8

Antinuclear antibodies
Anti-double stranded DNA antibodies
Anti-phospholipid antibodies

Question 9

arthralgia

Answer 9

Pain in joints
Tenderness but not obvious inflammation

Common in connective tissue disorders

Question 10

What is Raynaud’s phenomenon

Answer 10

Intermittent vasospasm of digits on exposure to cold
Typical colour changes – white to blue to red
Vasospasm leads to blanching of digit
Cyanosis as static venous blood deoxygenates
Reactive hyperaemia

Question 11

main manifestations of SLE

Answer 11

Malar rash – butterfly, photosensitive
Mouth ulcers
Hair loss
Raynaud’s phenomenon
Arthralgia and sometimes arthritis
Serositis (pericarditis, pleuritis, less commonly peritonitis)
Renal disease – glomerulonephritis (‘lupus nephritis’)
Cerebral disease – ‘cerebral lupus’ e.g. psychosis

Question 12

demographic of SLE

Answer 12

females 15-45

Question 13

Which rheumatoid conditions are seronegative

Answer 13

OA
Reactive arthritis
Gout
Ankylosing Spondylitis

Question 14

What type of hypersensitivity is associated with SLE

Answer 14

Type 3 hypersensitivity -due to formation of immune complexes

Question 15

three stages of Raynaud’s

Answer 15

1) Vasospasm
2) Cyanosis
3) Reactive hyperaemia

Question 16

key investigations for SLE

Answer 16

ESR
CRP is usually normal
Autoantibodies
Antiphospholipid antibodies

Question 17

haematological findings SLE

Answer 17

Haemolytic anaemia, Lymphopenia, Thrombocytopenia

Question 18

What is the main clinical risk in a neonate with neonatal lupus syndrome

Answer 18

Permanent heart block - conduct ECG

Question 19

renal investigations performed in patients with SLE

Answer 19

Measure urine protein: Creatinine ratio
Albumin
eGFR

Question 20

How can we measure disease activity in SLE

Answer 20

Immune complexes

Unwell lupus patient has LOW complement C3 and C4 levels and HIGH levels of anti-ds-DNA antibodies

Question 21

aim of SLE treatment

Answer 21

aims at remission or low disease activity and prevention of flares

Question 22

What should patients with SLE be assessed for

Answer 22

antiphospholipid antibody status

infectious and cardiovascular diseases risk profile

Question 23

What is Sjorgren’s syndrome

Answer 23

Autoimmune exocrinopathy
lymphocytic infiltration of especially exocrine glands and sometimes of other organs (extra-glandular involvement)
non-erosive arthritis and raynauds common

Question 24

What is inflammatory muscle disease

Answer 24

Proximal muscle weakness due to autoimmune-mediated inflammation either with (dermatomyositis) or without (polymyositis) a rash
associated with malignancy and pulmonary fibrosis

Question 25

skin changes in dermatomyositis

Answer 25

heliotrope rash on eyelids, malar regionm nsao-labial folds red or purple lesions on knuckles - gottrons papules subcutaneous calcinosis mechanics hands - cracking over finger pads

Question 26

Which serum markers are elevated in dermatomyositis

Answer 26

Creatine phosphokinase

Question 27

symptoms of sjorgens

Answer 27

dey eyes dry mouth parotid gland enlargement

Question 28

What is systemic sclerosis

Answer 28

Thickened skin with Raynaud’s phenomenon Dermal fibrosis, cutaneous calcinosis and telangiectasia Skin changes may be limited or diffuse

Question 29

What is overlap syndrome

Answer 29

When features of more than 1 connective tissue disorder are present e.g. SLE and inflammatory muscle disease we can use the term overlap syndrome

Question 30

investigation findings inflammatory muscle disease

Answer 30

elevated CPK abnormal electromyography abnormal muscle biopsy

Question 31

manifestations of diffuse systemic sclerosis

Answer 31

Fibrotic skin proximal to elbows or knees (excluding face and neck) Anti-topoisomerase-I (Anti-Scl-70) antibodies. Pulmonary fibrosis, renal (thrombotic microangiopathy) involvement. Short history of Raynaud’s phenomenon

Question 32

manifestations of limited systemic sclerosis

Answer 32

Fibrotic skin hands, forearms, feet, neck and face Anti-centromere antibodies Pulmonary hypertension Long history of Raynaud’s phenomenon

Question 33

Which antibodies are associated with CREST and limited systemic sclerosis

Answer 33

Anti-centromere antibodies

Question 34

CREST

Answer 34

sub-type of limited systemic sclerosis: | Calcinosis, Raynaud’s phenomenon, Oesophageal dysmotility, Sclerodactyly, Telangiectasia

Question 35

autoantibodies are associated with polymyositis

Answer 35

Anti-tRNA transferase

Question 36

auto-antibody is associated with mixed connective tissue disorder

Answer 36

Anti-U1 RNP antibody

Question 37

auto-antibody is associated with diffuse systemic sclerosis

Answer 37

Anti-Scl-70

Question 38

anti nuclear antibodies

Answer 38

seen in all SLE | not specific for SLE

Question 39

anti double stranded DNA antibodies

Answer 39

specific for SLE | levels indicate disease activity

Question 40

anti phospholipid antibodies

Answer 40

associated with risk of arterial and venous thrombosis in SLE can occur without SLE - primary antiphospholipid antibody syndrome

Question 41

investigations for synovitis

Answer 41

ultrasound - thickening, increased blood flow

Question 42

treatment of rheumatoid arthritis

Answer 42

``` steroids short term for the inflammation DMARDs - methotrexate and hydrochloriquine if DMARDs dont work: anti TNFa, anti IL-6, B cell biologic JaK inhibitors ```

Question 43

clinical signs for checking for psoriatic arthritis

Answer 43

nail signs - pitting, thickening under nail, oncholysis (separation)