Systemic Lupus Eryhtematosus

Question 1

defintion

Answer 1

autoimmune disease that can affect any part of the body

Question 2

who is SLE more common in

Answer 2

9x more common in females and in asians and afro-caribeans

Question 3

risk factors

Answer 3

UV light, smoking and infection especially EBV

Question 4

Pathophysiology

Answer 4

• due to defective immune clearance, necrotic cells release their nuclear material which is normaly cleared by the immune system
• but in SLE there is defective immune clearance so the immune system eventually believes the nuclear material are antigens (auto- antigens)
• the immune system stimulates B cells to produce autoantibodies (anti- nuclear antibodies) in response to these auto antigens
• the auto antigens and anti-nuclear antibodies then form an immune complex and migrate into blood vessels
• they eventually become lodged in a vessel wall in an organ triggering a localised inflammatory response which activates the complement system and recruits neutrophils
• the neutrophils produce enzymes which damage the endothelial basement membrane

Question 5

classical triad of general presentation

Answer 5

1. fever
2. arthralgia
3. malar butterfly rash

Question 6

Mucocutaneous features

Answer 6

• photosensitivity
• malar butterfly rash which spare the nasolabial folds
• non-scarring alopecia
• discoid lupus erythematous
• painless mouth ulcers
• sjogrens syndrome

Question 7

neurological features

Answer 7

• depression, psychosis
• seizures
• cranial or peripheral neuropathy
• mononeuritis multiplex

Question 8

serositis

Answer 8

• pleural effusion and pleurisy

- pericardial effusion and pericarditis

Question 9

msk features

Answer 9

• non-deforming arthritis which has the same distribution of rheumatoid arthritis but does not cause erosions
• the deforming arthropathy is called JACOUS arhtropathy which is ulnar deviation from 2nd to 5th fingers due to subluxation of MCP joints
• myopathy: weakness, myalgia and myositis

Question 10

haematological features

Answer 10

• lymphadenopathy (one of the earliest symptoms)
• haemolytic anaemia
• leucopenia
• lymphopenia
• thrombocytopenia

Question 11

what should you always be suspicious of in SLE

Answer 11

renal disease always do urinalysis or you’ll kill the person

Question 12

lupus nephritis

Answer 12

• most severe an deadly manifestation of lupus as it causes no symptoms until the kidneys completely fail
• causes a lupus glomerulonephritis causing proteinuria of greater than 500mg
• can also cause hypoalbunaemia, haematuria and hyperlipidaemia

Question 13

diagnosis of lupus nephritis

Answer 13

first do urinalysis and if there is protein in urine then do renal biopsy

Question 14

what syndrome is a disease on its own but is most common in people with SLE

Answer 14

anti-phospholipid syndrome

Question 15

what is anti-phospholipid syndrome

Answer 15

autoimmune disorder which causes recurrent arterial and venous thrombosis

Question 16

presentation of anti-phospholipid syndrome

Answer 16

recurrent miscarriages and recurrent unexplained thrombosis

Question 17

clinical signs of anti-phospholipdu syndrome

Answer 17

• livido reticularis

- prolonged APTT (not because deficiency in clotting factors but because the antibodies interfere with phospholipids)

Question 18

diagnosis of anti-phospholipid syndrome

Answer 18

one of three antibodies must be present on 2 occasions 12 weeks apart
- beta-2-glycoprotein
- lupus anti-coagulant
- anti-cardiolipin
the more antibodies present the higher the risk of thrombosis

Question 19

management of anti-phospholipid syndrome

Answer 19

life long warfarin but remember warfarin is teratogenic so if person wants to get pregnant you must take the off warfarin and put them on FRAGMIN which is a type of low molecular weight heparin

Question 20

diagnostic criteria

Answer 20

must meet 4 criteria with one being immunological and one being clinical

Question 21

antibodies in SLE

Answer 21

• ANA: sensitive but not at all specific
• anti-dsDNA: specific but not completely sensitive
• Anti-Ro
• Anti-Sm

Question 22

Anti-DsDNA

Answer 22

highly specific for SLE but not everyone with SLE has it, basically means that if you have it you definitely have SLE but if not is doesn’t rule it out

Question 23

anti-Ro

Answer 23

• associated with cutaneous manifestations and secondary sjogrens
• in pregnancy it causes neonatal lupus which can cause foetal heart block

Question 24

the higher the titre of antibodies

Answer 24

the more active the disease

Question 25

investigations

Answer 25

• URINALYSIS (then renal biopsy if indicated)
• antibodies
• FBS: haemolytic anaemia
• elevated ESR, PV but not CRP only elevated on background of infection in SLE, LOW COMPLEMENT LEVELS
• CXR
• ECHO
• ECG
• CT scan
• X-RAY
• MRI

Question 26

what treatment does every single person who is diagnosed with SLE get

Answer 26

HYDROXOCHLOQUINE+ NSAIDS

Question 27

what is the treatment if there is any renal involvement on renal biopsy

Answer 27

cyclosphosphamide and high dose steroids

Question 28

steroid usage in SLE

Answer 28

• small doses for cutaneous involvement and arthritis
• moderate doses for serositis and haematological abnormalities
• high doses for major haematological abnormalities, neurological involvement and renal involvement

Question 29

how long should steroids be used for

Answer 29

as little time as possible and if long term use required they should be used in combination with methotrexate or azathioprine

Question 30

last resort for SLE

Answer 30

biological agent called rituximab which targets B cells

Question 31

problems with cyclosphospahide

Answer 31

• everyone should be given prophylactic co-trimxazoel due to the risk of pneumocystis jirovecii
• long term use also increases risk of bladder cancer