SLE

Question 1

What proportion of females compared to males get SLE?

Answer 1

9:1

Question 2

What is the incidence of SLE?

Answer 2

1-25 per 100,000

Question 3

What is the 10 year survival rate of SLE?

Answer 3

80-90%

Question 4

Which single gene mutations cause SLE?

Answer 4

C1q, C4A and B, C2, TREX1

Question 5

What diseases are TREX1 gene mutations also associated with?

Answer 5

Aicardi-Gouteieres syndrome (early onset encephalopathy), RVCL (retinal vasculopathy with cerebral leukodystrophy)

Question 6

Which types of genes are often involved in SLE?

Answer 6

MHC class, IFN related pathway genes, T/B cell signalling/survival, immune complex clearance

Question 7

What are the different types of epigenetic changes in SLE?

Answer 7

hypomethylation of DNA, aberrant expression of miRNAs in peripheral blood cells, hypo or hyperacetylation in pro inflammatory genes

Question 8

What percentage of patients with SLE have a first degree relative with an autoimmune disease?

Answer 8

22.7%

Question 9

What environmental factors are involved in SLE?

Answer 9

UV light, infection, smoking, silica, pets with lupus

Question 10

Which drugs can cause drug induced lupus?

Answer 10

procainamide, hydralazine, minocycline, diltiazem, penicillamine, isoniazid, qunidine, IFN-alpha, methyldopa, chlorpromazine, TNF inhibitors

Question 11

What are the lupus specific mucocutaneous manifestations of lupus?

Answer 11

acute malar rash, generalised diffuse erythema, chronic discoid

Question 12

What is the pattern of arthralgia in SLE?

Answer 12

symmetrical, polyarticular, migratory often affecting knees, wrists, fingers

Question 13

What are some of the differences between arthritis in SLE vs RA?

Answer 13

synovial effusions uncommon, morning stiffness less prominent, non deforming, non erosive

Question 14

What is Jaccoud’s arthropathy?

Answer 14

tenosynovitis, RA-like swan neck deformity and ulnar deviation

Question 15

What are other musculoskeletal symptoms of SLE?

Answer 15

mylagias, myositis, fibromyalgia, avascular necrosis, tenosynovitis, tendon rupture, subcutaneous nodules

Question 16

What are the cardiovascular manifestations of SLE?

Answer 16

pericarditis, tachycardia, CCF, arrhythmias, cardiomegaly, valvular disease, coronary artery disease

Question 17

What is libman sacks endocarditis?

Answer 17

verrucous sterile valvular lesions

Question 18

What are the pulmonary manifestations of SLE?

Answer 18

pleurisy, pleural effusions, interstitial lung disease, acute lupus pneumonitis, pulmonary hypertension, vanishing lung syndrome, alveolar haemorrhage

Question 19

What is vanishing lung syndrome?

Answer 19

progressive dyspnoea, elevated hemidiaphragm, reduced lung volumes, restrictive lung defect

Question 20

What are the gastrointestinal manifestations of SLE?

Answer 20

abnormal LFTs, ileal and colonic perforation, intestinal pseudo-obstruction, regional enteritis, ascites, hepatitis, acute pancreatitis

Question 21

What are the haematological manifestations of SLE?

Answer 21

anemia, leucopaenia, thrombocytopaenia, lympahedenopathy, splenomegaly

Question 22

What are the central neuropsychiatric manifestations of SLE?

Answer 22

Aseptic meningitis
Cerebrovascular disease (5-18%)
Demyelinating syndrome
Headache (24-72%)
Movement disorder
Seizure disorder
Myelopathy
Acute confusionalstate
Anxiety disorder
Cognitive dysfunction (55-80%)
Mood disorder (14-57%)
Psychosis (~5%)

Question 23

What are the peripheral neuropsychiatric manifestations of SLE?

Answer 23

GuillainBarresyndrome
Autonomic neuropathy
Mononeuropathy
Myasthenia gravis
Cranial neuropathy
Plexopathy
Polyuneuropathy<1%

Question 24

How is SLE renal disease classified?

Answer 24

Class I-VI based on histopathology

Question 25

What is the probability of SLE if ANA is negative?

Answer 25

< 0.14%

Question 26

Does ANA correlate with disease activity?

Answer 26

No

Question 27

What other rheumatological conditions may have positive ANA?

Answer 27

scleroderma, MCTD, PM/DM, RA, Sjogren's

Question 28

What non rheumatic autoimmune conditions may have positive ANA?

Answer 28

hashimoto's, grave's disease, autoimmune hepatitis, primary biliary cirrhosis, primary autoimmune cholangitis

Question 29

Which infections may have positive ANA?

Answer 29

mononucleosis, hepatitis C, subacute bacterial endocarditis, TB, HIV

Question 30

Which autoantibodies are associated with neonatal lupus?

Answer 30

Ro, La

Question 31

What is anti dsDNA associated with?

Answer 31

disease activity, nephritis, TNF induced SLE

Question 32

What are measures of disease activity in lupus?

Answer 32

ESR > CRP, increase anti ds DNA Ab, low C3/C4

Question 33

What general measures should be implemented in managing all patients with SLE?

Answer 33

avoid triggers (stress, UV light, oestrogen, sulfonamides), vitamin D repletion, CV risk modification, immunisations, manage glucocorticoid side effects, minimise steroid use

Question 34

What is the treatment for minor disease SLE?

Answer 34

hydroxychloroquine, NSAIDs, steroids, occasionally

Question 35

What is the treatment for major disease SLE?

Answer 35

steroids, immunosupressive agents (azathioprine, methotrexate, leflunomide, cyclophosphamide, cyclosporine, tacrolimus, mycophenolate), rituximab

Question 36

What are the benefits of hydroxychloroquine in SLE?

Answer 36

decreased flares, decreased organ damage, improves survival, improves mycophenolate response in lupus nephritis, anti lipidaemic, anti thrombotic

Question 37

What is the main side effect of hydroxychloroquine?

Answer 37

retinal toxicity

Question 38

What is the treatment for lupus nephritis?

Answer 38

``` Class I, II, VI - no immunosuppression Class III, IV, V - immunosuppression ACE inhibitor if proteinuria > 0.5g/day target BP < 130/80 statins if LDL > 2.6 plasma exchange for thrombotic microangiopathy ```

Question 39

What type of immunosuppression is used for lupus nephritis?

Answer 39

IV pulse steroids and MMF or CYC

Question 40

What are the obstetric complications of SLE?

Answer 40

pre-term delivery, pre eclampsia, eclampsia, thrombosis, infection, PPH, C-section, thrombocytopaenia

Question 41

What are the predictors of obstetric complications of SLE?

Answer 41

active disease, prior lupus nephritis, APL Abs, thrombocytopaenia, primagravida

Question 42

What are the 'contraindications' to pregnancy in patients with SLE?

Answer 42

severe pulmonary hypertension, severe restrictive lung disease, advanced renal insufficiency, advanced heart failure, previous severe pre eclampsia, stroke within previous 6 months, flare within previous 6 months

Question 43

How should positive anti phospholipid antibodies be treated in pregnancy?

Answer 43

if asymptomatic just low dose aspirin if previous obstetric issues - aspirin and prophylactic clexane if previous thrombosis - therapeutic clexane

Question 44

What is the most serious complication of nenonatal lupus?

Answer 44

complete heart block