SLE College Lecture

Question 1

What is the female to male ratio of prevalence?

Answer 1

9:1

Question 2

Which ethnicity is SLE more common in?

Answer 2

Afro-Caribbeans > Asians > Aborigines > Caucasians

Question 3

What is the 10 year survival rate

Answer 3

80-90%

Question 4

What is the standardized mortality ratio?

Answer 4

3

Question 5

What is the pathogenesis of SLE?

Answer 5

Genetic predisposition > known/unknown trigger > activation of immune cascade > deposition of immune complexes

Question 6

What is the genetic pattern of factors

Answer 6

Likely combination of common variations in multiple genes. Monogenetic defects are rare

Question 7

What are some single gene defects?

Answer 7

Deficiencies of complement components - eg C1q deficiency

TREX1 gene mutations

Question 8

What epigenetic mechanism is the cause of drug-induced lupus

Answer 8

DNA hypomethylations eg procainamide and hydralazine

Question 9

What % of first degree relatives have SLE?

Answer 9

4.5% (Priori 2013)

Question 10

What is the immunopathogenesis of SLE?

Answer 10

Decreased phagocytosis and increased cellular apoptosis leads to increase apoptotic materal serving as potential autoantigens > trigger inflammatory cascade

Question 11

Which receptor is activated by self nucleic acids in immune complexes?

Answer 11

Toll-like receptors

Question 12

What is abnormal about B cells in lupus?

Answer 12

Elevated levels of B cell survival factors BAFF/BLyS and APRIL

Question 13

What are some triggers of SLE/flares

Answer 13

UV light
Infection
Smoking
Environmental pollutant (silica)
Pets

Question 14

Which medications more commonly induce lupus?

Answer 14

Procainamide
Hydralazine
Minocycle 
Diltiazen
Penicillamine 
Isoniazid
TNF inhibitor induced lupus

Question 15

What is the natural history of drug induced lupus

Answer 15

Usually self resolves with redrawal of medication

Question 16

What is the relationship between oestrogen and lupus

Answer 16

More oestrogen increases the risk of lupus

eg early menarche

Question 17

What are common symptoms of SLE?

Answer 17

Fatigue
Fever
Mucocutaneous
MSK

Question 18

What are some mucocutaneous manifestations?

Answer 18

Acute
- localised - malar rash (nasolabial fold sparing)
Subacte
- Annular papulosquamous, or both - 50% associated with SLE
Chronic
- Discoid lupus most common

Other
Panniculitis
Alopecia

Question 19

What is factors increase the risk of SLE with discoid lupus?

Answer 19

Positive ANA, FBE abnormalities, generalised disease

Question 20

Is lupus alopecia reversible?

Answer 20

Yes, will regrow as disease is controlled

Question 21

Are oral ulcers painfull or painless?

Answer 21

Can be both

Question 22

What is the pattern of MSK manifestation?

Answer 22

Usually symmetrical, polyarticular, migratory arthralgias with predilection to knees, wrists, and finger joints

Question 23

What’s Rhupus?

Answer 23

RA/SLE overlap - presence of anti-CCP antibodies

Question 24

What are the cardiovascular manifestations?

Answer 24

Pericarditis
Myocardial disease
Valvular disease
CAD

Question 25

What is Libman Sacks endocarditis

Answer 25

Verrucous (wart like) sterile valvular lesions at edge of aortic, mitral or tricuspid valves

Question 26

What is the prevalence of interstitial lung disease?

Answer 26

Rare

Question 27

What are less common resp manifestations of SLE?

Answer 27

Pulmonary hypertension Vanishing lung syndrome Alveolar haemorrhage

Question 28

What are the GI manifestation of SLE?

Answer 28

Hepatitis | Ileal and colonic perforation

Question 29

What are the haematological manifestations of SLE?

Answer 29

``` Cytopenias Coombs positive haemolytic anaemia Evans syndrome Thrombocytopenia Lymphadenopathy Splenomegaly ```

Question 30

What is Evans syndrome?

Answer 30

Autoimmune haemolysis and ITP

Question 31

What is the most common neuropsychiatric manifestation?

Answer 31

Cognitive impairment

Question 32

What is the pattern of myelitis in SLE?

Answer 32

Grey matter | White matter

Question 33

What is the classification of renal disease?

Answer 33

Class I to VI

Question 34

What diagnostic criteria is used for SLE?

Answer 34

1997 ACR classification criteria - 4 manifestations 2012 SLICC-ACR classification criteria - 4 or more. 1 clinic and 1 immunologic or biopsy-proven lupus nephritis with positive ANA

Question 35

What are the autoantibodies?

Answer 35

ANA - required for lupus Dx

Question 36

Is ANA/ENA a good marker of disease activity?

Answer 36

No

Question 37

What is anti-Sm (Smith) associated with?

Answer 37

Renal and CNS disease

Question 38

What is anti-dsDNA highly associated with?

Answer 38

SLE disease activity, nephritis, and TNFi-induced SLE

Question 39

What are chemical meaures of disease activity?

Answer 39

ESR/CRP High anti-dsDNA ab Low C3/C4

Question 40

What are general Mx options

Answer 40

``` Avoid triggers - UV light, oestrogen, sulfonamide Vitamin D repletion Cardiovascular risk modification Vaccination (avoid live vaccinations) Steroid SE's Minimise steroid ```

Question 41

What are options of minor disease?

Answer 41

Hydroxychloroquine NSAIDs Steroid Methotrexate/Azathioprine

Question 42

What are options for major disease?

Answer 42

High dose steroid until remission DMARD - Azathioprine, methotrexate, leflunomide, cyclophosphamide, cyclosporine/tac, mycophenolate Rituximab

Question 43

Who should get hydroxychloroquine?

Answer 43

Any patient with active disease

Question 44

What is the mechanism of hydrochloroquine?

Answer 44

immunomodulatory?

Question 45

What are the major SE's of hydroxychloroquine?

Answer 45

Retinal toxicity - high dose, long term use, pre-existing disease

Question 46

When to screen with hydroxy?

Answer 46

Baseline the annually after five years

Question 47

What are the treatment principles for lupus nephritis?

Answer 47

Depends on histological type

Question 48

Which lupus nephritis classes require immunosuppression?

Answer 48

3 and 4 +/- 5

Question 49

How do you treat class 3 and 4 LN

Answer 49

Initially pulse IV steroids | Then either mycophenolate or cyclophosphamide induction

Question 50

What are types of mycophenolate?

Answer 50

Mycophenolate mofetil and myfortic

Question 51

When do you evaluate for response post induction for LN?

Answer 51

6 months

Question 52

What do you use for maintenance for LN 3 + 4

Answer 52

Mycophenolate or azathioprine

Question 53

What biologics are used in SLE?

Answer 53

B cell targeting therapies - Rituximab (LUNAR and EXPLORER trials) - Belimumab

Question 54

What is the target of Belimumab

Answer 54

Targets B cell survival factors BAFF/Blys

Question 55

What is the impact of lupus on pregnancy?

Answer 55

Higher faetal and maternal mortality

Question 56

How do you differentiate lupus disease from pre-escampsia?

Answer 56

anti-dsDNA Complements levels Haematuria

Question 57

Which antibodies should oyu check before pregnacny?

Answer 57

Anti-phospholipid | Anti-RO and La

Question 58

What are the implications of pos antiphospholipid ab in pregnancy?

Answer 58

Asymt - low dose aspirin Prior obstetric morbidity - low aspirin and prophylactic clexane Prior thrombosis - therapeutic clexane - cont 6 months postpartum

Question 59

What are the manifestations of anti-Ro and La antibodies?

Answer 59

Rash, haematologic, hepatic | Complete heart block

Question 60

Which drugs can you continue in pregnancy?

Answer 60

Hydroxychloroquine, AZA, steroids

Question 61

Which drugs can't you use in pregnancy?

Answer 61

Methotrexate | NSAIDs (third trimester for ductus arterosis closure)