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BPT Rheumatology > SLE College Lecture > Flashcards

SLE College Lecture Flashcards

1
Q

What is the female to male ratio of prevalence?

A

9:1

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2
Q

Which ethnicity is SLE more common in?

A

Afro-Caribbeans > Asians > Aborigines > Caucasians

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3
Q

What is the 10 year survival rate

A

80-90%

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4
Q

What is the standardized mortality ratio?

A

3

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5
Q

What is the pathogenesis of SLE?

A

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

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6
Q

What is the genetic pattern of factors

A

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

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7
Q

What are some single gene defects?

A

Deficiencies of complement components - eg C1q deficiency

TREX1 gene mutations

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8
Q

What epigenetic mechanism is the cause of drug-induced lupus

A

DNA hypomethylations eg procainamide and hydralazine

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9
Q

What % of first degree relatives have SLE?

A

4.5% (Priori 2013)

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10
Q

What is the immunopathogenesis of SLE?

A

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

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11
Q

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

A

Toll-like receptors

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12
Q

What is abnormal about B cells in lupus?

A

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

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13
Q

What are some triggers of SLE/flares

A 
UV light
Infection
Smoking
Environmental pollutant (silica)
Pets
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14
Q

Which medications more commonly induce lupus?

A 
Procainamide
Hydralazine
Minocycle 
Diltiazen
Penicillamine 
Isoniazid
TNF inhibitor induced lupus
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15
Q

What is the natural history of drug induced lupus

A

Usually self resolves with redrawal of medication

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16
Q

What is the relationship between oestrogen and lupus

A

More oestrogen increases the risk of lupus

eg early menarche

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17
Q

What are common symptoms of SLE?

A

Fatigue
Fever
Mucocutaneous
MSK

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18
Q

What are some mucocutaneous manifestations?

A

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

Other
Panniculitis
Alopecia

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19
Q

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

A

Positive ANA, FBE abnormalities, generalised disease

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20
Q

Is lupus alopecia reversible?

A

Yes, will regrow as disease is controlled

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21
Q

Are oral ulcers painfull or painless?

A

Can be both

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22
Q

What is the pattern of MSK manifestation?

A

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

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23
Q

What’s Rhupus?

A

RA/SLE overlap - presence of anti-CCP antibodies

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24
Q

What are the cardiovascular manifestations?

A

Pericarditis
Myocardial disease
Valvular disease
CAD

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25
Q

What is Libman Sacks endocarditis

A

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

26
Q

What is the prevalence of interstitial lung disease?

A

Rare

27
Q

What are less common resp manifestations of SLE?

A

Pulmonary hypertension
Vanishing lung syndrome
Alveolar haemorrhage

28
Q

What are the GI manifestation of SLE?

A

Hepatitis

Ileal and colonic perforation

29
Q

What are the haematological manifestations of SLE?

A 
Cytopenias 
Coombs positive haemolytic anaemia
Evans syndrome
Thrombocytopenia
Lymphadenopathy
Splenomegaly
30
Q

What is Evans syndrome?

A

Autoimmune haemolysis and ITP

31
Q

What is the most common neuropsychiatric manifestation?

A

Cognitive impairment

32
Q

What is the pattern of myelitis in SLE?

A

Grey matter

White matter

33
Q

What is the classification of renal disease?

A

Class I to VI

34
Q

What diagnostic criteria is used for SLE?

A

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

35
Q

What are the autoantibodies?

A

ANA - required for lupus Dx

36
Q

Is ANA/ENA a good marker of disease activity?

A

No

37
Q

What is anti-Sm (Smith) associated with?

A

Renal and CNS disease

38
Q

What is anti-dsDNA highly associated with?

A

SLE disease activity, nephritis, and TNFi-induced SLE

39
Q

What are chemical meaures of disease activity?

A

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

40
Q

What are general Mx options

A 
Avoid triggers - UV light, oestrogen, sulfonamide
Vitamin D repletion 
Cardiovascular risk modification 
Vaccination (avoid live vaccinations) 
Steroid SE's
Minimise steroid
41
Q

What are options of minor disease?

A

Hydroxychloroquine
NSAIDs
Steroid
Methotrexate/Azathioprine

42
Q

What are options for major disease?

A

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

43
Q

Who should get hydroxychloroquine?

A

Any patient with active disease

44
Q

What is the mechanism of hydrochloroquine?

A

immunomodulatory?

45
Q

What are the major SE’s of hydroxychloroquine?

A

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

46
Q

When to screen with hydroxy?

A

Baseline the annually after five years

47
Q

What are the treatment principles for lupus nephritis?

A

Depends on histological type

48
Q

Which lupus nephritis classes require immunosuppression?

A

3 and 4 +/- 5

49
Q

How do you treat class 3 and 4 LN

A

Initially pulse IV steroids

Then either mycophenolate or cyclophosphamide induction

50
Q

What are types of mycophenolate?

A

Mycophenolate mofetil and myfortic

51
Q

When do you evaluate for response post induction for LN?

A

6 months

52
Q

What do you use for maintenance for LN 3 + 4

A

Mycophenolate or azathioprine

53
Q

What biologics are used in SLE?

A

B cell targeting therapies

  • Rituximab (LUNAR and EXPLORER trials)
  • Belimumab
54
Q

What is the target of Belimumab

A

Targets B cell survival factors BAFF/Blys

55
Q

What is the impact of lupus on pregnancy?

A

Higher faetal and maternal mortality

56
Q

How do you differentiate lupus disease from pre-escampsia?

A

anti-dsDNA
Complements levels
Haematuria

57
Q

Which antibodies should oyu check before pregnacny?

A

Anti-phospholipid

Anti-RO and La

58
Q

What are the implications of pos antiphospholipid ab in pregnancy?

A

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

59
Q

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

A

Rash, haematologic, hepatic

Complete heart block

60
Q

Which drugs can you continue in pregnancy?

A

Hydroxychloroquine, AZA, steroids

61
Q

Which drugs can’t you use in pregnancy?

A

Methotrexate

NSAIDs (third trimester for ductus arterosis closure)

BPT Rheumatology (17 decks)

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