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Rheumatology > SLE > Flashcards

SLE Flashcards

1
Q

Describe the typical patient with SLE

A

A woman of child bearing age 90%

  • highest prevalence is in women of African-American and Afro-caribbean origin.
  • lowest prevalence is in white men
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2
Q

Describe the basic pathogenesis of SLE

A

Gene-environment interactions result in abnormal immune responses that generate pathogenic autoantibodies and immune complexes* that deposit in tissue, activate complement, cause inflammation and over time lead to irreversible organ damage.
- abnormal immune responses vary between individuals

    • patients often have low C3/C4 - early complements are important for immune complex clearance
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3
Q

What are environmental risk factors for SLE

A

Smoking
EBV infection
Sunlight exposure
Silica exposure (most important in African Americans)

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4
Q

What are musculoskeletal manifestations of SLE

A

Symmetrical migratory polyarthirits, usually not erosive
Myositis
Ischaemic bone necrosis
Arthralgias/myalgia

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5
Q

What are skin manifestations of SLE

A

Photosensitive malar rash (butterfly distribution) and photosensitive rash in other sun exposed areas
Discoid lupus erythematosus
Alopecia
Oral ulcers
Subacute cutaneous lupus erythematous (looks a bit like a fungal rash)

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6
Q

What are the renal manifestations of lupus

A 
Minimal mesangial lupus nephritis
Mesangial proliferative lupus nephritis
Focal lupus nephritis
Diffuse lupus nephritis
Membranous lupus nephritis 
Advanced sclerotic lupus nephritis
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7
Q

What are the neurological manifestations of lupus

A 
Cognitive dysfunction 
Headache (usually bad during flares)
Mood disturbance and psychosis
Myelopathy
Mono/poly neuropathy
Seizures
Stroke/Tia (need to determine whether from vasculitis or atherosclerosis)
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8
Q

What are pulmonary manifestations of SLE?

A 
Pleuritis with or without pleural effusion
Interstitial fibrosis
Pulmonary hypertension
Pulmonary haemorrage
Shrinking lung syndrome
Lupus pneumonitis
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9
Q

What are the cardiac manifestations of SLE

A

Pericarditis
Myocarditis
Libman-Sacks endocarditis (fibrinous/sterile infective endocarditis)
Accelerated atherosclerosis with increased rates of MI at relatively young age

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10
Q

What are haematologic manifestations of SLE

A 
Anaemia of chronic disease
Haemolytic anaemia - positive Coombe's test
Leukopenia (predominantly lymphopenia)
Thrombocytopenia 
- ITP
- TTP ** - if red cell fragments and thrombocytopenia
Lymphadenopathy 
Splenectomy
Anti-phospholipid syndrome
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11
Q

What are the GI manifestations of SLE

A 
Nausea
Vomitting
Diarrhea
Raised LFTs
Intestinal vasculitis
Autoimmune peritonitis
Lupus enteritis (more common in Asians, thickened bowel loops)
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12
Q

What are ocular manifestations of SLE

A 
Sicca syndrome 
Retinal vasculitis
Optic neuritis 
Anterior uveitis
Episcleritis
Scleritis
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13
Q

What autoantibodies and markers can be used to monitor disease severity in SLE

A

Anti-Ds DNA
Complement (C3/C4) - low in flares

ANA does not correspond to disease severity

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14
Q

What autoantibodies are important to screen in women of childbearing age with SLE and why

A

Anti-Ro - higher rates of neonatal lupus with congenital heart block (need fetal hr monitoring regularly)
Anti-phospholipid antibodies - high rates of thrombosis and fetal loss

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15
Q

What is the best screening test for lupus?

A

ANA - 98% of those with SLE have positive but less specific (5-15% of normal population are also positive)
- probability of SLE with a neg ANA is

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16
Q

What are the immunological features of SLE?

A 
ANA (sensitive)
Anti-dsDNA (specific)
Anti-Sm (specific, less sensitive)
-  more common in African Americans and renal lupus
Anti-Ro and anti-La (SSA and SSB)
- also associated with Sjogrens
- associated with congenital lupus and heart block
Low Complement
- C3, C4, CH50
Direct Coombe's test positive (not necessarily haemolytic anaemia)
Anti-RNP
- 30% SLE
- alone - suggestive of MCTD
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17
Q

What is the recommended treatment for mild lupus

A

Hydroxychloroquine (for all) plus either NSAIDs or low dose steroids
- sometimes MTX, Leflunomide used if bad joint symptoms

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18
Q

What is the recommended treatment for severe lupus?

A

Induction with pulse Methylpred and cyclophosphamide or mycophenolate
Followed by maintainance tapering steroids and mycophenolate or azathioprine

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19
Q

Treatment for moderate SLE (significant symptoms but not organ threatening)

A

Hydroxychloroquine and steroids (higher dose then in mild)

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20
Q

Non-pharmacological treatment of SLE

A 
Vaccinations
- flu, Pneumococcal, HZV, Gardazil
Sun protection
Nutrition
Exercise
Smoking
Treat co-morbid conditions (CVS, pul HTN, osteoporosis, anti phospholipid)
Reproductive considerations

Also important to consider bone protection

21
Q

What drugs should be avoided in SLE

A

Sulphonamide antibiotics and high dose estrogen as can exacerbate flares

22
Q

What are some other therapies that can be considered in severe SLE with failure of primary treatment?

A

Belimumab

  • human mAB against soluble B lymphocyte stimulator (BLyS).
  • BLyS is a critical factor in the regulation of B cell survival and differentiation
  • works best in those with dsDNA or low C3/C4

Rituximab - anti CD20, only in refractory cases but ??? does it work
Cyclosporin

23
Q

What are poor prognostic factors in SLE

A 
Renal dysfunction 
Hypertension
Male
Young or old age at presentation
Poor socioeconomic status
African American
Overall high disease activity
Presence of anti-phospholipid antibodies
24
Q

What is the incidence of SLE?

A

In Caucasian population ~ 1/2500 but increased in African Americans, Indians and Aboriginal populations

25
Q

What is the key inflammatory mediator in SLE?

A

IFN alpha

26
Q

What are the most recent criteria for the diagnosis of SLE?

A
  • 4+ criteria of which 1 must be clinical and 1 must be immunological
  • biopsy proven nephritis with positive ANA or dsDNA
27
Q

What are the clinical criteria for SLE?

A 
Acute or subacute cutaneous lupus
• Chronic cutaneous lupus
• Oral/nasal ulcers
• Non scarring alopecia
• Inflammatory synovitis
• Serositis
• Renal: proteinuria 500mg/24hr or red cell casts
• Neurologic: seizures, mono neuritis multiplex, myelitis, peripheral or cranial neuropathy, cerebritis (acute confusion)
• Haemolytic anaemia
• Leucopenia
28
Q

What are the different types of cutaneous lupus?

A 
Acute cutaneous lupus
- malar rash, psoriaform
- associated with anti-Ro (SSA)
- heals well
Chronic cutaneous rash
- discoid rash
- scars
29
Q

What is Jaccoud’s arthritis?

A

deformities as you would see with RA but absence of erosions on x-ray
- 4% of SLE

30
Q

What is the definition of renal lupus?

A

renal biopsy suggestive
positive ANA
proteinuria > 0.5g/24 hours
red cell casts

31
Q

What are the WHO grades of lupus nephritis?

A 
I - minimal mesangial
II - Mesangial
III - focal proliferative
IV - diffuse proliferative
V - Membranous
V1 - advancing sclerosis

caused by sub endothelial immune complexes
- without treatment 5 year survival is ~ 20%

32
Q

What is the treatment for lupus nephritis?

A

Induction

  • pulse steroids
  • MMF (preferred in African Americans and Hispanics) or Cyclophosphamide

Maintenance

  • Prednisone
  • AZA or MMF
33
Q

What are the contraindications to pregnancy in SLE?

A
  • Active disease: Diffuse proliferative GN
  • Nephrotic syndrome
  • Moderate/severe HTN
  • Creatinine>140umol/L
  • Poor cardiopulmonary reserve

Pre-pregnancy counselling important
Pregnancy can flare SLE

34
Q

What are some pregnancy related complications of SLE?

A 
Pre-eclampsia
Fetal loss
Pre-term delivery
Congenital heart block (anti-Ro/La)
Congenital Lupus (anti Ro/La)
Increased rates of maternal death

Must check antiphospholipid Abs pre-conception even if no suggestion of Antiphospholipis syndrome

35
Q

What are favourable features for pregnancy in SLE?

A
  • Inactive disease for at least 6 months
  • Serum cr60ml/min
  • Proteinuria
36
Q

What is the female: male ratio for SLE?

A

10:1

37
Q

What are some genetic associations with SLE?

A

increased risk with FH of autoimmunity
~4% assoc. with FH of SLE

90% of patients homozygous for C1q deficiency develop SLE

38
Q

What are the 5 most common symptoms/signs of SLE?

A 
Arthralgia
Constitutional symptoms
Skin manifestations
Renal
Raynauds
39
Q

What are the 3 important features when assessing a renal biopsy for SLE?

A

WHO class
activity score
chronicity score

40
Q

What defines mild/mod/severe SLE?

A

Mild - athralgias, arthritis, fatigue, rash

Mod - pleuritis, pericarditis

Severe - lupus nephritis, CNS lupus

41
Q

What is the leading cause of death in SLE?

A

Cardiovascular disease

  • manage RFs
  • statins, ACE-i, BP management
  • minimise steroids
  • Hydroxychloroquine lowers cholesterol
42
Q

What are the proposed mechanisms behind the pathogenesis of anti-phospholipid syndrome?

A
  • antibodies to beta2 glycoprotein 1 form leading to activation of cell endothelial cell surface markers which induces inflammation and a pro-thrombotic phenotype
  • Production of antibodies against coagulation factors, including prothrombin, protein C, protein S, and annexin
  • Activation of platelets to enhance endothelial adherence
  • Reaction of antibodies to oxidized low-density lipoprotein, thus predisposing to atherosclerosis and myocardial infarction

can be primary form or secondary form - most commonly associated with SLE

43
Q

What are the Modified Sapporo Criteria for Anti-phospholipid syndrome?

A
  1. vascular thrombosis (arterial or venous)
  2. Pregnancy morbidity
    - 3 + consecutive miscarriages at 10/40 (placental damage)
    - placental insufficiency or pre-eclampsia with delivery 12 weeks apart
    - lupus anticoagulant on 2 occasionna > 12 weeks apart

Pregnancy complications are due to aPL-induced inhibition of trophoblast differentiation

44
Q

Why is it of utmost importance to bridge with Clexane on initiating Warfarin therapy in anti-phospholipid syndrome?

A

To prevent Warfarin induced skin necrosis

45
Q

What is the management of anti-phospholipid syndrome?

A

Secondary thromboprophylaxis
- Warfarin with bridging Clexane

In pregnancy

  • low dose Aspirin + Clexane
  • improves conception as well as reducing pregnancy related complications
46
Q

What tests should be performed if you suspect APL?

A

Anti-cardiolipin antibodies (IgG, IgM)
Anti–beta-2 glycoprotein I antibodies (IgG, IgM)
lupus anticoagulant
APTT
Serologic test for syphilis (false-positive result)
CBC count (thrombocytopenia, hemolytic anemia)
Dilute Russel Viper Venom Time (dRVVT)

47
Q

What is the Dilute Russell Viper Venom Time and why is it useful in APL syndrome?

A

In vivo antiphospholipid Abs promote clotting, in vitro they inhibit clot and therefore prolong the APTT

  • this principle is utilised in the dRVVT test to distinguish between an inhibitor and a factor deficiency
  • venom activates clotting factors which are blocked by APL Abs

mixing test is performed with patients plasma and normal plasma:

  • patient plasma with APL - prolonged APTT
  • Russell Viper venom added - delayed coagulation
  • normal serum added - delayed coagulation (not factor deficiency as this would correct at this point)
  • phospholipid added to supersaturate the APL Abs -> coagulation and APTT corrects

the ratio of the two times are calculated
a ratio > 1.2 suggests the presence of anti-phospholipid antibodies

48
Q

What test is used to monitor heparin in APL syndrome?

A

Factor 10a levels
- cannot used APTT as APL Abs prolong the APTT in most tests

  • there are sensitive APTT tests

Rheumatology (11 decks)

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