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Stage 2: Inflammation > SLE > Flashcards

SLE Flashcards

1
Q

What areas are affected in mild SLE?

A
  • Mucocutaneous

- Musculoskeletal

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2
Q

What conditions occur in moderate SLE?

A
  • Serositis
  • Cardiopulmonary
  • Vasculitis
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3
Q

What conditions occur in severe SLE?

A
  • Nephritis
  • CNS lupus
  • Thrombosis
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4
Q

What classification criteria is used to classify lupus and how is it used?

A

ACE/EULAR

  • All patients must have ANA ≥ 1:80
  • Must have ≥ 10 points to be classified as SLE
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5
Q

What are blood test features of SLE?

A
  • High ESR
  • Normal CRP
  • Cytopenia (leucopenia)
  • Thrombocytopenia
  • Low complement C3/C4
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6
Q

What autoantibodies are found in lupus?

A

1) Antinuclear antibodies (ANA) - almost everyone with lupus has this but not specific
2) Anti-dsDNA antibodies -specific (50-70%)
3) Anti-histone
4) RF?, anti-cardiolipin (aPL)

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7
Q

What antibodies to ENAs are found in lupus?

A

1) Anti-Ro (anti-SSA) - photosensitivity
2) Anti-La (anti-SSB) - neonatal lupus
3) Anti-Sm - Raynaud’s
4) Anti-RNP

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8
Q

What causes SLE?

A
  • Type III hypersensitivity (immune complexes)
  • Complexes of DNA and anti-DNA antibodies become localised in the skin and cause inflammation and rashes and also in other organs e.g. kidneys
  • Immune complexes deposit in capillaries in the skin which recruit complement and inflammatory cells e.g butterfly rash bc capillaries are very close to the face
  • Abnormal apoptosis exposes nuclear antigens to the immune system which leads to a range of antibodies being exposed
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9
Q

What are environmental factors that can trigger onset/flare ups of lupus?

A

1) Sunlight
2) Drug
3) EBV in children
4) NETosis (defective apoptosis) leading to autoantigens (and therefore ANAs)
- Something causes the switch from benign to pathogenic autoimmunity

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10
Q

What is the main cytokine in lupus?

A

Type 1 interferon

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11
Q

What is the pathogenesis of SLE?

A
  • Autoantibody producing plasma B cells produce autoantibodies
  • This forms immune complexes with the antigens aided by complement
  • The complement/immune complexes bind to tissue
  • This causes vascular inflammation and lupus nephritis
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12
Q

What are factors implicated in SLE (cause unknown)?

A
  • Female hormones
  • Genetics
  • Drugs
  • Environmental factors
  • Ethnic origin (common in those of African and Asian ancestry)
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13
Q

Describe the link between female gender and SLE

A
  • SLE is 9-10x more common in women
  • Disease onset often after menarche
  • There is a decline in disease activity with menopause (often can stop treatment)
  • Maybe something to do with X chromosomes and double genetic load
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14
Q

Describe the genetics of SLE

A
  • Complex genetics
  • Increased risk with sibling or twin
  • Key genetic marker is HLA region
  • Polymorphism in FCGR2B gene blocks the inhibitory function of this gene
  • This leads to B cell activation which protects against severe malaria but gives an increased risk of SLE
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15
Q

What is the peak age of onset of SLE?

A

15-45 years (peaks in third/fourth decade)

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16
Q

Describe the typical presentation of a patient with SLE (different from RA)

A
  • Young woman
  • Fatigue
  • Systemic - malaise, weight loss, fever, night sweats (similar to lymphoma)
  • Sun sensitive rashes
  • Arthritis/aches and pains
  • Mouth ulcers
  • Hair loss
  • Blood clot in leg (?)
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17
Q

What are the clinical features of discoid lupus erythematosus?

A
  • Demarcated, scaly, atrophic, discoid plaques

- Scarring alopecia - can be red and inflamed (acute) or more white and scarred

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18
Q

Describe the rash in subacute cutaneous lupus (SCLE)

A

Red smooth dots, overlapping, patchy, going up neck and face, hands and wrists

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19
Q

Describe chilblain lupus

A
  • Cold sensitive rash (patients nearly always have Raynaud’s as well)
  • Looks like vasculitis but lesions feel cold to the touch rather than hot
  • Red, swollen fingers, toes and hands
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20
Q

Describe Jaccoud’s/lupus arthritis (arthropathy)

A
  • Non-erosive
  • Due to tendon problems (tendonitis) rather than erosion of bone
  • The pattern of arthritis in lupus is similar to the pattern of arthritis in RA
  • Persistent, inflammatory symmetrical small joint arthritis (PISA)
  • First thing RA then lupus if young woman
  • See the same deformities as in RA e.g. swan neck
  • However, it is reversible and patients have full hand function
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21
Q

What are the types of lupus vasculitis?

A

1) Cutaneous vasculitis
2) Small vessel vasculitis
3) Digital vasculitis (symmetrical) - ears, elbows, knees, feet
4) Medium/large vessel vasculitis (less common)
5) Digital ischaemia/gangrene (uncommon)

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22
Q

What are the clinical features of small vessel vasculitis?

A
  • Splinter haemorrhages
  • Digital infarcts
  • Palpable purpura
  • Vasculitic ulcers
  • Urticarial vasculitis
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23
Q

What are the predictors of poor outcome/mortality in lupus?

A
  • Female
  • Black ethnicity
  • Infections
  • Younger age
  • Disease duration < 1 year
  • Renal disease
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24
Q

What is the SMR of lupus?

A

3

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25
Q

What cancer are lupus patients at an increased risk of (others generally lower risk)?

A

Lymphoma

26
Q

What would be used to see diffuse multi-vessel coronary artery disease?

A

Angiogram (can see blocked arteries?)

27
Q

Why does lupus atherosclerosis occur?

A
  • Vascular damage from inflammation in lupus and cytokines

- This leads to accelerated atherosclerosis

28
Q

What are lupus patients at increased risk of?

A

CVD/atherosclerosis

29
Q

What can peripheral vascular disease in SLE lead to?

A
  • Critical limb/digital ischaemia
  • Gangrene
  • Amputations
30
Q

What lung conditions can occur in lupus (90% have abnormal lung function/pulmonary involvement)?

A

1) Lupus pneumonitis

2) Lupus serositis

31
Q

Describe pericarditis in lupus

A
  • Common
  • Rapidly resolves with corticosteroids
  • Rarely leads to tamponade so shouldn’t need to treat it as tamponade
  • Rapid onset of pericardial effusion
  • ST elevation
32
Q

Describe myocarditis in lupus

A
  • Common in active SLE
  • Arrhythmias (myocardial scarring can be a focus for arrhythmias)
  • Conduction disturbance
  • Dilated cardiomyopathy
  • Cardiac failure
33
Q

What investigations do you do in myocarditis?

A
  • Troponin-T (sensitive marker of heart inflammation)
  • CK-MB
  • NT-proBNP - heart failure
  • Echocardiography
  • Cardiac MRI (useful for diagnosis, can see myocardial scar)
  • Endomyocardial biopsy (rare)
34
Q

Describe endocarditis in lupus

A
  • Most common on mitral valve but can be any
  • Not infective
  • Due to inflammation (sterile), but can become infection as valve is not functioning properly
  • Libman-Sacks endocarditis
35
Q

Describe features of neuropsychiatric lupus (90% of patients have chronic neuropsychiatric problem due to lupus or living with LTC)

A
  • Psychosis (hallucinations - patients often know they are not real, unlike schizophrenia) - nearly always well treated with anti-psychotics
  • Seizures
  • Strokes
  • Psychological disorders
  • Transverse myelitis
  • Neurological syndromes e.g. MG
36
Q

90% of which type of lupus patients present with (glomerulo)nephritis?

A

Juvenile lupus (young)

37
Q

What is the prevalence of nephritis in lupus?

A

40-75% (predictor of poor outcome)

38
Q

What are the serological features of lupus nephritis (increase risk of nephritis)?

A
  • Anti-dsDNA antibodies

- Low C3 and C4

39
Q

What are the presenting features of lupus nephritis?

A
  • Asymptomatic proteinuria
  • Nephrotic syndrome
  • Renal failure
  • Rapidly progressive glomerulonephritis (rare) like vasculitis patients
  • Typically present with class III or IV
40
Q

What are features of urine cytology in lupus nephritis (v sensitive for glomerulonephritis/ glomerular disease)

A
  • Dysmorphic/fragmented red cells

- Granular casts

41
Q

What investigation should you consider in lupus nephritis unless contraindicated?

A

Renal biopsy - v important to tell severity and how aggressively to treat

42
Q

Describe all the classes of lupus nephritis

A

I) Minimal mesangial LN
II) Mesangial proliferative LN
III) Focal LN (active/chronic)
IV-S) Diffuse segmental proliferative LN
IV-G) Diffuse global proliferative LN (most predictive of renal impairment)
V) Membranous LN (heavy proteinuria)
VI) Advanced sclerotic LN (scarred kidney, dialysis)

43
Q

What are the treatment aims for SLE?

A
  • Induction and maintenance of durable remission
  • Minimise toxicity and treatment related damage
  • Prevent disease flares - reduces damage
  • Avoid long term damage
  • Damage accumulation predicts early death
44
Q

Which drugs are contraindicated in SLE and why?

A

NSAIDs

- They increase risk of stroke and MI and lupus patients are already at increased risk of atherosclerosis

45
Q

Describe use of corticosteroids in SLE

A
  • Lowest effective dose for shortest period of time
  • Prednisolone ≤ 30mg od ± IV/IM methylprednisolone good for anti-inflammation
  • Use alongside hydroxychloroquine
  • Aim to taper to ≤ 7.5mg od or stop
  • Early use of immunosuppressive therapies
46
Q

Describe antimalarial therapy in SLE

A
  • Hydroxychloroquine
  • Combination antimalarials
  • Long term use well-tolerated incl. in pregnancy
  • 65% decreased risk of cardiac neonatal lupus
  • Disease modifying - decreased relapse rate and risk of renal flares
  • Improves lipid and glucose profiles for those on steroids
  • Anti-clotting effect (thromboprophylaxis)
  • Reduces risk of damage and mortality
  • Smoking reduces efficacy
47
Q

What is the first line treatment for all SLE patients?

A

Hydroxychloroquine (safest anti-rheumatic drug)

48
Q

What drugs are used in severe SLE (renal and extra-renal) or if people have poor compliance?

A
  • IV methylprednisolone 500mgs x 3
  • Fortnightly cyclophosphamide 500mgs (contraindicated in pregnancy)
  • Azathioprine
  • Mycophenolate 1.5-3g/day (contraindicated in pregnancy)
49
Q

What is the only licensed biologic for lupus against B cells?

A

Belimumab (also rituximab which binds to CD20, fixes complement and lyses B cells - B cell depleting antibody, atacicept) and infliximab is also used?

50
Q

What is the effect of belimumab?

A
  • Anti-BLyS
  • Blocks B lymphocyte stimulator so it can’t bind to B cells leading to apoptosis of autoreactive B cells
  • Decreases risk of severe SLE flare
  • Well tolerated, no serious adverse effects
51
Q

What things have to be considered in lupus?

A
  • Patient compliance/adherence predicts ESRD in LN
  • Minimising/avoiding steroids
  • CV risks
  • Thrombosis risk
  • Glucocorticoid bone loss
  • Vitamin D deficiency
  • Infection risks/immunisation
  • Risks of malignancy
  • Non-drug management - fatigue, poor sleep, psychological issues
52
Q

What type of disease is SLE?

A

Connective tissue disease incl. vasculitis and inflammation (arthropathy?)

53
Q

Describe the rash in lupus

A
  • Photosensitive distribution e.g. V of neck, forehead, cheeks (butterfly rash)
  • Photosensitive rash can blister
54
Q

What might you see on a fundus photograph of an eye in someone with SLE?

A
  • Vasculitis in eye e.g. retina
  • Patches of yellow = areas of ischaemia where blood supply has stopped
  • Blood vessels have become inflamed, stopped supplying blood to that tissue and the retina has died
55
Q

How do you treat neuropsychiatric lupus syndromes (organic brain syndrome, diffuse disease)?

A
  • Corticosteroids

- Immunosuppression

56
Q

What makes you more likely to die of lupus?

A

The more damage you acquire

57
Q

Which autoantibodies develop early and which ones develop closer to the appearance of symptoms?

A
  • Early = anti-Ro and anti-La

- Later = anti-Sm and anti-dsDNA

58
Q

What are common patterns of organ involvement in SLE?

A

1) Skin - diverse patterns, photosensitivity, alopecia
2) Joints - non-erosive arthritis and tendonitis
3) Sicca symptoms - salivary, lacrimal and genital tract
4) Glomerulonephritis - several patterns incl. mesangial, membranous and peripheral
5) Neurological - CNS, eye, PNS

59
Q

What are the mechanisms of antibody injury in SLE?

A

1) Direct cytotoxicity (and clearance) e.g. autoimmune haemolytic anaemia, thrombocytopenia
2) Immune complex formation and deposition e.g. skin rashes and glomerulonephritis
3) Trigger pro-inflammatory response in cells carrying Fc gamma receptors e.g. macrophages
4) Promote NK cell activation and/or cytotoxicity

60
Q

What happens in the immune system in SLE?

A

1) Complement consumption by immune complexes leading to complement diminishing
2) Excess production of alpha-IFN leading to a state of pseudo-viral infection by immune complexes
3) Activation of TLRs - TLR 7/9 bind to DNA and RNA
4) Recruitment and activation of neutrophils to create tissue damage and inflammation
5) Also involves T helper cells even though antibody mediated

Stage 2: Inflammation (39 decks)

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