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Systematic lupus erythematosus Flashcards

1
Q

epidemiology of systemic lupus erythematosus

A

rare - 3 in 10000pts approx

diagnosis is often missed

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2
Q

how does lupus relate to other diseases

A

part of a family of chronic overlapping autoimmune diseases - they all share features - spectrum from mainly affecting bone to mainly affecting muscle

  • Rheumatoid arthritis - mainly affect bone (mainly inflammatory joint disease)
  • Sjögren’s syndrome
  • systemic lupus erythematosus - can effect bone, liver, lung, kidneys and brain etc
  • Dermatomyositis (primarily muscle based)
  • Polymyositis (primarily muscle based)
  • Systemic sclerosis (muscle, skin and joint involvement)

distinguished by Ab profile and presentation

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3
Q

who and what does lupus generally effect *

A

females more than men

usually young 15-40 yrs - can be children and cross placeta (rarer - more likely to start before puberty)

increased prevalence in afro-caribbean, asian and chinese populations - because there is increased prevalence of the milder forms here

prevalence varies between 4-280/100000

mainly affects joints and skin, can effect lungs, kidney, haematology (bone marrow)

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4
Q

describe the genetic basis of lupus *

A

multiple genes implicated - polygenetic

number of disease suseptibility genes - some impact prognosis and outcome

of you have C1q and C3 deficiency - definitely going to get lupus

polymorphisms of Fc receptros, IRF5, CTLA4, and class II HLA genes are overespressed in lupus

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5
Q

what are the clincial features of lupus *

A

presentation - malaise, fatigue, fever, weight loss, lymphadenopathy - if pt keeps coming abck with these symptoms - think lupus

specific features - butterfly rash (rare), alopecia, arthralgia (low grade aches and pains), reynaud’s phenomenon (a lot of people have in population, depends on context and if the ot keeps coming back)

other features - inflammation of kidney (acute/chronic renal failure), CNS (psychosis), heart (life threatening myocarditis), lungs, accelarated atherosclerosis, vasculitis (rash affecting hands and feet)

depends on the organs effected

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6
Q

what is highlighted by the criteria for lupus *

A

it is a spectrum of disease

affects multiple systems

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7
Q

identify features of lupus *

A

pt look miserable - feel unwell - grinding fatigue

subtle rash on cheek (also in sun-exposed areas ie forearm and neck) - sparing nasolabial fold

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8
Q

identify the features of lupus *

A

superficial epidermal rash - no pus so different from acne

has erythema around (redness) and blocked comedones

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9
Q

identify features of lupus *

A

rash progress into dermis - affect pigmentation = scarring - need cosmetic treatment

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10
Q

identify features of lupus *

A

scarring alopecia - can respond to steroids, but need cosmetics

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11
Q

description of lupus rash *

A

wolf rash - pattern is similar to wolf colourings

lupus is latin word for wolf

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12
Q

describe the pathogenesis of lupus *

A

many things go wrong

environmental and genetic predisposition to autoimmune

environmental not well understood - perhaps virus eg EBV - signalling by toll-like receptors

when pts acquire mild disease (exposure to environmental anitgens) - can kick off severe disease flare - not in everyone indicationg genetic predisposition

this happens a number of times

then get chronically activated innate immune system (B cell hyperactivity is driven)

transition to adaptive immune response - this sets up generation of auto-Ab and immune complexes

this sets up a viscious cycle and disease state leading to aberrent amplification pathways and irreversible, complement mediated, tissue damage

ALSO there is abnormal clearance of apoptotic cells - have occult Ag on cell surface exposed to the active immune system - dendritic cells uptake the ag and activate b cell- b cell Ig class switch and affinity maturation - get IgG autoAb response to these nuclear Ag (production of anti-nuclear Ab) = immune complexes

the combination of B cell hyperactivity and inability to get rid of autoag = generation of auto-ab and immune complexes - settle in skin and kidney - they activate complement = complement mediated tissue injury

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13
Q

diagnosis of lupus *

A

look for antinuclear ab (ANA), not diagnositic but indicator that there is an autoimmune disease going on - the patterns differentiate between different disease types

anti-dsDNA - highly common in lupus

anti-Sm (smith ag) - specific for lupus, diagnositic, if present more likely to have severe disease

anti-Ro and or La - more closely associated to Sjogren’s but can be found in lupus, common in subacute cutaneous lupus, in neonatal lupus syndrome

increased complement consumption - low levels of C3 and 4 because complement is being used up - deposited in the skin amd activated in the lung and kidney

anti-cardiolipin Ab, lupus anticoagulanet and B1 glycoprotein - pro-coagulant ab - increase risk of thrombosis

see how severe damage to organs is:

haematology - lymphopenia, normochromic anaemia (due to haemolysis), leukopenia, AIHA, thrombocytopenia (risk of life threatening haemorrhage)

renal - proteinuria (injury to kidney), haematuria, active urinary sediment

eyes not normally effected

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14
Q

significance of the different patterns of ANA *

A

homogenous - Abs to DNA - in lupus

speckled - Abs to Ro, La, Sm, RNP - lupus overlap syndromes

nucleolar - topoisomerase - scleroderma

centromere - limited cutaneous scleroderma

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15
Q

how do you assess the severity of lupus *

A

identify the pattern of organ involvement

monitor the function of the organs

  • renal - BP, urea and electolytes and creatinine, GFR
  • lungs/cvs - lung func/echocardiography
  • skin, haematology, eyes

identify the pattern of the Ab expressed - anti-drDNA, anti-sm indicate renal diease, are anti-cardiolipin ab present

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16
Q

how do you pre-empt severe attacks *

A

look at clinical features - weight loss, fatigue, malaise, hairloss, alopecia, rash - are they better or worse

lab markers

  • ESR - gets increased in active disease
  • increased complement consumption = low C3 4 = active disease
  • increased anti-dsDNA
  • otehr ab ie ANA and CRP are poor indictors (CRP stays normal in lupus)
17
Q

what are the 3 groups of lupus *

A

mild - joint problems, maybe skin

moderate - inflammation of other organs, particularly kidney - pleuritis, pericarditis, mild nephritis

severe - sever inflammation in vital organs eg sever nephritis, CNS disease (fits, depression, psychosis), pul disease (shrinking lung - rare), cardiac involvement (myocarditis - rare), AIHA, thrombocytopenia, Thrombotic thrombocytopenic purpura (TTP)

18
Q

treatment of mild lupus *

A

first paracetamol +/- NSAID - monitor renal function

hydroxychloroquine if rash - athropathy, cutaneous manifestations, mild disease activity

topical corticosteroids - not for use on face

19
Q

treatment of moderate lupus *

A

indication - failure of hydroxychloroquine/NSAID, organ/life threatening disease

based on corticosteroids

  • initial high dose - suppress activity - send it into remission
  • iv methylprdnisolone 3x0.5-1g per 24hr
  • initial oral dose for 4 weeks
  • reduce slowly over 2-3months to 10mg/d
  • reduce slowly at 1mg/month

want to keep in remission by low dose/alternative to steroid because of SE

20
Q

SE of corticosteroids *

A

weight gain

dm

acne

osteoporosis

21
Q

treatment of severe lupus *

A

immunosuppressants

azathiorprine

  • safe in pregnancy
  • for moderate to severe disease - 2.5mg/kg/day
  • effective steroid sparing agent - use with steroids to reduce amount of steroids
  • SE - lower WBC (nwutropenia) BM suppression - therefore have to do regular FBC and biochem monitorinhg, can lower liver func

cyclophosphamide

  • for severe organ involvement - acute renal failure/myocarditis
  • iv pulsed or oral
  • for nephritis - 6/month IV pulses
  • bladder toxicity lead to carcinoma, BM suppression, infertility, cystitis
22
Q

mechanism of mycopgenolate mofetil for lupus *

A

reversible inhibitor of inosine monophosphate dehydrogenase

rate limiting enzyme in de nevo purine synthesis - lymphocytes are dependsant on this purine synthesis

well tolerated and has no effect on fertility

23
Q

describe rituximab for lupus *

A

B cell targeted therapy

anti-CD20 monoclonal Ab

binds to b cells in circulation - fixes complement

leads to the depletion of B cells

well tolerated

24
Q

describe belimumab in treatment of lupus *

A

blocks Blys = reduced activity of B cells

switches off the hyperactivity of the B cells

25
Q

describe the prognosis of lupus *

A

15yr survival when had no nephritis = 85%, when had nephritis = 60% - ie prognosis depends whether disease is mild/mod/severe

prognosis worse if black, male, or low socioeconomic status

26
Q

describe the bimodal mortality pattern of lupus *

A

as treatment got better - fewer people dies of infection, cardiomyocarditis, or renal failure

people survived for decades - another cause of death - MI - accelarated atherosclerosis which is more severe - plaques that are more likely to be a problem - cause strole/MI

27
Q

why do you look for oral ulceration in lupus *

A

sign of active disease

28
Q

what can be seen in a blood film of lupus *

A

schistocytes

spherocytes

anisocytosis

poikilocytosis

fibrin strands - because of inflammation - rip red cells = schistocytes

thrombocytopenia

29
Q

what can be seen in renal biopsy in lupus *

A

too many cells - proliferative

cresecnt of inflammation - crescentic glomerulonephritis

30
Q

how would you treat a pt with nephritis and nephrotic syndrome

MAHA
and pneumonia *

A

prednisolone 60mg daily - aggressive to target the lupus

cyclophospohade 750mg iv pulse

IV AB - aggressively treat the pneumonia

blood, platelet and plasma transfusions - supportive

quadruple anti-hypertensive therapy

31
Q

principles of treatment *

A

symptomatic - get people feeling better and more mobile

immunosuppressive

immue -modulating

musculoskeletal (12 decks)

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