Autoimmune - SLE

Question 1

OVERVIEW
i) what is it? how does it present? what causes it immunologically?
ii) which cells produce autoantibodies? what are they produced to?
iii) name a tissue where antibodies may be deposited and what this causes
iv) which two populations is there increased incidence in? what is F:M?

Answer 1

i) chronic multisystem autoimmine disorder that has a broad spectrum of clinical presentations - caused by an irreversible break in immune tolerance
ii) activated T and B cells produce autoantibodies to endog nuclear antigens
iii) antibodies can depos in the kidney and cause end organ damage
iv) increased prevalence in african and asian populations
F:M = 9:1

Question 2

CLINICAL FEATURES
i) name three systemic symptoms?
ii) name three MSK manifestations
iii) what may be seen in the hair? is this temporary or permanent?
iv) name three characteristic of rashes that may be seen? which antibody are these associated with?
v) name three other skin manifestations? what may be seen in the mouth

Answer 2

i) fatigue, lethargy, reduced exercise tolerance, episodic fever, weight loss
ii) MSK - migratory arthralgia (flitting joint pain), non erosive/non deforming arthritis (knees, wrist and PIPJ) and myalgia
iii) alopecia - usually temporary unless discoid lesions > scarring
iv) photosensitive (also exac systemic disease), classic butterfly shape, maculopapular, discoid lesions associated with anti Ro antibody
v) livedo reticularis (lacy redness), vasculitic tash, uritcarial rash, raynauds
mouth ulcers

Question 3

DISCOID LUPUS
i) what type of lesion is seen? (3)
ii) what may it cause on the scalp?
iii) does it always occur with systemic manifestations of lupus?

Answer 3

i) scarring, discrete annular plaque, background erythema, depressed central scar with scaling
ii) can cause rash on scalp that causes scarring alopecia that is permanent due to loss of follicles
iii) can occur in the absence of systemic manifestations > 10% with discoid lupus will go on to develop SLE

Question 4

RAYNAUDS PHENOMENON
i) what is it? what is it provoked by? name the three colour changes seen
ii) name three body parts it can affect
iii) what causes primary? what can cause secondary? (3)

Answer 4

i) periodic vasospastic disorder of extremities - ischaemia provoked by cold expsosure
see pallor, cyanosis and hyeraemia (white blue red)
ii) can affect fingers, toes, tip of nose, ear lobes
iii) primary is often familial and onset in teens
secondary - caused by AI disease, occupation (vibrating tools), drugs (beta blockers, bleomycin, vinblasine), chemicals (polyvinyl chloride)

Question 5

OTHER ORGAN INVOLEMENT IN SLE
i) name three pulmonary manifestations
ii) name three cardiovascular manifestations? what can neonatal lupus be associated with?
iii) what causes renal involvement? name two things that can result from this?
iv) name three neuropsych abnormalities
v) disease in which two organs confers a worse prognosis? what should be done in these cases?

Answer 5

i) pleuritic chest pain with pleural rub, lupus pneumonitis, pulm fibrosis, pulm hypertension
ii) pericarditis with pericardial rub, endocarditis, CAD, hypertension, conduction defects - neonatal lupus assoc with materal Ro antibody (passed from mum to baby)
iii) immune complexes get stuck in kidneys > prolif glomerulonephritis and membranous nephritis
iv) depression, headache, seizure, anxiety, psychosis (if severe), stroke
v) renal or CNS disease (can mimick demyelination) - treat aggressively

Question 6

ANTI PHOSPHOLIPID SYNDROME
i) name four features that may be seen? what plat counts may be seen?
ii) name two antibodies seen? is the condition anti or pro coag?
iii) which two medications can be given?
iv) which characteristic rash is seen?

Answer 6

i) venous thrombosis, arterial thrombosis (stroke) recurrent miscarriage and migraines
- see low plats
- ii) see anticadiolipin antibodies and beta 2 glycoprotein 1 antibodies
condition is pro coagulant
iii) can give antiplat agents eg aspirin and anti anticoag eg warfarin (doacs dont work very well)
iv) see livedo rash

Question 7

SLE INVESTIGATIONS
i) what may be seen on FBC? will ESR be low or high? is LFT and bone profile affected?
ii) what will CRP be?
iii) name a non spec and spec antibody seen? name two ENA antibodies?
iv) name three other investigations that may be done

Answer 7

i) low RBC and WBC
raised EST and normal LFT/bone profile
ii) CRP will be normal unless concurrent infection
iii) non spec ANA and spec DNA antibodies
ENA - Ro and La
iv) urinalysis, histology of the kidney and radiology

american college or rheum classific for SLE
malar rash, discoid rash, photosensitive, oral ulcer, non erosive arthritis, pleuritis/pericarditis, renal disorder, neuro disorder - need four or more

Question 8

SLE TREATMENT
i) what drug is usually given initially and is steroid sparing?
ii) what steroid may be given - what is done to dose if there is lung involvement?
iii) name three other drugs given if lung involvement
iv) what chemo drug can be given in renal or CNS disease?
v) when may rituximab be given?

Answer 8

i) hydroxychloroquinine
ii) given oral prednisolone and increase dose if lung involvement
iii) methotrexate, azothiaprine or mycophenoloate mofetil
iv) cyclo
v) if there is an acute flare of renal disease