Systemic Lupus Erythematosus(SLE)

Question 1

what type of disease is systemic lupus erythematosus(SLE)

Answer 1

a connective tissue disease

Question 2

describe what a connective tissue disease is

Answer 2

conditions associated with spontaneous overactivity of the immune system, specific auto-antibodies are present
(NOT diseases of connective tissue)

Question 3

describe what SLE is

Answer 3

systemic autoimmune condition that can affect any part of the body

Question 4

what group of people are more susceptible to SLE

Answer 4

female 9:1 male, usually presents in child bearing years(20-40), also in afro-caribbean, hispanic american and asian people

Question 5

what factors can influence SLE development

Answer 5

smoking, genetic predisposition, oestrogen, immunological factors

Question 6

what is the initial pathogenesis in SLE

Answer 6

loss of immune regulation

Question 7

describe what happens to apoptosis in SLE patients

Answer 7

increased and defective apoptosis

Question 8

describe the process which leads to auto-antibodies being produced in SLE pathogenesis

Answer 8

necrotic cells release nuclear materials which act as auto-antigens, auto-immunity results from exposure to these auto-antigens, B + T cells activated and produce the auto-antibodies

Question 9

what causes cytokine release in SLE

Answer 9

activation of complement system which attracts leucocyte, which then releases cytokines

Question 10

what does cytokine release result in with SLE

Answer 10

perpetuates inflammation which causes necrosis and scarring

Question 11

what constitutional symptoms are seen in SLE

Answer 11

fatigue and fever

Question 12

what classification criteria is used in SLE, and what score confirms diagnoses

Answer 12

EULAR/ACR criteria

score >10

Question 13

what must be positive for SLE diagnoses

Answer 13

ANA(anti-nuclear antibody)

it is not specific for SLE though

Question 14

what cutaneous(skin) features can be seen in SLE

Answer 14

subacute cutaneous/discoid lupus, acute cutaneous lupus, non-scarring alopecia, oral ulceration

Question 15

what different systems can SLE affect

Answer 15

cutaneous, neurological, renal, haematological, joints, serous membrane

Question 16

describe the effect SLE can have on the joints

Answer 16

arthritis- synovitis or tenderness in at least 2 joints with > 30mins early morning stiffness

Question 17

what neurological effects can SLE have

Answer 17

delirium, headache, seizure, psycosis

Question 18

how is the serous membrane affected in SLE

Answer 18

by serositis, usually presents as pleural effusion or pericardial effusion in acute pericarditis

Question 19

what haematological effects can SLE have

very common

Answer 19

leukopenia(common), thrombocytopenia, haemolytic anaemia

Question 20

what symptom is often seen in renal disease

Answer 20

asymptomatic usually until late stage

important to test for renal involvement early

Question 21

what different ways can renal involvement in SLE be screened for

Answer 21

proteinuria >0.5g in 24 hours(blood/protein in urine)

or biopsy proven nephritis

Question 22

what auto-antibodies are seen in SLE

Answer 22

anti-nuclear antibodies(ANA), anti-double stranded DNA antibody(dsDNA), antiphospholipid antibody(APLs)

Question 23

describe the specificity of the ANA, dsDNA and anti Sm auto-antibodies seen in SLE

Answer 23

ANA - in almost all SLE patients, but not that specific
dsDNA - very specific, but not present in all
anti Sm - highly specific for SLE

Question 24

describe the immunological marker of the complement system in SLE

Answer 24

C3 and C4 lower in more active disease