Systemic Lupus Erythematosis

Question 1

what type of condition is this

Answer 1

chronic autoimmune

Question 2

where does this mainly involve

Answer 2

skin, joints, kidneys, blood cells and nervous system

Question 3

how is lupus diagnosed

Answer 3

clinical findings and laboratory evidence

Question 4

what is the pathogenesis

Answer 4

• development of autoantibodies could involve a defect in apoptosis
• defective clearance of the apoptotic cell debris allows for the persistence of antigen and immune complex production

Question 5

what are clinical manifestations mediated by

Answer 5

• circulating immune complexes that form with antigens in various tissues
• immune complexes form in the small blood vessels leading to complement activation and inflammation
• antibody-antigen complexes are deposited on the basement membranes of skin and kidneys

Question 6

is there a genetic link in lupus

Answer 6

yes

Question 7

is lupus more common in women or men

Answer 7

women

90% of cases

Question 8

what age does lupus tend to start in women

Answer 8

childbearing age

women:men peaks at 11:1 at this age

Question 9

what is the 10 year survival rate

Answer 9

90%

Question 10

in people who die of lupus what do they die from

Answer 10

side effects of the medication rather than the disease e.g severe infections due to immunosuppression

Question 11

what are some general presentations

Answer 11

fever, fatigue, weight loss

Question 12

what are some MSK presentations

Answer 12

arthralgia, myalgia and inflammatory arthritis (arthropathy is rarely erosive or deforming), increased prevalence of avascular necrosis of femora head

Question 13

what are some muco-cutaneous presentations

Answer 13

marlar rash, photosensitivity, discoid lupus, subacute cutaneous lupus, oral/nasal ulceration and raynauds phenomenon

Question 14

what are some renal presentations

Answer 14

lupus nephritis

Question 15

what are some respiratory symptoms

Answer 15

pleurisy, pleural effusion, pneumonitis, PE, pulmonary hypertension and interstitial lung disease

Question 16

what are some haematological symptoms

Answer 16

leukopenia, lymphopenia, anaemia (may be haemolytic) and thrombocytopenia

Question 17

what are some neuropsychiatric symptoms

Answer 17

seizures, psychosis, headache, aseptic meningitis

Question 18

what are some cardiac symptoms

Answer 18

pericarditis, pericardial effusion, pulmonary hypertension, sterile endocarditis and accelerated ischaemic heart disease

Question 19

what are some GI symptoms

Answer 19

less common but autoimmune hepatitis, pancreatitis and mesenteric vasculitis

Question 20

what are some investigations

Answer 20

no one diagnostic test
FBC
anti-nuclear antibody
anti-dsDNA
anti-Sm
anti Ro, anti-La and anti-RNP
C3/4 levels
urinalysis
imaging

Question 21

how is lupus managed

Answer 21

depends on manifestations
skin disease and arthralgia: hydroxychloroquine, topical steroids and NSAID’s
inflammatory arthritis or organ involvement: immunosuppression (azathioprine or mycophenolate mofetil) and corticosteroids can be used for a short time
severe organ disease: IV steroids and cyclophosphamide
unresponsive cases: IV immunoglobulin and rituximab

Question 22

how is this monitored

Answer 22

check anti-dsDNA regularly as vary with disease activity, check urinalysis for blood or protein, evaluate and manage CV risk factors, BP and cholesterol too