Systemic Lupus Erythematosus

Question 1

Describe the pathogenesis of SLE?

Answer 1

Defect in apoptosis- causing increased cell death and disturbance in immune tolerance

Defective clearance of apoptotic debris allows persistence of antigen and immune complex production

Immune complexes form in small blood vessels, leading to complement activation and inflammation

Antibody-antigen complexes are deposited on the basement membranes of skin and kidneys

Question 2

Who is most likely to be affected by SLE?

Answer 2

Black people in UK and US

90% women

20-30

Question 3

What is the prognosis of SLE?

Answer 3

10 year survival 90%

Question 4

Constitutional symptoms of SLE?

Answer 4

Fever, fatigue, weight loss

Question 5

MSK symptoms of SLE?

Answer 5

Arthralgia, myalgia, inflammatory arthritis

Increased avascular necrosis of femoral head

Question 6

Mucocutaenous symptoms of SLE?

Answer 6

Malar rash, photosensitivity, discoid lupus, subacute cutaneous lupus, oral/nasal ulceration, raynauds

Question 7

Renal symptoms of SLE?

Answer 7

Lupus nephritis

Question 8

Respiratory symptoms of SLE?

Answer 8

Pleurisy, pleural effusion, pneumonitis, PE, pulmonary hypertension, ILD

Question 9

Haematological symptoms of SLE?

Answer 9

Leukopenia, lymphopenia, anaemia (potentially haemolytic), thrombocytopenia

Question 10

Neuropsychiatric symptoms of SLE?

Answer 10

Seizures, psychosis, headache, aseptic meningitis

Question 11

Cardiac symptoms of SLE?

Answer 11

Pericarditis, pericardial effusion, pulmonary hypertension, sterile endocarditis, accelerated IHD

Question 12

GI symptoms of SLE?

Answer 12

Less common

Autoimmune hepatitis, pancreatitis, mesenteric vasculitis

Question 13

Investigations for lupus

Answer 13

No one diagnostic test;
FBC

Anti-nuclear antibody - +ve >95% not specific

Anti-dsDNA antibody- +ve specific and varies with disease activity

3/4 levels= low when disease active

Urinalysis for glomerulonephritis

Image for organ involvement

Anti-Sm- specific but low sensitivity
Anti-Ro, Anti-La, anti-RNP- seen in multiple condition

Question 14

Describe the management of SLE presenting with Skin Disease and arthralgia?

Answer 14

Hydroxychloroquine (DMARD), topical steroids, NSAIDs

Question 15

Describe the management of SLE presenting with inflammatory arthritis or organ involvement?

Answer 15

Immunosuppression

• azathioprine
• mycophenolate mofetil

Corticosteroids for short periods

Question 16

Describe the management of SLE presenting with severe organ damage?

Answer 16

IV steroids and cyclophosphamide

Question 17

Describe the management of unresponsive SLE?

Answer 17

IV immunoglobulin and rituximab

Question 18

How is SLE monitored?

Answer 18

check anti-dsDNA antibodies and complement levels

Check urinalysis for blood or protein

BP and cholesterol