Systemic Lupus Erythematosus

Question 1

Define Systemic Lupus Erythematosus

Answer 1

Chronic autoimmune disease causing tissue inflammation in the presence of antibodies against self-antigens, principally affecting joints and skin, kidney, lungs, and haematology

Question 2

Aetiology of Systemic Lupus Erythematosus

Answer 2

Abnormal clearance of apoptotic cell amterial → dendritic cell uptake of autoantigens + activation of B cells → IgG autoantibodies → immune complex formation → complement activation

Associated with complement deficiency C1q and C3
May be drug-induced: hydralazine, procainamide, isoniazid, minocycline, phenytoin (anti-histone Abs)

Question 3

Epidemiology of Systemic Lupus Erythematosus

Answer 3

M:F = 1:9
Presentation at 15-40 years
Increased in Afro-Caribbean, Asian, Chinese

Question 4

Symptoms and signs of Systemic Lupus Erythematosus

Answer 4

Hair loss/alopecia
Cerebral: cerebral lupus e.g. psychosis
Malar rash (butterfly erythema, spares the nasolabial fold)
Photosensitive rash
Mouth ulcers
Raynaud’s phenomenon
Arthralgia and arthritis
Serositis: pericarditis, pleuritis, peritonitis
Renal disease: glomerulonephritis
Constitutional: Fatigue, Weight loss, Fever, Malaise

Question 5

Investigations for Systemic Lupus Erythematosus

Answer 5

urine dip: ?haematuria
Urine protein:creatinine ratio: ?renal disease

ANA:
- dsDNA (homogenous)
- anti-Ro, La, Sm, RNP (speckled)
- topoisomerase (nucleolar)
- Centromere
Cytoplasmic antibodies
- Anti-tRNA synthestase
- Antu ribosomal P
Complement: 1. C4 2. C3
Anti-cardiolipin, lupus anticoagulant, beta-2 glycoprotein
ESR
FBC: ?anaemia, leucopenia, thrombocytopenia
Coagulations screen: ?ALPSS (APTT falsely prolonged)

Question 6

How is disease activity/severity measured in Systemic Lupus Erythematosus

Answer 6

ESR (increases while CRP decreases)
Increased complement consumption
Increased anti-dsDNA
Other antibodies e.g. ANA and CRP poor indicators

Question 7

Maintenance management for Systemic Lupus Erythematosus

Answer 7

Conservative:
Sun protection: sun cream, low-dose steroids
Vaccination
Exercise
No smoking
Body weight
Blood pressure control
Lipids
Glucose
Test for ALPS

Maintenance
1. Hydroxychloroquine (max 5mg/kg)
± low-dose steroids PO/IM
± DMARDS (MTX, azathioprine, CNI, MMF, CYC)
2. Severe disease: biologicals (anti-B-cell) e.g. belimumab, rituximab

Question 8

Management for flares of Systemic Lupus Erythematosus

Answer 8

AIHA, nephritis, pericarditis, CNS disease) → prednisolone + IV cyclophosphamide
Proteinuria → ACEi
Aggressive GN (lupus nephritis) → immunosuppression

Question 9

What are the markers of low disease activity for Systemic Lupus Erythematosus

Answer 9

SLEDAI <4
HCQ
Pre <7.5mg/d

Question 10

Complications of treatment for Systemic Lupus Erythematosus

Answer 10

Steroid use: cataracts, osteoporosis, DM, avascular necrosis
Cyclophosphamide: amenorrhoea, male infertility, malignancy, haematuria

Question 11

Complications of Systemic Lupus Erythematosus

Answer 11

Chronic active lupus
Haem: anaemia, leukopenia, thrombocytopenia
Cardio: pericarditis, myocarditis, endocarditis, valvular heart disease
Resp: pleuritis, pleural effusion, pulmonary HTN, pulmonary haemorrhage
Vascular: raynaud’s phenomenon, arterial/venous thrombosis
Gastro: lupus peritonitis
MSK: tenosynovitis, Jaccoud’s arthritis, tendon rupture

Question 12

Prognosis for Systemic Lupus Erythematosus

Answer 12

15 year survival: No nephritis 85% | Nephritis 60%
Prognosis also worse if black, male, low socio-economic status
Patients with SLE have higher rates of death from all causes, regardless of sex, ethnicity, renal disease, cardiovascular disease, or infection. However, the risk of death due to malignancy is not increased. The most common cause of mortality is cardiovascular disease, followed closely by infection and severe disease activity.