Systemic Lupus Erythematosus

Question 1

What is SLE?

Answer 1

A multisystemic autoimmune disease with inadequate T cel suppressor activity and increased B cell activity.

It is autoantibody driven where patients have antibodies to certain cell nucleus components.

There is inadequate clearance of immmune complexes resulting in a host of immune responses which cause tissue inflammation and damage.

Question 2

Epidemiology of SLE.

Answer 2

9 times more common in women.

It is usually premenopausal women.

Peak age of onset is between 20 and 40 years.

Question 3

Aetiology of SLE.

Answer 3

Heredity and genetics

Sex hormone status (pre-menopausal more common)

Drugs lik hydralazine, isoniazid, procainamide and penicillamine can induce a mild form of SLE.

UV light triggers flares (rash is photosensitive)

Exposure to EBV.

Question 4

Common symptoms and signs of SLE.

Mnemonic SOAP BRAIN.

Answer 4

Serositis - pleurisy and pericarditis

Oral ulcers

Arthritis - small joints non-erosive

Photosensitivity - malar or discoid rash

Blood disorders

Renal involvment - GN

Autoantibody postive

Immunlogic tests

Neurologic disorder - seizures or psychosis.

Question 5

Expain oral ulcers in SLE.

Answer 5

Usually painless.

Palate is most specific

Question 6

Blood disorders in SLE.

Answer 6

Low WCC

Lymphopenia

Thrombocytopenia

Haemolytic anaemia

Question 7

General features of SLE.

Answer 7

Fever is common in exacerbations

Malaise and tiredness.

Question 8

Systems involved in SLE.

Answer 8

Joints - > 90%

Skin - 85%

Lungs - 50%

CVS - 25%

Kidneys - 30%

Nervous system - 60%

Eyes

GI

Question 9

Skin features of SLE.

Answer 9

Photosensitivity

Butterfly rash

Vasculitis

Purpura

Urticaria

Question 10

Joint features of SLE.

Answer 10

Aseptic necrosis of hip (rare)

Arthritis of small joints

Question 11

Lung features of SLE.

Answer 11

Pleurisy

Pleural effusion

Restrictive lung defect with fibrosis (rare)

Question 12

CVS features of SLE.

Answer 12

Pericarditis

Endocarditis

Aortic valve lesions

Raynaud’s

Vasculitis

Arterial and venous thromboses

Question 13

Kidney features of SLE.

Answer 13

Lupus nephritis

Question 14

Nervous system features of SLE.

Answer 14

Fits

Hemiplegia

Ataxia

Polyneuropathy

Cranial nerve lesions

Psychosis

Demyelinating syndromes

Question 15

Eye features of SLE.

Answer 15

Retinal vasculitis

Episcleritis

Conjunctivitis

Optic neuritis

2ndary Sjögren’s syndrome

Question 16

Investigations of SLE.

Answer 16

FBC

U&Es

Autoantibodies

Serum complement

Urinalysis

Skin and renal biopsy

Imaging

Question 17

FBC features in SLE.

Answer 17

May show leucopenia, lymphopenia and/or thrombocytopenia.

Anaemia of chronic disease or autoimmune haemolytic anaemia might be present.

ESR is raised and plasma viscosity is raised however CRP is normal.

Question 18

U&Es features of SLE.

Answer 18

Urea and creatinine only rise when renal disease is advanced.

Low serum albumin or high urine albumin:creatinine ratio are earlier indicators of lupus nephritis.

Question 19

Autoantibodies suggestive of SLE.

Answer 19

ANAs such as;

anti-dsDNA

anti-Ro

anti-Sm

anti-La

They are present in 90% of cases.

Question 20

What does antiphospholipid antibodies suggest?

Answer 20

Increased risk of pregnancy loss and thrombosis.

Antiphospholipid antibodies and antiphospholipid syndrome can occur secondary to SLE. They can occur in up to 40% of patients with SLE and are associated with an increased risk of venous thromboembolism.

Question 21

How can anti-DsDNA be used?

Answer 21

Titre rises with disease activity.

Question 22

C3 and C4 levels in SLE.

Answer 22

It will fall in active disease.

A combination of high ESR, high anti-dsDNA and low C3 may herald a flare of disease.

Question 23

Diagnostic criteria of SLE.

Answer 23

You can use the SLICC Criteria or the ACR Criteria for establishing a diagnosis.

Any ≥4 of the 11 criteria are required to classify a patient as having SLE.

This is ACR criteria

1 - Malar rash

2 - Discoid rash

3 - Photosensitivity

4 - Oral ulcers

5 - Arthritis

6 - Serositis

7- Renal disorder

8 - Neurological disorder

9 - Haematological disorder

10 - Immunologic disorder

11 - Antinuclear antibody

Question 24

Laboratory criteria of SLE.

Answer 24

(Based on SLICC criteria)

+ve ANA

Anti-dsDNA

Anti-Sm

Antiphospholipid Abs

Low complement

+ve Direct Coombs test

Question 25

General management of SLE.

Answer 25

Sun protection to prevent UV flares.

Screen for co-morbidities and medication toxicity

Advice on healthy lifestyle

Question 26

Maintenance management for SLE.

Answer 26

NSAIDs (unless renal) and hydroxychloroquine for joint and skin symptoms (in mild disease)
Steroids are used in more extensive disease.

In resistant or more severe lupus;

Azathioprine, methotrexate and mycophenolate as steroid sparing agents.

Refractory and severe lupus;

Belimumab and rituximab can be used as an add-on when disease is high.

Question 27

Treatment of mild flares with no serious organ damage.

Answer 27

Hydroxychloroquine or low dose steroids.

Question 28

Treatment of moderate flares with organ involvement.

Answer 28

May require DMARDs or mycophenolate

Question 29

Treatment of severe flares.

Answer 29

Urgent high-dose steroids, mycophenolate, rituximab, cyclophosphamide.

Question 30

Treatment of lupus nephritis.

Answer 30

Steroids and cyclophosphamide or mycophenolate.

BP control with ACEi

Renal replacement therapy may be indicated.

Question 31

Prognosis of SLE.

Answer 31

80% surv at 15 years.

Increased long-term risk of CVD and osteoporosis.

Question 32

Complications of SLE

Answer 32

Cardiovascular disease = Leading cause of death

Infection (immunosuppression

Anaemia of chronic disease

Pericarditis

Pleuritis

Interstitial lung disease

Lupus nephritis

Neuropsychiatric SLE

Recurrent miscarriage

Venous thromboembolism