Systemic Lupus Erythematosus Flashcards

1
Q

What is SLE?

A

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.

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2
Q

Epidemiology of SLE.

A

9 times more common in women.

It is usually premenopausal women.

Peak age of onset is between 20 and 40 years.

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3
Q

Aetiology of SLE.

A

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.

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4
Q

Common symptoms and signs of SLE.

Mnemonic SOAP BRAIN.

A

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.

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5
Q

Expain oral ulcers in SLE.

A

Usually painless.

Palate is most specific

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6
Q

Blood disorders in SLE.

A

Low WCC

Lymphopenia

Thrombocytopenia

Haemolytic anaemia

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7
Q

General features of SLE.

A

Fever is common in exacerbations

Malaise and tiredness.

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8
Q

Systems involved in SLE.

A

Joints - > 90%

Skin - 85%

Lungs - 50%

CVS - 25%

Kidneys - 30%

Nervous system - 60%

Eyes

GI

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9
Q

Skin features of SLE.

A

Photosensitivity

Butterfly rash

Vasculitis

Purpura

Urticaria

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10
Q

Joint features of SLE.

A

Aseptic necrosis of hip (rare)

Arthritis of small joints

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11
Q

Lung features of SLE.

A

Pleurisy

Pleural effusion

Restrictive lung defect with fibrosis (rare)

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12
Q

CVS features of SLE.

A

Pericarditis

Endocarditis

Aortic valve lesions

Raynaud’s

Vasculitis

Arterial and venous thromboses

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13
Q

Kidney features of SLE.

A

Lupus nephritis

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14
Q

Nervous system features of SLE.

A

Fits

Hemiplegia

Ataxia

Polyneuropathy

Cranial nerve lesions

Psychosis

Demyelinating syndromes

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15
Q

Eye features of SLE.

A

Retinal vasculitis

Episcleritis

Conjunctivitis

Optic neuritis

2ndary Sjögren’s syndrome

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16
Q

Investigations of SLE.

A

FBC

U&Es

Autoantibodies

Serum complement

Urinalysis

Skin and renal biopsy

Imaging

17
Q

FBC features in SLE.

A

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.

18
Q

U&Es features of SLE.

A

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.

19
Q

Autoantibodies suggestive of SLE.

A

ANAs such as;

anti-dsDNA

anti-Ro

anti-Sm

anti-La

They are present in 90% of cases.

20
Q

What does antiphospholipid antibodies suggest?

A

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.

21
Q

How can anti-DsDNA be used?

A

Titre rises with disease activity.

22
Q

C3 and C4 levels in SLE.

A

It will fall in active disease.

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

23
Q

Diagnostic criteria of SLE.

A

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

24
Q

Laboratory criteria of SLE.

A

(Based on SLICC criteria)

+ve ANA

Anti-dsDNA

Anti-Sm

Antiphospholipid Abs

Low complement

+ve Direct Coombs test

25
Q

General management of SLE.

A

Sun protection to prevent UV flares.

Screen for co-morbidities and medication toxicity

Advice on healthy lifestyle

26
Q

Maintenance management for SLE.

A

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.

27
Q

Treatment of mild flares with no serious organ damage.

A

Hydroxychloroquine or low dose steroids.

28
Q

Treatment of moderate flares with organ involvement.

A

May require DMARDs or mycophenolate

29
Q

Treatment of severe flares.

A

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

30
Q

Treatment of lupus nephritis.

A

Steroids and cyclophosphamide or mycophenolate.

BP control with ACEi

Renal replacement therapy may be indicated.

31
Q

Prognosis of SLE.

A

80% surv at 15 years.

Increased long-term risk of CVD and osteoporosis.

32
Q

Complications of SLE

A

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