Systemic Lupus Erythematosus Flashcards

(32 cards)

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
General management of SLE.
Sun protection to prevent UV flares. Screen for co-morbidities and medication toxicity Advice on healthy lifestyle
26
Maintenance management for SLE.
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
Treatment of mild flares with no serious organ damage.
Hydroxychloroquine or low dose steroids.
28
Treatment of moderate flares with organ involvement.
May require DMARDs or mycophenolate
29
Treatment of severe flares.
Urgent high-dose steroids, mycophenolate, rituximab, cyclophosphamide.
30
Treatment of lupus nephritis.
Steroids and cyclophosphamide or mycophenolate. BP control with ACEi Renal replacement therapy may be indicated.
31
Prognosis of SLE.
80% surv at 15 years. Increased long-term risk of CVD and osteoporosis.
32
Complications of SLE
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