Treatment / Management

• Course of the disease
• SLE with renal involvement
• SLE without renal involvement
• SLE with central nervous system involvement

The Course of the Disease

“SLE is a chronic condition in which a low level of baseline activity is punctuated by flares of higher activity”. – Oxford Textbook of Medicine

However, the course of the disease is highly unpredictable and there are limited reliable tools available for assessing disease severity for different people.

The natural history of the disease is usually that of an initial flare, followed by many years of low- grade activity. This, however, varies from person to person and some people may have very aggressive, active disease for many years.

The nature and frequency of the flares determine the severity of the disease. There is a wide variation in the clinical features, and the clinical picture is different for different people. Management depends on the systems involved, and the severity of the symptoms.

Low activity disease

This is manifested by a mild rash, fatigue, photosensitivity to ultraviolet light and flitting joint pain (arthralgia).

• Treatment with NSAID (non- steroidal anti- inflammatory) or paracetamol may be sufficient to control joint pain.
• Sunscreen to prevent sunlight affecting the skin. The protection factor should be at least 30.

More Severe Symptoms

• If these simple measures are not effective in controlling symptoms (arthralgia, fatigue and rashes), the anti-malarial Hydroxdychloroquine at a dose of 400mg per day should be used, for 6 months, reducing to 200mg per day maintenance dose. If necessary, the dose can be increased to 400mg per day again.
• A yearly review by an Optician is advised if the person is maintained on a higher dose (as there is a cumulative effect over many years).

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• Visual Impairment

• For those whose main symptoms are related to the anti-phospholipid syndrome, low - dose aspirin at 75mg per day is used.

In the case of those who have had previous thrombosis (venous or arterial clots) or infarcts (death of tissue) lifelong anticoagulants (warfarin) may be needed, and can be used in fairly high doses.

More active disease (not controlled by the above simpler measures)

• Immunosuppressive drugs are likely to be used. Immuno-suppressive drugs such as Azathioprine or Cyclophosphamide are sometimes used in conjunction with steroids, generally in severe and life-threatening disease such as that affecting he kidneys and central nervous system. Short-term intra-muscular (IM) or intravenous (IV) steroids at a dose of 500mg to 1 gram are injected while new drugs are beginning to work.
• Some people have low activity of the disease, but may require low-dose steroids to keep it under control. The dose would be 5mg to 7.5mg orally per day. Higher doses may be required during an acute flare.
• A new generation of biological treatments are being tested and may become important in the management of more severe cases.

SLE with renal involvement

Mild to Moderately Active Renal Disease:

• Steroids
• Azathioprine
• Mycophenolate and Mofetil

Severe Disease:

• Intravenous Cyclophosphamide, (monthly for 6 months) then follow on
• oral Azathioprine or MMF. Treatment may vary with the practice in a area
• Blood pressure control in the form of a diuretic, ACE Inhibitor and/or

SLE without renal involvement

Minor rash and/or Arthralgia:

• Non steroidal anti-inflammatory drugs (NSAID’s)
• Anti-malarial drugs
• Topical steroids

Arthritis, Pleuritis and Pericarditis:

• NSAID’s
• Anti-malarial drugs
• Low dose steroids (If non-responsive)
• Steroids increased to 20-30mg per day (If still non-responsive)
• Add Azathioprine (occasionally Cyclophosphamide)

Haematological (severe haemolytic anaemia):

• Prednisolone 60-80mg per day plus either azathioprine or cyclophosphamide (intravenously or orally)

SLE with central nervous system involvement

• Usually give steroids at around 30mg per day plus relevant anti-psychotic and anti-epileptic medication.
• If there is little response, double the steroids and add azathioprine and intravenous cyclophosphamide.

Note:

• The disease and its response to treatment should be monitored closely. Once an acute episode is controlled, the dose of steroid should be gradually reduced and stopped if possible to keep side effects to a minimum.
• Blood pressure should be controlled in all lupus patients, and annual cardiovascular review should be undertaken.
• Avoid oestrogen hormones such as in the contraceptive pill; use low- dose preparations with caution. (The POP [progesterone- only pill] is safe to use in conjunction with a barrier method such as condoms.
• Avoid exposure to sunlight.
• Avoid infections (especially when using immunosuppressive drugs). Infections can carry a high risk of mortality in SLE.

Amended November 2008