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Systemic Lupus Erythematous

Checked: 23-09-2023 by Rob Adams Next Review: 23-09-2025

Overview

Systemic lupus erythematosus (SLE) is a multisystem, autoimmune disease, involving complex pathogenetic mechanisms that can present at any age. It most commonly presents in women in the reproductive age group although SLE is increasingly recognised after the age of 40 particularly in Europeans.(1)

The disease is prone to relapses and remissions, resulting in considerable morbidity due to flares of disease activity and accumulated damage, with an increased risk of premature death mostly due to infection or cardiovascular disease.(1)

Lupus erythematosus describes the typical rash of SLE and the term systemic emphasises the potential for multi-organ involvement. Discoid lupus and other forms of cutaneous lupus can occur in the absence of any systemic features.(1,2)

The cause of SLE is unknown.

Who to Refer

Consider investigation of patients with symptoms that raise the suspicion of SLE and then refer if at least one relevant immunological abnormality.

A list of symptoms is available on Patient.info - see Systemic Lupus Erythematosus. Lupus| Patient for details.

Red Flags

Symptoms of SLE may mimic many other conditions including cancer so investigate red flag symptoms via 2WW pathways as indicated.

Before Referral

SLE is a multisystem autoimmune disorder. The diagnosis requires a combination of clinical features and the presence of at least one relevant immunological abnormality. (1)

If there is a clinical suspicion of lupus, blood tests including serological markers should be checked:

Bloods

FBC - mild normocytic anaemia is common. Leukopenia and thrombocytopenia may also be found.
Inflammatory markers - CRP may be normal unless co-existent inflammatory features . (ESR is usually raised but not routinely offered in local laboratories).
Anti-nuclear antibodies - ANA (search under Connective Tissue Disease on UHBW ICE and Anti-nuclear on NBT ICE) - present in about 95% of SLE patients. If the test is negative, there is a low clinical probability of the patient having SLE. A positive ANA occurs in approximately 5% of the adult population and alone has poor diagnostic value in the absence of clinical features of autoimmune rheumatic disease.
Anti-dsDNA antibodies (search under SLE monitoring on ICE) - highly predictive of SLE who have clinical features.
Antiphospholipid antibodies (Antiphospholipid profile on ICE).
Complement (C3 and C4) - often low in active SLE

Urinalysis - check for proteinuria and haematuria

Other tests

Other investigations will depend on system involvement and will usually be led by a specialist - eg, MRI brain scan, echocardiogram, renal biopsy.
Women with SLE are at greatly increased risk of premature atherosclerosis and the risk is independent of established cardiovascular risk factors. Monitoring for all cardiovascular risk factors is therefore essential.

Diagnostic uncertainty

Consider requesting Advice and Guidance via eRS if there is diagnostic uncertainty following initial investigations.

Referral

Rheumatology Referral

If SLE is suspected clinically and there are supporting investigations then refer to Rheumatology via eRS or request advice and guidance.,

Dermatology Referral

If Cutaneous Lupus Erythematous or DLE (2) is suspected then request Dermatology Advice and Guidance or refer via eRS.

Nephrology Referral

If renal involvment is suspected then consider referral to nephrology via eRS.

Resources

(1) Systemic Lupus Erythematosus. Lupus treatment; information | Patient

(2) Lupus erythematosus (pcds.org.uk)

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