Pertinent points from the British Society for Rheumatology Lupus guideline
Article kindly written by Dr. Louise Warburton, Co-president of The Primary Care Rheumatology and Musculoskeletal Medicine Society (PCRMM), plus The British Society for Rheumatology
The new guideline builds on the first guideline which was published in 2017. There are newer pharmaceutical treatments for lupus now, and a guideline review was necessary.
The guideline was developed by a multi-disciplinary team of adult and paediatric rheumatologists, general practitioners, podiatrist, pharmacists and patients, and a rigorous evidence search was undertaken.
The guideline acknowledges that systemic lupus erythematosis (SLE) is a complex, lifelong disease which causes inflammation and subsequent damage to multiple organs.
15-20% of people develop SLE in childhood.
In adulthood, females are nine times more likely to be affected than males, with peak incidence in the reproductive years. Approximately 1 in 1000 adults in the UK have SLE.
Globally, SLE is more common in South Asians, East Asian and other non-white groups.
People of Black African ancestry, East Asian, South Asian and Hispanic have more severe disease and damage accumulation. In the UK, SLE is most frequently observed in people of Black African and South Asian ancestry, thus prevalence across regions of the UK varies in line with these demographics.
Diagnosis
The guideline poses the question; what clinical and serological features should prompt consideration of a diagnosis of SLE in primary or secondary care?
The guideline admits that diagnosis can be challenging as SLE can present in a variety of ways and early symptoms can mimic other medical conditions. Also, some of the cardinal features are not present at the time of presentation and develop over time.
A survey by Lupus UK reported that mean time from developing initial symptoms to reaching a diagnosis of SLE was 6.4 years and that 47% of respondents were originally misdiagnosed. A delay in SLE diagnosis can lead to increased disease activity, organ damage, and significantly impair quality of life and long-term health outcomes.
Studies have shown that the rates of GP consultations and healthcare resources utilised in the community by people with an ultimate diagnosis of SLE increased exponentially in the years leading to diagnosis. So, all clinicians working in Primary Care and possibly coming across new presentations of SLE, should maintain a high level of suspicion.
The guideline asks Primary Care clinicians to make timely referrals to specialist care once the disease is suspected, and to include in the referral detailed accounts of symptoms and investigations, to allow effective triage.
The guideline makes the recommendations;
- People suspected to have SLE should be referred to secondary or tertiary care for diagnosis. Those with a strong suspicion of a new diagnosis of SLE should be prioritised as a 3-week wait target.
- SLE should be diagnosed by clinicians experienced in its management and requires a combination of clinical, laboratory, and immunological features attributable to SLE.
Investigations in Primary Care
Anti-nuclear antibody (ANA) is one of the diagnostic serological markers of the disease but confusingly is falsely positive in many other diseases and in older people. 5-10% of healthy adults have a positive test for ANA.
The actual classification criteria for SLE includes several other serological markers such as
Anti-dsDNA, positive extractable nuclear antigens such as anti-Sm and positive anti-phospholipid antibody, low complement levels. Specialists will use the results of a battery of tests including these mentioned, to diagnose SLE or raise a suspicion of SLE in ambiguous cases.
So, in Primary Care, we are most likely to be requesting ANA antibodies and the guideline suggests that:
- Antinuclear antibody (ANA) test should only be performed where there is reasonable clinical suspicion of SLE or related diseases. ANAs are present in 95-99% of people with SLE, so a negative test makes SLE unlikely (results can fluctuate so a negative test does not rule out SLE where there have been past positives.
Some children and adolescents may be ANA-negative at the time of diagnosis. If the ANA is positive and there are suggestive clinical features, assessment of the other serological markers could be undertaken in Primary Care; these include:
- ANA test should be supplemented by tests for anti-dsDNA antibodies, extractable nuclear antigen antibodies and C3/C4 complement levels since the presence of anti-dsDNA antibodies, low complement levels , and/or anti-Smith (Sm) antibody is highly supportive of SLE diagnosis in people with relevant clinical symptoms.
Assessment: what is the best way to assess people with SLE?
- Clinical manifestations in people with SLE should be assessed to determine if they are due to disease activity, damage, drug toxicity, infection, thrombosis or the presence of comorbidities.
- People with SLE should be assessed for manifestations in all systems and relevant clinical examination and investigations performed.
So, for the initial assessment and referral in Primary Care, a full clinical examination should be undertaken and blood pressure and urinalysis performed, as blood or protein in the urine may indicate lupus nephritis and the need for more urgent specialist assessment.
Monitoring for drug toxicities and damage
The BSR suggests that monitoring for drug toxicities should be a collaborative effort between Primary and secondary care, using shared-care agreements. These have been difficult to establish in some areas due to lack of funding for Primary Care Clinicians to undertake the monitoring, and the very specialist nature of some of these medications.
The guideline is keen to discuss a ‘Treat to Target ‘approach to minimise complications developing and organ damage occurring, which can result in poor outcomes for the patients.The aim is to achieve disease remission.
Non-pharmaceutical approaches
There are non-pharmaceutical approaches to treatment for SLE and the multi-disciplinary team of doctors, nurses, occupational therapists, pharmacists, AHPS and physiotherapists, psychologists, should all be involved in supporting patients. The guideline recommends offering education and self-management support.
Sun protection
People with SLE are sensitive to UV light and this can exacerbate skin rashes and even cause a flare of the disease. Therefore, sun protection is strongly advised.
The Guideline recommends a high sun-protection factor application (at least factor 30) with broad-spectrum UV-A and UV-B protection and this advice should be re-enforced in Primary Care.
Physical Activity
Physical Activity is generally beneficial for all people, but there are considerations for those with SLE due to disease activity; the guideline recommends assessing disease status before prescribing exercise. This is to allow modification of exercise recommendations during flare of the disease.
Diet
No specific diet was recommended, but a daily vitamin D supplement was thought sensible at a dose of 10 micrograms of vitamin D3.
People with SLE are at increased risk of cardiovascular disease, therefore assessment and management of modifiable risk factors such as hypertension and diabetic risk and lipid profile is recommended. The guideline recommends annual assessment of these risk factors, and some of this management will occur in Primary care as the skills to undertake this lie within Primary Care.
Vaccination
Also, people with SLE should receive eligible vaccinations, as infection is a leading cause of death in SLE, therefore vaccination should be encouraged.
Vaccines could include seasonal influenzae, pneumococcal, tetanus, respiratory syncytial virus (RSV), varicella zoster, herpes zoster, human papilloma virus (HPV), measles, mumps and rubella (MMR) and COVID-19.
Bone Health
People with SLE are at risk of osteoporosis, with an approximately double risk to people of the same sex and age. The guideline recommends an annual assessment of bone health for post-menopausal women and men aged over 50 years. This would be using either the FRAX tool or Q-risk and measurement of bone mineral density when indicated.
Summary
The BSR has produced a very useful and wide-reaching guideline on the diagnosis and support of people living with SLE and I am sure, will be adopted by Primary Care team.