First UK Guidelines for Adults with Lupus

November 7, 2017
Mariah Zebrowski Leach, JD, MS

Up-to-date guidelines were developed to help improve patient outcomes.

The first UK guidelines on the care of adults with systemic lupus erythematosus (SLE) were published recently by the British Society for Rheumatology.

Because general recommendations for the management of SLE have not been published since 2008, up-to-date guidelines were warranted to help improve patient outcomes, noted the multidisciplinary group that developed them.

The group included academic and NHS consultants in rheumatology and nephrology, rheumatology trainees, a general practitioner, a clinical nurse specialist, a patient representative, and a lay member. The opinions of other key stakeholders, such as podiatrists, nurse specialists, and representatives of Lupus UK, were also solicited.

The guidelines were published in Rheumatology.

The recommendations are based on an extensive literature review (to June 2015) of non-renal SLE, supplemented as necessary by UK expert opinion and consensus agreement. The finalized guidelines have been accredited by the National Institute for Health and Care Excellence. The primary objective was to provide comprehensive, evidence-based recommendations that cover diagnosis, assessment, monitoring, and treatment of mild, moderate, and severe active SLE. 

Recommendations for diagnosis

• Diagnosis requires the presence of at least 1 relevant immunological abnormality. Blood tests, including serological marker tests, should be checked.
• If the ANA test is negative, the clinical probability of SLE is low. 
• Anti-dsDNA antibodies, low complement levels, and anti-Smith antibodies are highly predictive of SLE.
• aPLs should be tested at baseline in all patients who have SLE, especially those with an adverse pregnancy history or arterial/venous thrombotic events.

Recommendations for assessment

• Ascertaining whether clinical manifestations are the result of active inflammation or thrombosis is important, because this will define treatment strategies.
• Clinical assessment should include a thorough history and review of systems, full clinical examination and monitoring of vital signs, urinalysis, laboratory tests, assessment of health status and quality of life, and measurement of disease activity and damage using standardized SLE assessment tools. When indicated, imaging, renal, and other biopsies should be performed.
• Mild disease activity is clinically stable, with no life-threatening organ involvement; moderate disease activity has more serious manifestations; and severe disease activity is defined as organ- or life-threatening.

Recommendations for monitoring

• Patients with SLE should be monitored on a regular basis for disease manifestations, drug toxicity, and comorbidities.
• Patients with active disease should be reviewed at least every 1 to 3 months, including blood pressure, urinalysis, renal function, anti-dsDNA antibodies, complement levels, C-reactive protein level, full blood count, liver function tests and, as necessary, further tests. Patients with stable low disease activity or in remission can be monitored every 6 to 12 months.
• aPLs should be re-evaluated before pregnancy or surgery or in the presence of a new severe manifestation or vascular event.
• Anti-Ro and anti-La antibodies are associated with neonatal lupus and should be checked before pregnancy.
• Management of modifiable risk factors for comorbidities should be reviewed at baseline and at least annually.
• Immunosuppressive therapy should be monitored closely with regular laboratory tests and clinical assessment.

Recommendations for mild SLE management

• Treatments to be considered include the disease-modifying antirheumatic drugs hydroxychloroquine (HCQ) and methotrexate (MTX) and a short course of NSAIDs for symptomatic control.
• Prednisolone treatment at a low dose (less than 7.5 mg/d) may be required for maintenance therapy. Topical preparations may be used for cutaneous manifestations and intra-articular injections for arthritis.
• Also important are high sun protection factor UV-A and UV-B sunscreen, sun avoidance, and the use of protective clothing.

Recommendations for moderate SLE management

• Management involves higher doses of prednisolone (up to .5 mg/kg/d) or the use of IM or IV doses of methylprednisolone. Immunosuppressive agents are often required to control active disease.
• MTX, azathioprine, mycophenolate mofetil (MMF), cyclosporine (CYC), and other calcineurin inhibitors should be considered in cases of arthritis, cutaneous disease, serositis, vasculitis, or cytopaenias if HCQ is insufficient.
• For refractory cases, belimumab or rituximab may be considered.

Recommendations for severe SLE management

• Patients need thorough investigation to exclude other etiologies, including infection. Treatment depends on the underlying etiology (inflammatory or thrombotic or both), and patients should be treated accordingly with immunosuppression or anticoagulation or both.
• Immunosuppressive regimens for severe active SLE involve IV methylprednisolone or high-dose oral prednisolone (up to 1 mg/kg/d) to induce remission, either on their own or more often as part of a treatment protocol with another immunosuppressive drug.
• MMF and CYC are used for most cases of lupus nephritis and for refractory severe nonrenal disease.
• The biologic therapies belimumab and rituximab may be considered when patients have not responded to other immunosuppressive drugs.
• IVIG and plasmapheresis may be considered in patients who have refractory cytopenia, thrombotic thrombocytopenic purpura, rapidly deteriorating acute confusional state, or the catastrophic variant of APS.

References:

Gordon C, Amissah-Arthur MB, Gayed M, et al. “The British Society for Rheumatology guideline for the management of systemic lupus erythematosus in adults.” Rheumatology (Oxford). 2017 Oct 6. doi: 10.1093/rheumatology/kex286. [Epub ahead of print]
Gordon C, Amissah-Arthur MB, Gayed M, et al. “The British Society for Rheumatology guideline for the management of systemic lupus erythematosus in adults: Executive Summary.” Rheumatology (Oxford). 2017 Oct 6. doi: 10.1093/rheumatology/kex291. [Epub ahead of print]