The British Society for Rheumatology has issued a new U.K. guideline for the management of systemic lupus erythematosus (SLE), focusing on nearly all aspects of the disease.
From diagnosing, assessing, and managing common manifestations of nonrenal lupus, such as skin rashes and arthritis, through dealing with less common but potentially more serious problems such as kidney disease, the guideline aims to help everyone involved in the management of patients with SLE to give the best, evidenced-based care.
“As a result of this guideline, I would expect that patients will experience measurable improvements in care,” lead guideline author Caroline Gordon, MD , professor of rheumatology at the University of Birmingham (England), said in a press statement issued by her institution to coincide with the publication of the guidelines (Rheumatology [Oxford]. 2017 Oct 6. doi: 10.1093/rheumatology/kex286 ).
The British Society for Rheumatology’s (BSR) guideline is the first to specifically cover lupus management in the United Kingdom, and it builds on existing European League Against Rheumatism (EULAR) guidance published almost a decade ago ( Ann Rheum Dis. 2008;67:195-205 ), and more recent EULAR/European Renal Association–European Dialysis and Transplant Association ( Ann Rheum Dis. 2012;71:1771-82 ) and American College of Rheumatology ( Arthritis Care Res. 2012;64:797-808 ) recommendations on managing lupus nephritis (LN).
The BSR’s full guideline provides a summary of the EULAR/ERA-EDTA’s LN guidelines, and the degree to which their new guidelines concur.
As with all BSR guidelines, the recommendations have been developed by a multidisciplinary team. This included academic and consultant rheumatologists and nephrologists, rheumatology trainees, a primary care physician, a clinical nurse specialist, a patient representative, and a lay member. This should make the guideline relevant to anyone who may come across someone with SLE, including primary care physicians, dermatologists, and emergency medicine practitioners.
“These recommendations are based on the literature review covering the diagnosis, assessment, monitoring, and treatment of mild, moderate, and severe lupus, including neuropsychiatric disease,” the guideline authors stated. They noted that the reason they looked only at nonrenal disease was because the EULAR/ERA-EDTA recommendations for LN have been published close to the time that work was started on the guideline. Each of the recommendations the multidisciplinary team devised was carefully graded and the degree to which members of the team agreed with each recommendation was calculated.
One of the key recommendations regarding the diagnosis of SLE is that a combination of clinical features and at least one relevant immunologic irregularity needs to be present. Blood tests, including serologic marker tests, should be performed if there is clinical suspicion of lupus.
Another recommendation on diagnosis is that if antinuclear antibodies are absent, then it is unlikely that the patient has lupus. This is because around 95% of SLE patients will test positive for antinuclear antibodies. Antiphospholipid antibodies should be tested in all patients with lupus at baseline, according to the guideline.
“Patients with SLE should be monitored on a regular basis for disease manifestations, drug toxicities, and comorbidities,” Dr. Gordon and her associates advised in one of the recommendations on monitoring patients. In another, they wrote that those with active disease need reviewing at least every 1-3 months, which should include evaluation of patients’ blood pressure, urine, renal function, anti-dsDNA antibodies, complement, a full blood count, and liver function tests.
It is also important to monitor patients for the presence of antiphospholipid antibodies, which are associated with thrombotic events, and it is always important to be on the lookout for comorbidities such as atherosclerotic disease and manage modifiable risk factors such as hypertension. The guideline does not go into detail about managing all of the potential complications of lupus, however, as these are covered by other national guidelines.
Guidance on treatment is separated into how to treat patients with mild, moderate, and severe SLE. The guideline does not cover topical or systemic treatment for isolated cutaneous lupus, nor does it look at how to manage pediatric patients, the authors noted. General guidance is given on how to treat patients, and specific dosing regimens are beyond the scope of the guidelines.
The recommendations encourage the use of a variety of treatments to try to ensure less reliance on the use of steroids to control symptoms. The guideline authors noted that only hydroxychloroquine, corticosteroids, and belimumab are currently licensed treatments for lupus in the United Kingdom.
For mild disease, the disease-modifying antirheumatic drugs hydroxychloroquine and methotrexate are suggested, as are nonsteroidal anti-inflammatory drugs. If prednisolone is used, then it should be in low doses (7.5 mg or less per day). Patients should be encouraged to use sunscreen and sun avoidance to protect them against ultraviolet-induced skin lesions.
For moderate disease, higher steroid doses may be needed and immunosuppressives might be warranted, and in refractory cases, monoclonal antibody treatment may be necessary.
For severe disease, thorough investigation is essential to exclude other possible causes of any renal or neuropsychiatric manifestations. Immunosuppressive treatment is recommended, with biologic therapies considered on a case-by-case basis. Intravenous immunoglobulin and plasmapheresis may also be an option in certain patients.
Key standards of care
As general standards of care, Dr. Gordon and her coauthors wrote that “lupus patients should be referred to a physician with experience in managing lupus who can confirm the diagnosis, assess the level of disease activity, and provide advice on treatment and monitoring of the disease, its complications, and side effects of therapy.”
Furthermore, a multidisciplinary team approach is needed, and if a treatment is not helping get patients into a state of low disease activity within an expected time frame, “it is important to consider adherence to treatment and adjusting the therapy to reduce the accumulation of chronic damage.” It is also recommended that patients receive personalized advice, written information, and education about the disease and its drug treatment.
No specific funding was received from any organizations in the public, commercial, or not-for-profit sectors to carry out the work described in the guideline.
Dr. Gordon has undertaken consultancies and received honoraria from Bristol-Myers Squibb, Eli Lilly, GlaxoSmithKline, MedImmune, Merck Serono, Parexel, Roche, and UCB. She has been a member of the speakers bureau for GlaxoSmithKline, UCB, and Eli Lilly. She also has received research grant support from Aspreva/Vifor Pharma in the past and currently receives it from UCB.