Lupus Management Guidelines From the British Society for Rheumatology

Survival may be improving, but life expectancy is still poor compared with the general population.

The European League Against Rheumatism (EULAR) guidelines for lupus management lacked detail and were published in 2008.

The following recommendations are based on literature review supported by expert opinion.

The study group recommended that lupus nephritis (LN) management be guided according to the EULAR/European Renal Association-European Dialysis and Transplant Association recommendations for LN with their endorsement.

aCL, anticardiolipin; ANA, antinuclear antibody; GOR, grade of recommendation; LA, lupus anticoagulant; LOE, level of evidence; SOA, strength of agreement.

^a If there is clinical suspicion of lupus, blood tests (including serological marker tests) should be checked (LOE 2++, GOR B, SOA 98%).
^b A positive ANA test occurs in 5% of the adult population and as such is of little use without symptoms. ANAs are present in 95% of patients with SLE.
^c Anti-Ro/La and anti-RNP antibodies are less specific markers of SLE (2+/C), as they are found in other autoimmune rheumatic disorders as well as SLE (2+/C) (SOA 95%).
^d Confirmatory tests for antiphospholipid syndrome are positive LA, aCL (IgG, IgM), and/or anti-beta-2 glycoprotein-1 (IgG, IgM) on 2 occasions at least 12 weeks apart (2++/B) (SOA 97%).

GOR, grade of recommendation; LOE, level of evidence; SOA, strength of agreement.

^a In the case of disease activity, it is important to ascertain whether this is due to active inflammation or thrombosis, as this will define treatment strategies (LOE 2++, GOR B, SOA 97%).
^b Imaging (4/D), renal (2++/B) and other biopsies (4/D) should be performed where indicated (SOA 100%).
^c Mild disease activity is clinically stable lupus with no life-threatening organ involvement, mainly manifesting as arthritis, mucocutaneous lesions, and mild pleuritis. Patients with moderate disease activity have more serious manifestations, and severe disease is defined as organ- or life-threatening (4/D) (SOA 93%).

aPLs, antiphospholipids; CRP, C-reactive protein; GOR, grade of recommendation; LOE, level of evidence; SOA, strength of agreement.

^a Patients with stable low disease activity or in remission can be reviewed less frequently: for example, every 6-12 months (4/D) (SOA 99%).

CHB, congenital heart block; SOA, strength of agreement.

^a Management of modifiable risk factors, including hypertension, dyslipidemia, diabetes, high BMI, and smoking, should be reviewed at baseline and at least annually (4/D) (SOA 98%).

HCQ, hydroxychloroquine; MTX, methotrexate; NSAID, nonsteroidal anti-inflammatory drug; SOA, strength of agreement; SPF, sun protection factor; UV, ultraviolet.

^a These drugs allow for the avoidance of or dose reduction of corticosteroids (SOA 94%).
^b Topical preparations may be used for cutaneous manifestations, and intra-articular injections for arthritis (4/D) (SOA 93%).
^c Patients must also be advised about sun avoidance and the use of protective clothing (4/D) (SOA 97%).

AZA, azathioprine; HCQ, hydroxychloroquine; IM, intramuscular; IV, intravenous; MMF, mycophenolate mofetil; MTX, methotrexate; SOA, strength of agreement.

^a Immunosuppressive agents are often required to control active disease and are steroid-sparing agents (2+/C). They can also reduce the risk of long-term damage accrual (4/D) (SOA 98%).

MMF, mycophenolate mofetil; SOA, strength of agreement.

^a Treatment is dependent on the underlying etiology (inflammatory and/or thrombotic), and patients should be treated accordingly with immunosuppression and/or anticoagulation, respectively (4/D) (SOA 98%).

HCQ, hydroxychloroquine.

^a The physician should be able to confirm the diagnosis, assess the level of disease activity, and provide advice on treatment and monitoring of the disease, its complications, and adverse effects of therapy.

Patients benefit from personalized advice, written information, and education about the disease and its treatment.