New Clinical Practice Guidelines, November 2017

John Anello; Brian Feinberg; Richard Lindsey; Cristina Wojdylo; Olivia Wong, DO; Yonah Korngold; John Heinegg; Sam Shlomo Spaeth

Disclosures

November 08, 2017

In This Article

Systemic Lupus Erythematosus

British Society for Rheumatology

Any sign of renal involvement—in particular, urinary findings such as reproducible proteinuria ≥0.5 g/24 hr, especially with glomerular hematuria and/or cellular casts—should be an indication for renal biopsy. Renal biopsy is indispensable, since, in most cases, clinical, serologic and laboratory tests cannot accurately predict renal biopsy findings. 

Treatment should aim for complete renal response with UPCR (urine protein:creatinine ratio) <50 mg/mol and normal or near-normal (within 10% of normal GFR if previously abnormal) renal function. Partial renal response, defined as ≥50% reduction in proteinuria to subnephrotic levels and normal or near-normal renal function, should be achieved preferably by 6 months but no later than 12 months following initiation of treatment. 

For patients with class IIIA or IIIA/C and class IVA or IVA/C lupus nephritis (LN), mycophenolic acid (MPA) (mycophenolate mofetil [MMF] target dose: 3 g/day for 6 months, or MPA sodium at equivalent dose) or low-dose IV cyclophosphamide (CYC) (total dose 3 g over 3 months), in combination with glucocorticoids, are recommended as initial treatment, as they have the best efficacy/toxicity ratio. 

To increase efficacy and reduce cumulative glucocorticoid doses, treatment regimens should be combined initially with 3 consecutive pulses of IV methylprednisolone 500-750 mg, followed by oral prednisone 0.5 mg/kg/day for 4 weeks, reducing to ≤10 mg/day by 4-6 months.

In pure class V nephritis with nephrotic-range proteinuria, MPA (MMF target dose 3 g/day for 6 months) in combination with oral prednisone (0.5 mg/kg/day) may be used as initial treatment based on better efficacy/toxicity ratio. CYC or calcineurin inhibitors (cyclosporine, tacrolimus) or rituximab are recommended as alternative options or for nonresponders. 

For patients who fail treatment with MPA or CYC, either because of lack of effect (as defined above) or due to adverse events, it is recommended that the treatment be switched from MPA to CYC, or CYC to MPA, or that rituximab be given. 

Antiphospholipid antibodies (aPLs) should be tested in all lupus patients at baseline, especially in those with an adverse pregnancy history or arterial/venous thrombotic events. Confirmatory tests for antiphospholipid antibody syndrome (APS) are positive lupus anticoagulants (LA), anticardiolipin (aCL) (IgG, IgM), and/or anti-beta-2 glycoprotein-1 (IgG, IgM) on 2 occasions at least 12 weeks apart.

Those with active disease should be reviewed at least every 1 to 3 months, with blood pressure, urinalysis, renal function, anti-dsDNA (double-stranded DNA) antibodies, complement levels, CRP, full blood count, and liver function tests forming part of the assessment, and further tests as necessary. Patients with stable low disease activity or in remission can be reviewed less frequently.

Treatments to be considered for the management of mild non–organ-threatening disease include the disease-modifying drugs hydroxychloroquine (HCQ) and methotrexate (MTX), and short courses of NSAIDs for symptomatic control. These drugs allow for the avoidance of or dose reduction of corticosteroids. Prednisolone treatment at a low dose of ≤7.5 mg/day may be required for maintenance therapy. Topical preparations may be used for cutaneous manifestations, and intra-articular injections for arthritis.

The management of moderate SLE involves higher doses of prednisolone (up to 0.5 mg/kg/day), or the use of IM or IV doses of methylprednisolone. Immunosuppressive agents are often required to control active disease and are steroid-sparing agents. They can also reduce the risk of long-term damage accrual.

MTX, azathioprine (AZA), MMF, cyclosporine, and other calcineurin inhibitors should be considered in cases of arthritis, cutaneous disease, serositis, vasculitis, or cytopenias if HCQ is insufficient.

For refractory cases, belimumab or rituximab may be considered.

Patients who present with severe SLE, including renal and NP manifestations, need thorough investigation to exclude other etiologies, including infection. Treatment is dependent on the underlying etiology (inflammatory and/or thrombotic), and patients should be treated accordingly with immunosuppression and/or anticoagulation, respectively.

Immunosuppressive regimens for severe active SLE involve IV MP or high-dose oral prednisolone (up to 1 mg/kg/day) to induce remission, either on their own or more often as part of a treatment protocol with another immunosuppressive drug.

MMF or CYC is used for most cases of LN and for refractory, severe nonrenal disease.

Biologic therapies belimumab or rituximab may be considered, on a case-by-case basis, where patients have failed to respond to other immunosuppressive drugs, due to inefficacy or intolerance.

IVIG and plasmapheresis may be considered in patients with refractory cytopenias, thrombotic thrombocytopenic purpura (TTP), rapidly deteriorating acute confusional state, and the catastrophic variant of APS.

References

  • Kelly JC. New SLE guideline aims to improve diagnosis, reduce steroids. Medscape News. WebMD Inc. October 17, 2017.

  • 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.

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