In the US, approximately 35% of adults with systemic lupus erythematosus (SLE) have clinical evidence of nephritis at the time of diagnosis, with an estimated total of 50–60% developing nephritis during the first 10 years of disease (1–4). The prevalence of nephritis is significantly higher in African Americans and Hispanics than in whites, and is higher in men than in women. Renal damage is more likely to develop in nonwhite groups (1–4). Overall survival in patients with SLE is approximately 95% at 5 years after diagnosis and 92% at 10 years after diagnosis (5,6). The presence of lupus nephritis (LN) significantly reduces survival to approximately 88% at 10 years, with even lower survival in African Americans (5,6).
The American College of Rheumatology (ACR) last published guidelines for management of SLE in 1999 (7). That publication was designed primarily for education of primary care physicians and recommended therapeutic and management approaches for many manifestations of SLE. Recommendations for management of LN consisted of pulse glucocorticoids followed by high-dose daily glucocorticoids in addition to an immunosuppressive medication, with cyclophosphamide (CYC) viewed as the most effective immunosuppressive medication for diffuse proliferative glomerulonephritis. Mycophenolate mofetil (MMF) was not yet in use for LN and was not mentioned. Since that time, many clinical trials of glucocorticoids plus immunosuppressive interventions have been published, some of which are high-quality prospective trials, and some that are not only prospective but also randomized. Therefore, the ACR determined that a new set of management recommendations was in order. A combination of an extensive literature review and the opinions of highly-qualified experts, including rheumatologists, nephrologists, and pathologists, has been used to reach the recommendations. The management strategies discussed here apply to LN in adults, particularly to those receiving care in the US, and include interventions that were available in the US as of February 2012.
While these recommendations were developed using rigorous methodology, guidelines do have inherent limitations in informing individual patient care; hence, the selection of the term “recommendations.” While they should not supplant clinical judgment or limit clinical judgment, they do provide expert advice to the practicing physician managing patients with LN.