Systemic lupus erythematosus, lupus nephritis and end-stage renal disease: a pragmatic review mapping disease severity and progression

Abstract

Objective: The understanding of systemic lupus erythematosus (SLE) and lupus nephritis (LN) pathogenesis remains incomplete. This review assessed LN development in SLE, within-LN progression and progression to end-stage renal disease (ESRD).

Methods: A keyword-based literature search was conducted, and 26 publications were included.

Results: Overall, 7-31% of patients had LN at SLE diagnosis; 31-48% developed LN after SLE diagnosis, most within 5 years. Class IV was the most commonly found LN class and had the worst prognosis. Histological transformation occurred in 40-76% of patients, more frequently from non-proliferative rather than proliferative lesions. Cumulative 5- and 10-year ESRD incidences in patients with SLE were 3% and 4%, respectively, and 3-11% and 6-19%, respectively, in patients with SLE and LN.

Conclusions: Elevated serum creatinine was identified as a predictor of worsening disease state, and progression within LN classes and from SLE/LN to ESRD. This review highlights the substantial risk for developing LN and progressing to ESRD amongst patients with SLE.

Keywords: Nephritis; renal lupus; systemic lupus erythematosus.

Figure 1.

Proportion of patients (a) with SLE who were diagnosed with LN at SLE diagnosis, (b) with SLE who developed LN at any time during follow-up and (c) with LN who developed ESRD. ESRD: end-stage renal disease; LN: lupus nephritis; SLE: systemic lupus erythematosus.

Figure 2.

WHO/ISN/RPS histological class distribution (%*) across studies at (a) initial biopsy and (b) repeat biopsy. WHO classification (class I = normal kidney; class II = mesangial glomerulonephritis; class III = focal segmental proliferative glomerulonephritis; class IV = diffuse proliferative glomerulonephritis; class V = membranous glomerulonephritis; class VI = chronic sclerosing glomerulonephritis); ISN/RPS classification (class I = minimal mesangial LN; class II = mesangial proliferative LN; class III = focal LN; class IV = diffuse LN; class V = membranous LN; class VI = advanced sclerosis LN; ‘other’ ISN/RPS categories include: class III (A) = active lesions: focal proliferative LN; class III (A/C) = active and chronic lesions: focal proliferative and sclerosing LN; class III (C) = chronic inactive lesions with glomerular scars: focal sclerosing LN; class IV-S (A) = active lesions: diffuse segmental proliferative LN; class IV-G (A) = active lesions: diffuse global proliferative LN; class IV-S (A/C) = active and chronic lesions: diffuse segmental proliferative and sclerosing LN; class IV-G (A/C) = active and chronic lesions: diffuse global proliferative and sclerosing LN; class IV-S (C) = chronic inactive lesions with scars: diffuse segmental sclerosing LN; class IV-G (C) = chronic inactive lesions with scars: diffuse global sclerosing LN). *Percentages calculated based on the number of patients with available data. ISN/RPS: International Society of Nephrology/Renal Pathology Society; LN: lupus nephritis; WHO: World Health Organization.

Figure 3.

Onset of renal involvement within each WHO classification (%). WHO: World Health Organization.

Figure 4.

Mapping of disease progression from SLE to LN and ESRD. Dx: diagnosis; ESRD: end-stage renal disease; LN: lupus nephritis; SLE: systemic lupus erythematosus. Figure reproduced under the terms of the Creative Commons CC-BY-NC licence from Justyna Amelio, Kerry Gairy, Anadi Mahajan, Gavneet Kaur, Damon Bass, Roger Abramino Levy, David Roth. Mapping disease severity and progression of renal involvement in patients with systemic lupus erythematosus (Abstract 87). Lupus Sci Med 2019; 6: A63.