Very delayed lupus nephritis: a report of three cases and literature review

Abstract

Lupus nephritis (LN) affects up to 50% of patients with Systemic Lupus Erythematosus (SLE) and is associated with a worse prognosis. LN usually develops within the first 5 years of the onset of the disease. We report three patients with very delayed LN (DLN) diagnosed after 15 or more years after SLE diagnosis. The three patients were non-Caucasian women with adolescent or adult-onset SLE. Each had antinuclear, anti-dsDNA and anti-Ro antibodies. Hydroxychloroquine was prescribed for each. Their disease courses were characterised by sporadic non-renal flares controlled by steroids and, in two cases, by one cycle of rituximab. Unexpectedly, they developed proteinuria, haematuria and lowering of estimated glomerular filtration rate with clinical signs of renal disease. LN was confirmed by renal biopsy. Reviewing them, each showed serological signs of increasing disease activity (rising levels of anti-dsDNA antibodies and fall in C3) that predated clinical or laboratory signs of LN by 1–3 years. Reviewing the literature, we found a lack of knowledge about DLN starting more than 15 years after SLE diagnosis. With the increasing life expectancy of patients with SLE it is likely that more cases of very DLN will emerge.

Comparison of clinical features and outcomes between patients with early and delayed lupus nephritis

“…The results of this study showed that lupus nephritis affected more than 60% of patients at the time of SLE diagnosis; however, a substantial number of patients developed lupus nephritis after SLE was diagnosed. In this study, the median disease duration of SLE in the delayed lupus nephritis group was < 5 years, which is consistent with data of previous studies [ 8 ]. Regarding the clinical characteristics, when lupus nephritis developed early in the course of SLE, affected patients were more likely to have higher disease activity and prominent multiple organ involvement.…”

“…In general, lupus nephritis primarily occurs at the time of or within the first year of SLE diagnosis [ 7 ]. On the contrary, the frequency of lupus nephritis occurrence gradually decreases with time; the onset of lupus nephritis is reported to be uncommon after 5 years from SLE diagnosis [ 8 ].…”

Lupus Nephritis With Collapsing Glomerulopathy: A Rare Association

“…Lupus nephritis is a common and serious complication in patients with systemic lupus erythematosus (SLE), developing in almost 50% of cases of SLE [ 1 , 2 ]. In the majority of patients, LN develops within 5 years of diagnosis of SLE [ 3 ].…”