Very Late Onset Lupus Nephritis: Single-Centre Experience and A Review of the Literature

Abstract

Background: In the majority of lupus patients, lupus nephritis (LN) develops within the first five years of diagnosis. Here we evaluated patients treated in our department during the past 40 years distinguishing those with very early onset [within one year of diagnosis]; early onset [ two to five years post diagnosis]; medium-term onset [between six to ten years after diagnosis]; late onset [presenting six to 15 years after diagnosis] and very late onset LN [presenting 16 years or later after diagnosis]. Early onset [ two to five years post diagnosis]; medium-term onset [between six to ten years after diagnosis]; late onset [presenting six to 15 years after diagnosis] and very late onset LN [presenting 16 years or later after diagnosis].early onset [within two years of diagnosis]; medium-term onset [between two and five years after diagnosis]; late onset [presenting five to ten years after diagnosis] and very late onset LN [presenting ten years after diagnosis]. Aim: To compare the differences in demography, clinical data, serological profile and follow up of patients with LN of early, medium, late and very late onset. Methods: This was a retrospective study of 226 systemic lupus erythematosus (SLE) patients with biopsy proven nephritis. We focused on a comparison of their epidemiology, serology, clinical and follow-up data. Results: We identified 97 (43%) very early-onset patients; 69 (31%) early-onset; 28 (12%) medium onset; 22 (10%) late-onset and 10 (4%) very late-onset LN patients. Comparing those patients whose LN was delayed by> 10 years post their lupus diagnosis compared to those with LN onset <10 years we found that these patients were statistically significantly more likely to be Caucasian [p=0.04]; to have arthritis [p=0.001] and to be leucopaenic [p=0.004]. There were no other difference between the groups. Among the 10 patients with very late onset LN there was an increase trend towards them having higher ANA titer (>1:640) [p=0,044] and having biopsy-proven combined type of LN (III+V, IV+V) [p=0.034]. Conclusion: Although the majority of SLE patients who get LN do so within 10 years of diagnosis in our experience 14% [approximately 1:7] developed renal involvement 10 years or more after SLE diagnosis. The observation emphasis the need to be vigilant when caring for SLE patients long term.