Clinicopathological characteristics and long-term outcomes of adult patients with proliferative lupus nephritis.

BACKGROUND

Proliferative lupus nephritis (PLN) is the most severe form of lupus nephritis (LN). There are limited data available on renal outcomes of PLN from developing countries.

AIM: To determine the clinicopathological characteristics and long-term outcomes in terms of remission, requirement of kidney replacement therapy (KRT), and patient survival.

METHODS

A retrospective analysis was conducted on biopsy-proven focal or diffuse PLN cases diagnosed between 1998 and 2019 at the Sindh Institute of Urology and Transplantation and followed up at the renal clinic for a minimum of 5 years. All patients were induced with a combination of intravenous cyclophosphamide and corticosteroids for 6 months, followed by maintenance treatment with azathioprine (AZA) or mycophenolate mofetil (MMF). Data were analyzed using Statistical Package for the Social Sciences, version 22.0. P ≤ 0.05 was considered statistically significant.

RESULTS

The mean age at the onset of systemic lupus erythematosus was 24.12 years ± 8.89 years, and at LN onset, 26.63 years ± 8.61 years. There was a female predominance of 184 (88.9%) cases. Among baseline characteristics, reduced estimated glomerular filtration rate, presence of hypertension, requirement of KRT, and underlying renal histology (International Society of Nephrology/Renal Pathology Society class IV than class III) were significantly associated with end-stage kidney disease (ESKD) and mortality. The renal outcomes were negatively correlated with age, duration of symptoms, and 24-hour urinary protein excretion. The overall remission rate was 89.8% at the end of induction therapy. At 5 years, 141 (68.11%) patients were in complete and partial remission (94 [45.4%] and 47 [22.7%], respectively). In total, 19 (9.2%) patients required KRT on presentation, and at 5 years, 38 (18.4%) patients developed ESKD, and 28 (13.5%) patients died. Thirty-four (16.4%) patients had a renal relapse, more with AZA than MMF (30 [88.2%] vs 4 [11.76%], respectively; P = 0.04). Renal survival at 6 months was 89.8%, while at 5 years, it was 68.11%, showing a significant improvement in patients who did not need KRT at the time of presentation (P < 0.0001).

CONCLUSION

Baseline renal functions, requirement of KRT, and diffuse proliferative disease were the most relevant prognostic factors for kidney survival among this cohort. Short-term renal outcomes were good. Long-term outcomes were poorer with AZA-based maintenance therapy than with MMF, with more ESKD and mortality.