Abstract
Despite current advances in treatment, refractory lupus nephritis (RLN) continues to pose a challenge. The present paper studies the clinical profile and treatment outcomes in patients with RLN. This observational, bidirectional study enrolled consecutive lupus nephritis (LN) patients from August 2018 to January 2019, who either failed to improve within three months, did not achieve partial renal response (PR) at six months, or did not achieve complete renal response (CR) after two years of treatment. Patients were followed every three months; treatment details and outcomes [CR, PR, no renal response (NR)], doubling serum creatinine, and death were recorded. Group comparisons were made using ANOVA and chi-square test. Factors affecting renal response were studied using linear regression. Forty-five of forty-eight enrolled patients completed at least nine months of follow-up and were included in outcome analysis. The median (IQR) SLE duration was three years (2-6 years). The majority of patients (n = 25) had proliferative LN (ISN/RPS class III/IV), with nine patients having pure membranous LN (class V). The mean activity and chronicity indices were 8 and 0. Over a median (IQR) follow-up period of 15 (12-27) months, 28 had CR, 9 had PR, and 8 showed no response to a switch in an immunosuppressive (IS) agent. Repeat renal biopsy (n = 8) with a mean (±SD) biopsy interval of 2 (±1) years showed histological class transformation in more than half of the patients. There was no significant difference in treatment outcome and time to attain response based on individual IS agent or sequence of IS agents used. None of the variables (duration of SLE or nephritis, baseline SLEDAI, leukopenia, hypertension, elevated anti-dsDNA, low complements, serum albumin, 24-hour urinary protein, biopsy class) predicted renal response on univariate analysis. No patient had a doubling of serum creatinine or progression to end-stage renal disease. There were three deaths, all related to infection. A change in immunosuppression produces response in most RLN patients while a fifth of them showed no response to therapy. No predictor of renal response was identified. Histologic class switch was frequent. Renal function did not decline over a year of follow-up.
Highlights
Lupus nephritis (LN) is present in about one-third of systemic lupus erythematosus patients at diagnosis and up to two-thirds during the disease course [1]–[3]
There was no significant difference in treatment outcome and time to attain response based on individual immunosuppressive (IS) agent or sequence of IS agents used
No patient had a doubling of serum creatinine or progression to end-stage renal disease
Summary
Lupus nephritis (LN) is present in about one-third of systemic lupus erythematosus patients at diagnosis and up to two-thirds during the disease course [1]–[3]. Progression to end-stage renal disease (ESRD) is a dreaded outcome. Even though the use of newer agents in the treatment, between 20%-70% of patients with LN, do not respond to initial immunosuppressive (IS) therapy [4]–[6]. Non-responders have poor survival, including renal survival at ten years compared to complete or partial responders [7]. Poor prognostic factors reported for LN include hypertension, hypocomplementemia, anti-dsDNA antibodies, high serum creatinine and nephrotic syndrome at initial presentation, and low hematocrit as well as failure to achieve clinical remission within the first year of treatment [8]. Higher NIH activity index (AI) and chronicity index (CI) scores on renal histology are associated with doubling serum creatinine, end-stage renal disease, or death [9]. Class VI LN, thrombotic microangiopathy, antiphospholipid syndrome, or lupus podocytopathy are other causes of persistent renal dysfunction to be considered before labeling as refractory LN (RLN) [10]
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