Diffuse proliferative lupus nephritis: long-term observations in patients treated with ancrod

Area of Science:

• Nephrology
• Rheumatology
• Pharmacology

Background:

• Diffuse proliferative lupus nephritis (DPLN) is a severe autoimmune kidney disease.
• Patients often present with deteriorating renal function despite high-dose corticosteroids.
• Glomerular thrombosis and sclerosis are common in severe DPLN.

Purpose of the Study:

• To report the long-term outcomes of patients with severe DPLN treated with ancrod and nitrogen mustard.
• To identify factors associated with long-term renal function in DPLN patients.
• To evaluate the efficacy of ancrod and nitrogen mustard in severe lupus nephritis.

Main Methods:

• A cohort of 22 patients with histologically confirmed DPLN and glomerular thrombosis was treated.
• Treatment involved a 14-day course of ancrod, followed by nitrogen mustard in most patients.
• Long-term follow-up averaged 58 months for all patients.

Main Results:

• Three patients died from non-renal causes; 11 developed end-stage renal disease (ESRD) at an average of 27 months post-treatment.
• Eight patients (36%) remained alive with stable renal function at an average of 70 months post-treatment.
• Factors predicting deterioration included prior prednisone use, glomerular sclerosis, high alpha 2-antiplasmin, and later SLE/DPLN relapses.

Conclusions:

• Ancrod and nitrogen mustard treatment yielded a satisfactory long-term outcome in a subset of severe DPLN patients, considering disease severity.
• Prior treatment, histological damage, and disease activity significantly influence long-term renal prognosis.
• Further research is needed to optimize treatment strategies for severe lupus nephritis.