Clinical characteristics and prognosis of diffuse proliferative lupus nephritis with thrombotic microangiopathy

We retrospectively analyzed the clinicopathologic characteristics and prognosis of 33 patients with diffuse proliferative lupus nephritis (class IV LN) complicated with thrombotic microangiopathy (TMA). Eighty-one percent of patients had renal dysfunction (mean Scr 3.1 ± 2.0 mg/dl), among whom 42.4% needed acute hemodialysis. Nephrotic proteinuria, gross hematuria and hypertension were presented in 57.6%, 24.2% and 93.9% of the patients. Microangiopathic hemolytic anemia, serum anti-dsDNA and anticardiolipin antibodies were found in 60.6%, 75.8% and 33.3% of the patients. Renal biopsy showed IV-G in 75.8%, class IV with class V in 21.2%, and IV-S in 1.23% of the patients. Glomerular segmental necrosis, microthrombi, crescents and arteriolar thrombosis were found in 51.5%, 69.7%, 60.6% and 60.7% of the patients, respectively. The follow up was 1 to 101 months (median 13 months). Only 50% of patients showed response to treatment. Three patients died, 10 developed end-stage renal failure (ESRF). The 5-year patient and renal survival rate was 69.2% and 46.7%, respectively. Major risks for ESRF included: a need for acute dialysis on admission, no response to the treatment and high renal chronic index. The results showed that class IV lupus nephritis with TMA has high mortality and low renal survival.