Lupus Podocytopathy:Clinic-pathological Characteristics And Outcome

Objective:To investigated the clinical histological characteristics and outcomes of SLE patients with lupus podocytopathy.Methodology:Lupus podocytopathy cases were screened from 3750 patients with lupus nephritis(LN) from Jan 2000 to Dec 2013 in the Research Institute of Nephrology, Jinling Hospitial and divided into 3 groups including minimal change disease (MCD), mesangial proliferation (MP) and focal segmental glomerulosclerosis (FSGS) by light microscopy(LM). Patients with proliferative LN(class Ⅲ,Ⅳ, Ⅲ/Ⅳ+Ⅴ) or LN class Ⅴ/Ⅵ were excluded. Their clinical, immunological. pathological features and prognosis were retrospectively analyzed. Different pathological types were also compared with each other.Results:Among 3750 LN patients screened from.53(1.41%) patients were found to have lupus podocytopathy, including 48 females and 5 males, age 12-67 years, SLE disease duration 0.5-84 months (median 5 months). LN duration 0.5-48 months (median 1.5 months). SLE-DAI 2-26 (median10).50 patients presented as nephrotic syndrome in which 17 patients were complicated by acute kidney injury(AKI). Urinary NAG. RBP, KIM-1 levels were significantly higher in patients with AKI than in patients without AKI(P<0.05). Microscopic hematuria and hypertension were found in 9 patients (17%). Damage of hematological system (54.7%) was the most extro-kidney manifestation, the second was malar rash (49.1%) and arthritis (35.8%).The positive rates of anti-dsDNA and ACL were both 26.4%. Low serum C3 was found in 69.8%patients while low serum C4 in 30.2%. Pathological manifestation under LM showed MP in 31 patients, MCD in 13 and FSGS in 9. No obvious difference of foot process fusion and urinary protein was found among different glomerular subtypes.The incidence of AKI was much higher in FSGS group than in MCD and MP group (P<0.01). Compared with MCD group,the ratio of low serum C3 level was significantly higher in MP and FSGS group(P<0.01).Induction treatment with corticosteroids or corticosteroids in combination with other immunosuppressant in 12 weeks led to remission in 94.3% of all patients, with 69.8% received complete remission(CR).The CR rate in FSGS group was much lower than in the other two groups(P<0.01).29(54.7%) patients relapsed during follow-up for 9-125 months(median 44months). The relapse rate of patients treated with corticosteroids was much higher than that treated with corticosteroids in combination with other immuno suppressant (P<0.05).13 patients had repeat renal biopsy while histological transition was found in 6. No patient progressed to end stage renal failure or died.Conclusions:Lupus podocytopathy is characterised by nephrotic syndrome.often complicated by AKI, and different histologic changes including MCD, MP and FSGS. It has high remission rate and benign prognosis, but easy to relapse. Patients with FSGS showed more serious renal lesion and worse therapeutic response.