LAUSR

Abstract To explore the clinicopathological characteristics and prognosis of idiopathic membranous nephropathy (IMN) with focal segmental sclerosis lesions (FSL). A total of 70 IMN patients with FSL (FSL+group) were enrolled in this study, and 140 patients were randomly selected by age and sex matching as disease controls (FSL-group). The clinical and renal histopathological data on renal biopsy and clinical data of patients regularly followed were collected. Serum anti-phospholipase A2 receptor (PLA2R) autoantibody, thrombospondin type-1 domain-containing 7A (7A) autoantibody, glomerular PLA2R and 7A expression, and IgG4 deposition were detected. First, the clinical and pathological significance of IMN combined with the FSL group was analyzed. Whether FSL is a risk factor for renal outcomes was further analyzed.1. Compared with the FSL- group, patients in the FSL+ group had a significantly higher incidence of hypertension and a longer duration of hypertension as well as higher levels of systolic blood pressure, serum creatinine, serum triglycerides, serum cholesterol, 24-hour urinary protein excretion, and lower eGFR and urine osmotic pressure. Patients in the FSL+ group had an increased frequency of Churg stage III and more severe glomerulosclerosis and interstitial fibrosis. The remission rate was significantly lower in the FSL+ group than in the FSL- group (50.0% vs 75.9%, P = .027).2. Multivariate Cox regression analysis showed that FSL (HR = 3.01, 95%CI = 1.07–8.52, P = .038) was an independent risk factor for progression of renal function deterioration, and FSL (HR = 3.25, 95%CI = 1.43–7.38, P = .005) and high levels of serum anti-PLA2R antibody (HR = 1.89, 95%CI = 1.27–2.82, P = .002) were independent risk factors for nonremission of IMN. IMN patients who developed FSL had more severe clinical and pathological characteristics than those without FSL. FSL was an independent risk factor for poorer prognosis. When the appearance of FSL in IMN patients with a high level of serum anti-PLA2R antibody, the treatment needs to be more aggressive to promote remission and to delay the progression of renal function.