Adult primary glomerular diseases due to podocytopathies: a single center experience on patient characteristics, treatment and outcomes

Ismail Bulgur,Sait Sen,Banu Sarsik Kumbaraci, Meltem Sezis Demirci,Mumtaz Yilmaz,Gulay Asci

CUKUROVA MEDICAL JOURNAL(2024)

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摘要
Purpose: This study aims to evaluate the demographic, clinical, and pathologic characteristics and response to immunosuppressive therapy, particularly corticosteroids, in adult patients with primary focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD), which are classified as podocytopathies. Materials and Methods: Between January 1998 and December 2014, this study included 44 patients (27 with primary FSGS and 17 with MCD) aged older than 18 years with a histopathologic diagnosis, symptoms of nephrotic syndrome, and a minimum follow-up of six months. Patients were divided into two groups according to the treatment they received and three groups according to their response to treatment. Patients diagnosed with primary FSGS and MCD were evaluated based on clinical, demographic, and laboratory findings, as well as response to treatment, and a comparison was conducted between the two groups. Results: 59.1% of the patients were male with a mean age of 44.8 +/- 17.7 years. At the time of diagnosis, there were no statistically significant differences in clinical and demographic characteristics between MCD and primary FSGS patients. However, in patients with MCD, the mean creatinine clearance (118.0 +/- 46.7 ml/min) was higher and the rate of microscopic hematuria (11.8%) was lower at the time of diagnosis. There was an increased need for alternative immunosuppressive treatments besides corticosteroids in patients with primary FSGS to achieve partial or complete remission. At both the third and sixthmonth follow-ups, MCD patients achieved a higher rate of complete remission (proteinuria <0.3 g/day) than FSGS patients. Conclusion: Compared to MCD, primary FSGS is more likely to progress, requires more immunosuppressive therapy beyond corticosteroids to achieve partial or complete remission, and has a lower treatment response rate.
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关键词
Podocytopathy,primary focal segmental glomerulosclerosis,minimal change disease,proteinuria,prognosis
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