Evaluation of Clinical Findings and Treatment Outcomes of Patients Diagnosed with Primary Membranous Nephropathy: A Single Center Experience

Yıl 2025, Cilt: 11 Sayı: 3, 419 - 427, 29.09.2025

Gamze Coşkun Kılınç , Ömer Faruk Akçay , Ebru Gök Oğuz , Mehmet Deniz Aylı

https://doi.org/10.53394/akd.1542976

Öz

Objective: Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in non-diabetic white adults. The primary aim of our study is to compare the demographic and clinical characteristics, treatment outcomes, and the occurrence of remission in patients with primary MN.
Materials and Methods: Our study included 35 patients diagnosed with primary MN between January 2012 and January 2021. Based on risk classification, patients were distributed across low, moderate, high, and very high-risk groups. All patients were treated under Kidney Disease: Improving Global Outcomes (KDIGO) guidelines.
Results: The mean age of our patients was 55.9±10.8 years, and 71.4% were male. At diagnosis, almost all patients (97.1%) had nephrotic-level proteinuria. In the risk classification of the patients with MN, 13 (37.1%) patients were low risk, 11 (31.4%) patients were moderate risk, 8 (22.9%) patients were high risk, and 3 (8.6%) patients were in the very high-risk group. The overall remission rate at the end of the first year was 68.6%. The mean age of patients who achieved remission was 52.8±10.5, while the mean age of patients who did not achieve remission was 62.9±8.4 (p=0.008). The most commonly used immunosuppressive (IS) agent that combined with steroids was cyclophosphamide (65.7%), followed by calcineurin inhibitors (45.7%).
Conclusion: High overall remission rates were observed at the end of the first year of MN treatment. IS treatment applied to MN patients may contribute to early remission.

Anahtar Kelimeler

Membranous nephropathy , remission , immunosuppressive treatment , proteinuria

Primer Membranöz Nefropatili Hastaların Klinik Bulgularının ve Tedavi Sonuçlarının Değerlendirilmesi: Tek Merkez Deneyimi

Öz

Amaç: Membranöz nefropati (MN), diyabetik olmayan beyaz yetişkinlerde nefrotik sendromun en yaygın nedenidir. Çalışmamızın temel amacı, primer MN tanısı alan hastaların demografik ve klinik özelliklerini, tedavi sonuçlarını ve remisyon oluşumunu karşılaştırmaktır.
Gereç ve Yöntemler: Çalışmamıza, Ocak 2012 ile Ocak 2021 arasında primer MN tanısı alan 35 hasta dahil edilmiştir. Risk sınıflandırmasına dayalı olarak hastalar düşük, orta, yüksek ve çok yüksek risk gruplarına dağıtılmıştır. Tüm hastalar, Böbrek Hastalığı: Sonuçların İyileştirilmesi (KDIGO) kılavuzları altında tedavi edilmiştir.
Bulgular: Hastalarımızın ortalama yaşı 55.9±10.8 yıl olup, çoğunluğu (%71.4) erkekti. Tanı anında hastaların neredeyse tamamında (%97.1) nefrotik seviyede proteinüri mevcuttu. MN için yapılan risk sınıflandırmasında, 13 (%37.1) hasta düşük risk grubunda, 11 (%31.4) hasta orta risk grubunda, 8 (%22.9) hasta yüksek risk grubunda ve 3 (%8.6) hasta çok yüksek risk grubunda yer aldı. İlk yıl sonunda genel remisyon oranı %68.6 idi. Remisyon elde eden hastaların ortalama yaşı 52.8±10.5 iken, remisyon elde edemeyen hastaların ortalama yaşı 62.9±8.4 idi (p=0.008). Steroidlerle birlikte en yaygın kullanılan immünosupresif (IS) ajan, siklofosfamid (%65.7) olup, bunu kalsinörin inhibitörleri (%45.7) takip etti.
Sonuç: Tek merkezli çalışmamızda, literatürle tutarlı olarak MN tedavisinin ilk yılı sonunda yüksek genel remisyon oranları gözlemlenmiştir. MN hastalarına uygulanan IS tedavi, erken remisyona katkıda bulunabilir.

Anahtar Kelimeler

Membranöz nefropati , remisyon , immünosupresif tedavi , proteinüri

Kaynakça

• 1. Cattran DC, Brenchley PE. Membranous nephropathy: integrating basic science into improved clinical management. Kidney International 2017; 91:566-74.
• 2. Ronco P, Debiec H. Pathophysiological advances in membranous nephropathy: time for a shift in patient's care. Lancet 2015; 385:1983-92.
• 3. Alsharhan L, Beck LH, Jr. Membranous Nephropathy: Core Curriculum 2021. Am J Kidney Dis 2021; 77:440-53.
• 4. Couser WG. Primary Membranous Nephropathy. Clin J Am Soc Nephrol 2017; 12:983-97.
• 5. Polanco N, Gutiérrez E, Covarsí A, Ariza F, Carreño A, Vigil A, Baltar J, Fernández-Fresnedo G, Martín C, Pons S, Lorenzo D, Bernis C, Arrizabalaga P, Fernández-Juárez G, Barrio V, Sierra M, Castellanos I, Espinosa M, Rivera F, Oliet A, Fernández-Vega F, Praga M. Spontaneous remission of nephrotic syndrome in idiopathic membranous nephropathy. J Am Soc Nephrol 2010; 21:697-704.
• 6. Hofstra JM, Beck LH, Jr., Beck DM, Wetzels JF, Salant DJ. Anti-phospholipase A₂ receptor antibodies correlate with clinical status in idiopathic membranous nephropathy. Clin J Am Soc Nephrol 2011; 6:1286-91.
• 7. Rovin BH, Adler SG, Barratt J, Bridoux F, Burdge KA, Chan TM, Cook HT, Fervenza FC, Gibson KL, Glassock RJ, Jayne DRW, Jha V, Liew A, Liu ZH, Mejía-Vilet JM, Nester CM, Radhakrishnan J, Rave EM, Reich HN, Ronco P, Sanders JF, Sethi S, Suzuki Y, Tang SCW, Tesar V, Vivarelli M, Wetzels JFM, Lytvyn L, Craig JC, Tunnicliffe DJ, Howell M, Tonelli MA, Cheung M, Earley A, Floege J. Executive summary of the KDIGO 2021 Guideline for the Management of Glomerular Diseases. Kidney Int 2021; 100:753-79.
• 8. Rovin BH, Adler SG, Barratt J, Bridoux F, Burdge KA, Chan TM, Cook HT, Fervenza FC, Gibson KL, Glassock RJ, Jayne DRW, Jha V, Liew A, Liu Z-H, Mejía-Vilet JM, Nester CM, Radhakrishnan J, Rave EM, Reich HN, Ronco P, Sanders J-SF, Sethi S, Suzuki Y, Tang SCW, Tesar V, Vivarelli M, Wetzels JFM, Floege J. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney International 2021; 100:S1-S276.
• 9. Radhakrishnan J, Cattran DC. The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines—application to the individual patient. Kidney International 2012; 82:840-56.
• 10. Francis JM, Beck LH, Jr., Salant DJ. Membranous Nephropathy: A Journey From Bench to Bedside. Am J Kidney Dis 2016; 68:138-47.
• 11. Larsen CP, Messias NC, Silva FG, Messias E, Walker PD. Determination of primary versus secondary membranous glomerulopathy utilizing phospholipase A2 receptor staining in renal biopsies. Modern Pathology 2013; 26:709-15.
• 12. Shiiki H, Saito T, Nishitani Y, Mitarai T, Yorioka N, Yoshimura A, Yokoyama H, Nishi S, Tomino Y, Kurokawa K, Sakai H. Prognosis and risk factors for idiopathic membranous nephropathy with nephrotic syndrome in Japan. Kidney Int 2004; 65:1400-7.
• 13. Sun J, Li M, Zhu Q, Jia Y, Tian J, Zhang C, Du X. Glomerulosclerosis is a prognostic risk factor in patients with membranous nephropathy and non-nephrotic proteinuria. Ren Fail 2023; 45:2188088.
• 14. Yildiz A, Ulu S, Oruc A, Ucar AR, Ozturk S, Alagoz S, Eren N, Kocyigit I, Koksal Cevher S, Haras AB, Sumnu A, Arinsoy T, Sahin G, Suleymanlar G, Cavdar C, Kumru Sahin G, Kurultak I, Unsal A, Sahin G, Kazan S, Tatar E, Dıkec M, Dursun B, Sayarlioglu H, Turkmen K, Artan AS, Aktas N, Yilmaz Z, Behlul A, Dheir H, Kutlay S, Seyahi N. Clinical and pathologic features of primary membranous nephropathy in Turkey: a multicenter study by the Turkish Society of Nephrology Glomerular Diseases Working Group. Ren Fail 2022; 44:1048-59.
• 15. Jiang Z, Cai M, Dong B, Yan Y, Wang Y, Li X, Shao C, Zuo L. Renal outcomes of idiopathic and atypical membranous nephropathy in adult Chinese patients: a single center retrospective cohort study. BMC Nephrol 2021; 22:148.
• 16. Ameh OI, Swanepoel CR, Aderibigbe A, Kengne AP, Okpechi IG. Out of Africa: Complete and partial remissions as a combined outcome in patients with idiopathic membranous glomerulonephritis in Cape Town. Nephrology (Carlton) 2016; 21:1010-6.
• 17. Larsen CP, Beggs ML, Walker PD, Saeed M, Ambruzs JM, Messias NC. Histopathologic effect of APOL1 risk alleles in PLA2R-associated membranous glomerulopathy. Am J Kidney Dis 2014; 64:161-3.
• 18. Glassock RJ. Diagnosis and natural course of membranous nephropathy. Semin Nephrol 2003; 23:324-32.
• 19. Bae E, Lee SW, Park S, Kim DK, Lee H, Huh H, Chin HJ, Lee S, Ryu DR, Park JI, Kim S, Park DJ, Kang SW, Kim YS, Oh YK, Kim YC, Lim CS, Park JT, Lee JP. Treatment and clinical outcomes of elderly idiopathic membranous nephropathy: A multicenter cohort study in Korea. Arch Gerontol Geriatr 2018; 76:175-81.
• 20. Albertazzi V, Fontana F, Giberti S, Aiello V, Battistoni S, Catapano F, Graziani R, Cimino S, Scichilone L, Forcellini S, De Fabritiis M, Sara S, Delsante M, Fiaccadori E, Mosconi G, Storari A, Mandreoli M, Bonucchi D, Buscaroli A, Mancini E, Rigotti A, La Manna G, Gregorini M, Donati G, Cappelli G, Scarpioni R, for the Gruppo di Studio della Glomerulonefrite Membranosa in Emilia R. Primary membranous nephropathy in the Italian region of Emilia Romagna: results of a multicenter study with extended follow-up. Journal of Nephrology 2024; 37:471-82.
• 21. von Groote TC, Williams G, Au EH, Chen Y, Mathew AT, Hodson EM, Tunnicliffe DJ. Immunosuppressive treatment for primary membranous nephropathy in adults with nephrotic syndrome. Cochrane Database Syst Rev 2021; 11:Cd004293.
• 22. Schieppati A, Perna A, Zamora J, Giuliano GA, Braun N, Remuzzi G. Immunosuppressive treatment for idiopathic membranous nephropathy in adults with nephrotic syndrome. Cochrane Database Syst Rev 2004; Cd004293.