Podositopatilere bağlı erişkin primer glomerüler hastalıklar: hasta özellikleri, tedavileri ve sonuçlarına ilişkin tek merkez deneyimi

Year 2024, Volume: 49 Issue: 1, 21 - 30, 29.03.2024

İsmail Bulgur , Sait Şen , Banu Sarsık Kumbaracı , Meltem Seziş , Mümtaz Yılmaz , Gülay Aşçı

https://doi.org/10.17826/cumj.1382008

Abstract

Amaç: Bu çalışma podositopatiler çatısı altında değerlendirilen primer fokal segmental glomerüloskleroz (FSGS) ve minimal değişiklik hastalığı (MDH) tanılı erişkin hastaların demografik, klinik, patolojik özelliklerini ve başta kortikosteroidler olmak üzere uygulanan immunsupresif tedaviye yanıtlarını değerlendirmeyi amaçlamaktadır.
Gereç ve Yöntem: Çalışmaya Ocak 1998 ve Aralık 2014 tarihleri arasında, en az 6 ay takip edilen, nefrotik sendrom bulguları gösteren, histopatolojik olarak MDH ve primer FSGS tanısı almış, 18 yaş üstü 44 hasta (27 primer FSGS ve 17 MDH) dahil edildi. Hastalar uygulanan tedavi açısından 2 gruba, tedaviye cevap durumuna göre ise 3 gruba ayrıldı. Primer FSGS ve MDH tanılı hastalar klinik, demografik, laboratuvar bulgularının yanı sıra tedaviye yanıt açısından değerlendirilmiş ve iki grup arasında karşılaştırma yapılmıştır.
Bulgular: Hastaların %59.1’i erkek olup, yaş ortalaması 44.8±17.7 yıldı. Klinik ve demografik özellikler açısından tanı anında MDH ve primer FSGS hastaları arasında istatistiksel olarak anlamlı fark saptanmadı. Fakat, MDH’da tanı anında kreatinin klirensi ortalamasının (118.0±46.7 ml/dk) daha yüksek olduğu saptanmış, mikroskobik hematüri oranı (%11.8) ise daha düşük bulunmuştur. Primer FSGS hastalarında parsiyel veya tam remisyon elde edebilmek amacıyla kortikosteroid dışında diğer immunsupresif tedavilere daha fazla ihtiyaç duyulmuştur. 3. ve 6. ay takiplerinde, tam remisyona (proteinüri <0,3 g/gün) girme oranının MDH hastalarında FSGS hastalarından daha yüksek olduğu saptanmıştır.
Sonuç: Primer FSGS’nin MDH’a kıyasla daha çok progresyon gösterme eğiliminde olduğu, parsiyel veya tam remisyon elde edebilmek amacıyla kortikosteroid dışında diğer immunsupresif tedavilere daha fazla ihtiyaç duyulduğu ve tedaviye yanıtının da daha düşük olduğu saptanmıştır.

Keywords

Podositopati, primer fokal segmental glomerüloskleroz, minimal değişiklik hastalığı, proteinüri, prognoz

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

Abstract

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 sixth-month 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.

Keywords

Podocytopathy, Primary focal segmental glomerulosclerosis, minimal change disease, proteinuria, prognosis

References

• Floege J, Amann K. Primary glomerulonephritides. Lancet. 2016;387:2036-48.
• Ahn W, Bomback AS. Approach to diagnosis and management of primary glomerular diseases due to podocytopathies in adults: core curriculum 2020. Am J Kidney Dis. 2020;75:955-64.
• Mathieson PW. Minimal change nephropathy and focal segmental glomerulosclerosis. Semin Immunopathol. 2007;29:415-26.
• Maas RJ, Deegens JK, Smeets B, Moeller MJ, Wetzels JF. Minimal change disease and idiopathic FSGS: manifestations of the same disease. Nat Rev Nephrol. 2016;12:768-76.
• Couser WG. Pathogenesis of glomerular damage in glomerulonephritis. Nephrol Dial Transplant. 1998;13:10-5.
• Bonegio RG, Salant DJ, Fervenza FC. Mechanisms of immune injury of the glomerulus. https://www.uptodate.com/contents/3068 (accessed Sept 2023).
• Rovin BH, Adler SG, Barratt J, Bridoux F, Burdge KA, Chan TM et al. KDIGO 2021 Clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021;100:1-276.
• Waldman M, Crew RJ, Valeri A, Busch J, Stokes B, Markowitz G et al. Adult minimal-change disease: clinical characteristics, treatment, and outcomes. Clin J Am Soc Nephrol. 2007;2:445-53.
• Hogan J, Radhakrishnan J. The treatment of minimal change disease in adults. J Am Soc Nephrol. 2013;24:702-11.
• Meyrier A, Radhakrishnan J, Fervenza FC. Minimal change disease: treatment in adults. https://www.uptodate.com/contents/minimal-change-disease-treatment-in-adults (accessed Sept 2023).
• Bagchi S, Agarwal S, Kalaivani M, Bhowmik D, Singh G, Mahajan S et al. Primary FSGS in nephrotic adults: clinical profile, response to immunosuppression and outcome. Nephron. 2016;132:81-5.
• Korbet SM. Treatment of primary FSGS in adults. J Am Soc Nephrol. 2012;23:1769-76.
• Moura LR, Franco MF, Kirsztajn GM. Minimal change disease and focal segmental glomerulosclerosis in adults: response to steroids and risk of renal failure. J Bras Nefrol. 2015;37:475-80.
• Maas RJ, Deegens JK, Beukhof JR, Reichert LJ, Ten Dam MA, Beutler JJ et al. The clinical course of minimal change nephrotic syndrome with onset in adulthood or late adolescence: a case series. Am J Kidney Dis. 2017;69:637-46.
• Huang J-J, Hsu S-C, Chen F-F, Sung J-M, Tseng C-C, Wang M-C. Adult-onset minimal change disease among Taiwanese: clinical features, therapeutic response, and prognosis. Am J Nephrol. 2001;21:28-34.
• Nolasco F, Cameron JS, Heywood E, Hicks J, Ogg C, Williams DG. Adult–onset minimal change nephrotic syndrome: a long–term follow–up. Kidney Int. 1986;29:1215-23.
• Mak S, Short C, Mallick N. Long-term outcome of adult-onset minimal-change nephropathy. Nephrol Dial Transplant. 1996;11:2192-201.
• Dieng A, Ba M, Sy A, Ndongo M, Ndiaye B, Diawara M et al. Patterns of primary focal segmental glomerulosclerosis (FSGS) in adults treated in a Dakar single center: about 58 cases. Open Journal of Nephrology. 2020;10:265-73.
• Ren H, Shen P, Li X, Pan X, Zhang Q, Feng X et al. Treatment and prognosis of primary focal segmental glomerulosclerosis. Contrib Nephrol. 2013;181:109-18.
• Stirling C, Mathieson P, Boulton-Jones J, Feehally J, Jayne D, Murray H et al. Treatment and outcome of adult patients with primary focal segmental glomerulosclerosis in five UK renal units. QJM. 2005;98:443-9.
• Fervenza FC, Sethi S. Focal segmental glomerulosclerosis: clinical features and diagnosis. https://www.uptodate.com/contents/focal-segmental-glomerulosclerosis-clinical-features-and-diagnosis (accessed Sept 2023).
• Schwartz MM, Evans J, Bain R, Korbet SM. Focal segmental glomerulosclerosis: prognostic implications of the cellular lesion. J Am Soc Nephrol. 1999;10:1900-7.
• Cattran DC, Feehally J, Cook HT, Liu ZH, Fervenza FC, Mezzano SA et al. Kidney disease: improving global outcomes (KDIGO) glomerulonephritis work group. KDIGO clinical practice guideline for glomerulonephritis. Kidney Int. 2012;2:139-274.
• Cattran DC, Appel GB, Fervenza FC, De Vriese AS. Focal segmental glomerulosclerosis: treatment and prognosis. https://www.uptodate.com/contents/focal-segmental-glomerulosclerosis-treatment-and-prognosis (Accessed 01.09.2023).