İdiyopatik Membranöz Glomerülonefrit Tanısı Alan Yetişkin Hastaların Değerlendirilmesi: Tek Merkez Deneyimi

Öz

İmmünkompetan bir nefropati olan membranöz glomerülonefrit (MGN), nefrotik sendromun en yaygın nedenleri arasındadır. Spontan remisyon meydana gelebilse de, uzun vadeli son dönem böbrek yetmezliği gelişme riski vardır. MGN tedavi yaklaşımları konusunda tam bir fikir birliği yoktur; ancak immünosüpresif tedaviler kritik öneme sahiptir. Bu retrospektif, kesitsel çalışmaya, Mart 2007 ile Kasım 2015 tarihleri arasında merkezimizi ziyaret eden ve MGN tanısı alan 99 hasta dahil edilmiştir. Hasta verileri hastane bilgi sisteminden retrospektif olarak taranmış ve kaydedilmiştir. 99 hastanın 43’ü (%43,4) kadındı. Hastaların ortalama yaşı 46,7 yıl ve ortalama takip süresi 18,5 ay idi. Toplam 71 hasta (%71,7) nefrotik proteinüri ile başvururken, 57 hastada (%57,6) nefrotik sendrom vardı. 85 hastaya (%85,9) immünosupresif ilaç reçete edildi. İmmünosupresif tedavi görenler arasında 57 hastada (%79,9) tanı anında proteinüri devam ediyordu. İmmünosupresif tedavi uygulanan 63 hastada (%74,1) remisyon sağlanırken, konservatif tedavi uygulanan 10 hastada (%71,3) remisyon sağlandı. Hipertansiyon ve IgG birikimi, idiyopatik membranöz glomerülonefrit (MGN) tanısı alan hastalarda daha zayıf tedavi yanıtıyla ilişkili olabilir. Özetle, MGN, farklı yaş ve cinsiyetlerdeki bireyleri etkileyebilen nefrotik sendromun yaygın bir nedenidir. Çalışmamız, immünosüpresif tedavi gören hastalarda daha yüksek remisyon oranları tespit etmiş olup, bu durum, etkili hastalık yönetimi için bireysel risk faktörlerinin değerlendirilmesi ve uygun tedavi stratejilerinin seçilmesinin önemini vurgulamaktadır.

Anahtar Kelimeler

• Membranöz Glomerulonefrit
• İmmunsupresif tedavi
• Remisyon

Evaluation of Adult Patients Diagnosed with Idiopathic Membranous Glomerulonephritis: A Single-Center Retrospective Study

Öz

Membranous glomerulonephritis (MGN), an immunocompetent nephropathy, is among the most prevalent causes of nephrotic syndrome. While spontaneous remission may occur, there is a risk of developing long-term end-stage renal failure. Treatment approaches for MGN lack full consensus; however, immunosuppressive therapies are critically important. This retrospective, cross-sectional study included 99 patients who visited our center between March 2007 and November 2015 and were diagnosed with MGN. Patient data were scanned and recorded retrospectively from the hospital information system. Of the 99 patients, 43 (43.4%) were female. The mean age of the patients was 46.7 years, with a mean follow-up duration of 18.5 months. A total of 71 patients (71.7%) presented with nephrotic proteinuria, while 57 patients (57.6%) had nephrotic syndrome. Immunosuppressive drugs were prescribed to 85 patients (85.9%). Among those receiving immunosuppressive therapy, 57 patients (79.9%) still exhibited proteinuria at the time of diagnosis. Remission was achieved in 63 patients (74.1%) who underwent immunosuppressive therapy, compared to 10 patients (71.3%) who received conservative treatment. Hypertension and IgG deposition may be associated with a poorer treatment response in patients diagnosed with idiopathic membranous glomerulonephritis (MGN). In summary, MGN is a common cause of nephrotic syndrome that can affect individuals of various ages and genders. Our study identified higher remission rates in patients treated with immunosuppressive therapy, which emphasizes the value of evaluating individual risk factors and selecting appropriate treatment strategies for effective disease management.

Anahtar Kelimeler

• membranous glomerulonephritis
• immunosuppressive therapy
• remission.

Kaynakça

1. 1.Beck LH Jr, Salant DJ. Membranous nephropathy: Recenttravels and new roads ahead. Kidney Int 2010;77:765-70.
2. 2.Nangaku M, Shankland SJ, Couser WG. Cellular response toinjury in membranous nephropathy. J Am Soc Nephrol2005;16:1195-204.
3. 3.Vaughan RW, Demaine AG, Welsh KI. A DQA1 allele isstrongly associated with idiopathic membranous nephropathy.Tissue Antigens 1989;34:261-9.
4. 4.Le Petit JC, Laurent B, Berthoux FC. HLA-DR3 and idiopathicmembranous nephritis (IMN) association. Tissue Antigens1982;20:227- 8.
5. 5.Fervenza FC, Sethi S, Specks U. Idiopathic membranousnephropathy diagnosis and treatment. Clin J Am Soc Nephrol2008;3:905-19.
6. 6.Gluck MC, Gallo G, Lowenstein J, et al. Membranousglomerulonephritis: Evolution of clinical and pathologicfeatures. Ann Intern Med 1973;78:1- 12.
7. 7.Haas M. IgG subclass deposits in glomeruli of lupus andnonlupus membranous nephropathies. Am J Kidney Dis1994;23:358-64.
8. 8.Kuroki A, Shibata T, Honda H, et al. Glomerular and serumIgG subclasses in diffuse proliferative lupus nephritis,membranous lupus nephritis, and idiopathic membranousnephropathy. Intern Med 2002;41:936-42.

9. 9.KDIGO Clinical Practice Guideline for Glomerular Diseases.Kidney Int Suppl. 2021;11(3):S1–S576.
10. 10.Tomas NM, Beck LH Jr, Meyer-Schwesinger C, et al.Thrombospondin type-1 domain-containing 7A in idiopathicmembranous nephropathy. N Engl J Med. 2014;371(24):2277–2287. doi:10.1056/NEJMoa1409354. PMID: 25470565.
11. 11.Cattran D. Management of membranous nephropathy: whenand what for treatment. J Am Soc Nephrol 2005;16:1188-94.
12. 12.Rihova Z, Honsova E, Merta M, et al. Secondary membranousnephropathy-one center experience. Ren Fail 2005;27:397-402.
13. 13.Hitoshi Y, Takashi T, Hitoshi S, Hiroshi S. Membranousnephropathy in Japan: analysis of the Japan Renal BiopsyRegistry (J-RBR).Clin Exp Nephrol 2012;16:557–63.
14. 14.Dumoulin A, Hill GS, Montseny JJ, Meyrier A. Clinical andmorphological prognostic factors in membranous nephropathy: significance of focal segmental glomerulosclerosis. Am JKidney Dis 2003;41:38-48.
15. 15.Gupta R, Sharma A, Mahanta PJ, Jacob TG, et al. Focalsegmental glomerulosclerosis in idiopathic membranousglomerulonephritis: a clinico-pathological and stereological study. Nephrol Dial Transplant 2010;25:444–9.
16. 16.Unver S, Haholu A, Atasoyu EM, et al. A rare complication ofidiopathic membranous nephropathy: crescentic transformation.Ren Fail 2008;30:573–5.
17. 17.Koethe JD, Gerig JS, Glickman JL, et al. Progression of membranous nephropathy to acute crescentic rapidlyprogressive glomerulonephritis and response to pulsemethylprednisolone. Am J Nephrol 1986;6:224–8.
18. 18.James SH, Lien YH, Ruffenach SJ, et al. Acute renal failure inmembranous glomerulonephropathy: a result of superimposedcrescentic glomerulonephritis. J Am Soc Nephrol 1995;6:1541–6.
19. 19.Singh A, Arif F, Mangat S, et al. Acute cellular crescenticglomerulonephritis in association with antiglomerular basementmembrane disease and superimposed membranousnephropathy. Am J Kidney Dis 2004;43-8.
20. 20.Kadatz M, Zimpelmann J, Dember LM, et al. Cost-effectiveness of rituximab versus cyclosporine in the treatmentof membranous nephropathy: a model-based analysis of theMENTOR trial. Nephrol Dial Transplant. 2024;39(5):688–696. doi:10.1093/ndt/gfae084. PMID: 38621719
21. 21.Fervenza FC, Appel GB, Barbour SJ, et al. Rituximab orCyclosporine in the Treatment of Membranous Nephropathy. NEngl J Med. 2019;381(1):36–46. doi:10.1056/NEJMoa1814427. PMID: 31154981