İDİOPATİK MEMBRANÖZ GLOMERÜLONEFRİTLERDE C4d VE IgG4 BOYANMASININ KLİNİKOPATOLOJİK PARAMETRELERLE İLİŞKİSİ

Giriş: Membranöz glomerülonefrit (MGN) yetişkinlerdeki nefrotik sendromların en s ık nedenidir. Primer MGN, heterojen bir klinik gidişe sahip, spesifik tanısal belirteçlerin henüz mevcut olmadığı ve bu nedenle tedavi sürecinde de belirsizlikler ya şanan bir glomerülonefrittir. Çalışmamızda, MGN olgularında immun depozitlerde varlığı bildirilen major IgG alt tipi olan IgG4’ün ve bir kompleman ürünü olan C4d’nin pozitifliğinin, gerek primer ve gerekse de sekonder MGN olgularındaki mevcudiyeti yanı sıra bu depozisyonların klinikopatolojik verilerle ve hastaların tedaviye verdiği yanıtlarla ilişkisinin araştırılması amaçlanmıştır. Gereç ve Yöntem: Primer ve sekonder membranöz glomerülonefrit tanısı konulan 37 hastada Hematoksilen Eozin, PAS, Mason Trikrom, Jones Metanamin Silver ve Kongo Red histokimyasal boyaları, IgG, IgM, IgA, Kappa, Lambda, Fibrinojen, C3 ve C1q immunfloresan boyaları, C4d ve IgG4 immunohistokimyasal boyalar ile boyanmış preparatlar değerlendirildi. Bulgular: Klinik verilerine ulaşılan 25 vakanın 15’i primer, 10’u sekonder membranöz glomerülonefritti. Tüm olguların 27’sinde C4d pozitifliği ve 19’unda IgG4 pozitifliği saptandı. IgG4 ve C4d pozitiflik oranı, literatürle uyumlu olacak şekilde idiyopatik MGN olgularında daha yüksek bulunmakla birlikte sekonder MGN’li olgularda da izlendi. Sonuç: Serimizde primer ve sekonder MGN olgularında glomerüler IgG4 pozitifliğinin dağılımında istatistiksel anlamlı bir ilişki saptanamadı. Tedavi sonucu ve takiplerine ulaşabildiğimiz az sayıda olgu olmasına rağmen, glomerüler IgG4 pozitifliği saptanan ve interstisyel yang ı içinde IgG4 pozitif plazma hücreleri bulunduran olgularda, steroid tedavisine oransal olarak anlamlı bir yanıt izlenmiştir.

RELATIONSHIP OF C4d AND IgG4 STAINING WITH CLINICOPATHOLOGICAL PARAMETERS IN IDIOPATHIC MEMBRANOUS GLOMERULONEPHRITIS

Intraduction: Membranous glomerulonephritis (MGN) is the most common cause of nefrotic syndrom in adults. Idiopathic MGN has a heterogenous clinical course, in which no diagnostic markers exist and so there are uncertainities in the treatment process. In this study, the aim is as follows, to determine the existence and, if any, the proportion of the positivity of C4d, a complement product, and IgG4, a major IgG subtype whose existence in the immune deposits in the idiopathic MGN cases is reported in few studies, and to determine the correlation between those values and the clinicopathologic data and the patients’ response to the disease Material and Method: Hematoxylin Eosin, PAS, Mason Trcihrome, Jones Metanamin Silver and Congo Red histochemical stains in 37 patients diagnosed with primary and secondary membranous glomerulonephritis, slides stained with IgG, IgM, IgA, Kappa, Lambda, Fibrinogen, C3 and C1q immunoflorescent dyes and C4d and IgG4 immunohistochemical stains were evaluated. Results: Of the cases whose clinical data were available, 15 were primary and 14 were secondary membranous glomerulonephritis. C4d positivity was found in 27 of all cases and IgG4 positivity was found 19 of them. IgG4 and C4d positivity rates were found to be higher in idiopathic MGN cases, which is consist ent with the literature, but were also observed in cases with secondary MGN. Conclusion: In our series, no statistically meaningful connection was found in the analysis of the distribution of glomerular IgG4 positivity in the primary MGN and secondary MGN cases. Even though there were few cases whose treatment and follow-up results we managed to obtain, there was a pro portionally and meaningfully positive response to the steroid treat ment in the cases who exhibited glomerular IgG4 positivity and had IgG4 positive plasma cells in interstitial inflammation.

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