Sıla KN, Öğüt B, Gürevin S, Öz Ö, Helvacı Ö

Language: English
References: 15
Page: 205-210
PDF size: 406.72 Kb.

ABSTRACT

Fibrillary glomerulonephritis is a rare form of glomerulonephritis, characterized by the deposition of haphazardly arranged fibrils in the glomeruli, leading to renal dysfunction. Here, we present the first familial case of fibrillary glomerulonephritis reported from Türkiye, highlighting its histopathological resemblance to membranous nephropathy. A 45-year-old female with a family history of kidney disease presented with edema and was diagnosed with fibrillary glomerulonephritis. Immunohistochemical staining for DNAJB9 confirmed the diagnosis. The literature review revealed only six reported families with familial fibrillary glomerulonephritis, suggesting a possible autosomal dominant inheritance pattern. Compared to previous cases, our patient and affected family members are female and diagnosed at ages 40-50. The patient’s treatment involves angiotensin receptor blockade due to non-nephrotic proteinuria and normal renal function. The prognosis for fibrillary glomerulonephritis is poor, and therapeutic options are limited. Future research should focus on understanding the genetic basis of familial fibrillary glomerulonephritis and developing effective treatments. This case underscores the importance of considering fibrillary glomerulonephritis in the differential diagnosis of glomerulonephritis, especially in familial cases, and highlights the need for further studies to improve its management.

REFERENCES

1. Erickson SB, Zand L, Nasr SH, Alexander MP,Leung N, Drosou ME, et al. Treatment of fibrillaryglomerulonephritis with rituximab: a 12-month pilotstudy. Nephrol Dial Transplant. 2021;36(1):104-10.

2. Rosenstock JL, Markowitz GS. FibrillaryGlomerulonephritis: An Update. Kidney Int Rep.2019;4(7):917-22.

3. Andeen NK, Yang HY, Dai DF, MacCoss MJ,Smith KD. DnaJ Homolog Subfamily B Member 9Is a Putative Autoantigen in Fibrillary GN. J Am SocNephrol. 2018;29(1):231-9.

4. Nasr SH, Vrana JA, Dasari S, Bridoux F, Fidler ME, KaakiS, et al. DNAJB9 Is a Specific ImmunohistochemicalMarker for Fibrillary Glomerulonephritis. Kidney IntRep. 2018;3(1):56-64.

5. Andeen NK, Smith KD, Vasilescu ER, Batal I.Fibrillary Glomerulonephritis Is Associated WithHLA-DR7 and HLA-B35 Antigens. Kidney Int Rep.2020;5(8):1325-7.

6. Chan TM, Chan KW. Fibrillary glomerulonephritis insiblings. Am J Kidney Dis. 1998;31(5):E4.

7. Ying T, Hill P, Desmond M, Agar J, Mallett A.Fibrillary glomerulonephritis: An apparent familialform? Nephrology (Carlton). 2015;20(7):506-9.

8. Watanabe K, Nakai K, Hosokawa N, WatanabeS, Kono K, Goto S, et al. A Case of FibrillaryGlomerulonephritis with Fibril Deposition in theArteriolar Wall and a Family History of Renal Disease.Case Rep Nephrol Dial. 2017(1):26-33.

9. Andeen NK, Troxell ML, Riazy M, Avasare RS, LapasiaJ, Jefferson JA, et al. Fibrillary Glomerulonephritis:Clinicopathologic Features and Atypical Cases froma Multi-Institutional Cohort. Clin J Am Soc Nephrol.2019;14(12):1741-50.

10. Jeyabalan A, Batal I, Piras D, Morris HK, AppelGB. Familial Fibrillary Glomerulonephritis in LivingRelated Kidney Transplantation. Kidney Int Rep.2021;6(1):239-42.

11. Rosenstock JL, Markowitz GS, Valeri AM,Sacchi G, Appel GB, D’Agati VD. Fibrillary andimmunotactoid glomerulonephritis: Distinct entitieswith different clinical and pathologic features. KidneyInt. 2003;63(4):1450-61.

12. Raikar M, Shafiq A. Fibrillary Glomerulonephritis:A Great Mimicker of Rapidly ProgressiveGlomerulonephritis. Cureus. 2022;14(6):e26001.

13. Sabanis N, Liaveri P, Geladari V, Liapis G, MoustakasG. DNAJB9 Fibrillary Glomerulonephritis withMembranous-Like Pattern: A Case-Based LiteratureReview. Cureus. 2023;15(10):e47862.

14. Javaugue V, Karras A, Glowacki F, McGregor B,Lacombe C, Goujon JM, et al. Long-term kidneydisease outcomes in fibrillary glomerulonephritis:a case series of 27 patients. Am J Kidney Dis.2013;62(4):679-90.

15. Hogan J, Restivo M, Canetta PA, Herlitz LC,Radhakrishnan J, Appel GB, et al. Rituximabtreatment for fibrillary glomerulonephritis. NephrolDial Transplant. 2014;29(10):1925-31.