ABSTRACT

Aim:

Focal segmental glomerulosclerosis (FSGS) is one of the common glomerulonephritis in Turkey and the world. The aim of our study was to determine demographic and clinical features and biopsy results of patients who were followed up with primary FSGS in our center.

Methods:

Ninety-nine adult patients with FSGS diagnosed via renal biopsy, who were followed up in our outpatient clinic, were included in our single-center, retrospective, cross-sectional study. Patients with secondary FSGS were excluded. Demographic and clinical features and biopsy results were examined retrospectively.

Results:

Fifty-nine patients (59.6%) were women. Nephrotic syndrome was the most common biopsy indication (68.7%) and the mean estimated glomerular filtration rate at presentation was 78.9±35.8 mL/min/1.73 m². The mean serum albumin level was 3.2±0.9 g/dL and urine protein level was 3485 mg/day (interquartile range: 1739-7275). Interstitial fibrosis was detected in 63.4% and tubular atrophy in 74.7% of patients.

Conclusion:

The complaints and clinical data of our patients with FSGS were similar with the country and world data. Approximately 40% of the patients presented with decreased renal functions at the time of biopsy. Biopsy should be considered earlier in patients suspected of FSGS, as tubular atrophy and interstitial fibrosis rates were high in biopsies.

References

1Ozturk S, Sumnu A, Seyahi N, ve ark. Demographic and clinical characteristics of primary glomerular diseases in Turkey. Int Urol Nephrol 2014;46:2347-55.

2Haas M, Meehan SM, Karrison TG, Spargo BH. Changing etiologies of unexplained adult nephrotic syndrome: a comparison of renal biopsy findings from 1976-1979 and 1995-1997. Am J Kidney Dis 1997;30:621-31.

3Hommos MS, De Vriese AS, Alexander MP, ve ark. The Incidence of Primary vs Secondary Focal Segmental Glomerulosclerosis. A Clinicopathologic Study Mayo Clin Proc 2017; 92:1772-81.

4Kitiyakara C, Eggers P, Kopp JB. Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States. Am J Kidney Dis 2004;44:815-25.

5Crew RJ, Appel GB. Focal segmental glomerulosclerosis. In Greenberg A, ed. The NKF Primer on Kidney Disease, 4th ed. Philadelphia, Elsevier 2005; 178-82.

6Chun MJ, Korbet SM, Schwartz MM, Lewis EJ. Focal segmental glomerulosclerosis in nephrotic adults: presentation, prognosis, and response to therapy of the histologic variants. J Am Soc Nephrol 2004;15:2169-77.

7Deegens JK, Steenbergen EJ, Wetzels JF. Review on diagnosis and treatment of focal segmental glomerulosclerosis. Neth J Med 2008;66:3-12.

8Deegens JK, Dijkman HB, Borm GF, ve ark. Podocyte foot process effacement as a diagnostic tool in focal segmental glomerulosclerosis. Kidney Int 2008;74:1568-76.

9Rydel JJ, Korbet SM, Borok RZ, Schwartz MM. Focal segmental glomerular sclerosis in adults: Presentation, course, and response to treatment. Am J Kidney Dis 1995;25:534-42.

10Cattran DC, Rao P. Long-term outcome in children and adults with classic focal segmental glomerulosclerosis. Am J Kidney Dis 1998;32:72-9.

11Pokhariyal S, Gulati S, Prasad N, ve ark. Duration of optimal therapy for idiopathic focal segmental glomerulosclerosis. J Nephrol 2003;16:691-6.

12Crook ED, Habeeb D, Gowdy O, Nimmagadda S, Salem M. Effects of steroids in focal segmental glomerulosclerosis in a predominantly African-American population. Am J Med Sci 2005;330:19-24.

13Thomas DB, Franceschini N, Hogan SL, ve ark. Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. Kidney Int 2006;69:920-6.

14Sethi S, Zand L, Nasr SH, Glassock RJ, Fervenza FC. Focal and segmental glomerulosclerosis: clinical and kidney biopsy correlations. Clin Kidney J 2014;7:531-7.

15Kambham N, Markowitz GS, Valeri AM, Lin J, D’Agati VD. Obesity-related glomerulopathy: an emerging epidemic. Kidney Int 2001;59:1498-509.

16Praga M, Hernández E, Morales E, ve ark. Clinical features and long-term outcome of obesity-associated focal segmental glomerulosclerosis. Nephrol Dial Transplant 2001;16:1790-8.

17Praga M, Borstein B, Andres A, ve ark. Nephrotic proteinuria without hypoalbuminemia: clinical characteristics and response to angiotensin-converting enzyme inhibition. Am J Kidney Dis 1991;17:330-8.

18Praga M, Morales E, Herrero JC, ve ark. Absence of hypoalbuminemia despite massive proteinuria in focal segmental glomerulosclerosis secondary to hyperfiltration. Am J Kidney Dis 1999;33:52-8.

19Schonholzer KW, Waldron M, Magi AB. Intraglomerular foam cells and human focal glomerulosclerosis. Nephron 1992;62:130-6.

20D’Agati V. Pathologic classification of focal segmental glomerulosclerosis. Semin Nephrol 2003;23:117-34.

21Kwon YH, Han SH, Kie JH, ve ark. Clinical features and outcomes of focal segmental glomerulosclerosis pathologic variants in Korean adult patients. BMC Nephrol 2014;15:52.

22Bradeb GL, Mulhern JG, O’Shea MH, ve ark. Changing incidence of glomerular diseases in adults. Am J Kidney Dis 2000;35:878-83.

23Kitiyakara C, Eggers P, Kopp JB. Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States. Am J Kidney Dis 2004;44:815-25.

24Chou YH, Lien YC, Hu FC, ve ark. Clinical outcomes and predictors for ESRD and mortality in primary GN. Clin J Am Soc Nephrol 2012;7:1401-8.

25De Vriese AS, Sethi S, Nath KA, Glassock RJ, Fervenza FC. Differentiating Primary, Genetic, and Secondary FSGS in Adults: A Clinicopathologic Approach. J Am Soc Nephrol 2018;29:759-74.

26Stiles KP, Abbott KC, Welch PG, Yuan CM. Effects of angiotensin-converting enzyme inhibitor and steroid therapy on proteinuria in FSGS: a retrospective study in a single clinic. Clin Nephrol 2001;56:89-95.

27Korbet SM. Angiotensin antagonists and steroids in the treatment of focal segmental glomerulosclerosis. Semin Nephrol 2003;23:219-28.

28D’Agati VD, Fogo AB, Bruijn JA, Jennette JC. Pathologic classification of focal segmental glomerulosclerosis: a working proposal. Am J Kidney Dis 2004;43:368-82.

29Fuiano G, Comi N, Magri P. Serial morphometric analysis of sclerotic lesions in primary “focal” segmental glomerulosclerosis. J Am Soc Nephrol 1996;7:49-55.

30Schwartz MM, Korbet SM. Primary focal segmental glomerulosclerosis: pathology, histological variants, and pathogenesis. Am J Kidney Dis 1993;22:874-83.

31Wehrmann M, Bohle A, Held H, ve ark. Long-term prognosis of focal sclerosing glomerulonephritis. An analysis of 250 cases with particular regard to tubulointerstitial changes. Clin Nephrol 1990;33:115-2.

Demographic, Clinicopathological and Laboratory Features of Focal Segmental Glomerulosclerosis Patients

ABSTRACT

References