L LSUHSC-S Renal Pathology Consultative Services

Interesting Case

Case Study #: 5

09/14/09 Answers

By: Stephen M. Bonsib, M.D.

Xin Gu, M.D.

Ami Bhalodia, M.D.

318-675-4557 phone

318-675-4541 fax

sbonsi@lsuhsc.edu

Clinical History: A twenty-one year old African American female presenting with nephrotic syndrome, 8 grams of proteinuria, and a creatinine of 1.1.

The patient has no history of hypertension and multiple serologies are negative.

Case courtesy of Dr. Kenneth Abreo, MD and Dr. Mary Buffington, MD

Figure 1: Silver stain of a glomerulus, where is the lesion located?

Figure 2: Silver stain highlighting a lesion; what is it near?

Figure 3: IF for IgG

Figure 4: EM of the urinary pole of the glomerulus

Answers:

The glomerulus shows a small area of segmental sclerosis that is localized to the ostia of the proximal tubule.

2.

The IgG staining on IF represents protein resorption droplets within the podocyte cytoplasm.

3.

Focal segmental glomerulosclerosis, glomerular tip variant. 4.

Of all the FSGS variants, the glomerular tip lesion has a more favorable prognosis with the highest rate of renal survival at 3 years (76%) and 5 years (76%) according to a study by DB Thomas et al. They also noted the least severe tubulointerstitial injury and arteriosclerosis in patients with the glomerular tip variant of FSGS.

References:

Thomas DB, Franceschini N, Hogan SL, Ten Holder S, Jennette CE, Falk RJ, Jennette JC. Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. Kidney Int. 2006 Mar; 69(5):920-6.

Howie AJ, Pankhurst T, Sarioglu S, Turhan N, Adu D. Evolution of nephrotic-associated focal segmental glomerulosclerosis and relation to the glomerular tip lesion. Kidney Int. 2005 Mar; 67(3):987-1001.

Chun 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 Aug;15(8):2169-77.

Stokes MB, Markowitz GS, Lin J, Valeri AM, D'Agati VD. spectrum. Kidney Int. 2004 May; 65(5):1690-702.

Haas M, Yousefzadeh N. Glomerular tip lesion in minimal change nephropathy: a study of autopsies before 1950. Am J Kidney Dis. 2002 Jun; 39(6):1168-75.