Abstract
Background: There is no information on the frequency and clinicopathological presentation
of the variants of primary focal segmental glomerulosclerosis (FSGS) in adults
presenting with idiopathic nephrotic syndrome (INS) in Pakistan.
Objectives: The aim of this study was to determine the frequencies of different histologic
variants of primary FSGS with INS at our center and to compare our findings
with those published in literature.
Patients and Methods: All consecutive adults (≥18 years) with INS, and diagnosis of
FSGS on renal biopsies, were included. Their clinicopathological features at the time
of presentation were retrieved and compared among the variants.
Results: There were 120 (65.2%) males and 64 (34.8%) females. The mean age
was 30.62±12.02 years. The mean 24-hr urinary protein excretion was 4.69±2.36
grams. Microscopic hematuria was found in 30 (16.3%) patients. The mean serum
creatinine was 1.58±0.87 mg/dL. At presentation, 128 (69.6%) patients were
normotensive, while 56 (30.4%) exhibited hypertension. FSGS, not otherwise
specified (NOS) was the predominant variant, comprising 76.6% of all; collapsing
variant comprised 12%, tip variant, 9.8%, perihilar, 1.1%, and cellular, 0.5%. The
mean number of glomeruli involved by segmental scarring was 3.41±2.87 and
there was significant difference among the variants (p= 0.001). Arteriolopathy was
found in 23.4 % cases and fibrointimal thickening of arteries in 18.5%. Tubular
atrophy and interstitial fibrosis (IF/TA) was noted in 93% of cases. There was no
significant difference in vasculopathy and IF/TA among the variants.
Conclusions: Collapsing variant was the second most common variant following NOS
and these findings are different from other regional studies.