Primary Focal Segmental Glomeruloslcerosis Pathologic Variants in Adults and Children
M Asgari, N Bavbek, AB Fogo. Iran University of Medical Sciences, Tehran, Islamic Republic of Iran; Vanderbilt University Medical Center, Nashville, TN
Background: The Colombia classification proposed five histopathologic variants of idiopathic Focal Segmental Glomeruloslcerosis (FSGS), namely collapsing (COLL), cellular (CELL), tip lesion variant (TIP), not otherwise specified (NOS) and perihilar (PH). Their clinical and prognostic implications have been examined in several biopsy series of adult patients, and these studies have shown worse prognosis of COLL, and better for TIP. We examined whether similar correlations were present for these different pathologic variants and their clinical characteristics and prognosis in biopsies from our practice, including both adult and children.
Design: All biopsies diagnosed as primary FSGS by LM, IF and EM in our referral practice from 1995 till 2006 were reviewed. Diagnosis was based on the presence of at least one segmentally sclerotic glomerulus, negative IF and extensive foot process effacement (>70%) without specific evidence of a secondary etiology. Biopsies were then classified by the Colombia Schema. Clinical history was reviewed and follow-up was classified as complete (CR) or partial remission (PR) of proteinuria, no remission (NR) or end stage renal disease (ESRD).
Results: 168 patients (92 male,74 female) met entry criteria. Average age was 42 ± 21 years (range 2 to 85) with 27 patients ≤ 18 year. 42 (25%) cases were African American and 86 (51%) were Caucasian, with ethnic origin other or not specified in the remaining patients. The frequency of FSGS variants was 13.7 % (N=23) COLL, 8.3% (N=14) CELL, 21.4% (N=36) TIP, 51.8 % (N=87) NOS, 4.8 % (N=8) PH. Black race showed more COLL and NOS compared to other variants and less in TIP. Edema and nephrotic proteinuria were present more in COLL and TIP. Hypertension and high serum creatinine at presentation were seen more in COLL and NOS. Outcome analysis of available data showed NR and ESRD more in COLL and CELL, compared to NOS and TIP. Conversely, NOS and TIP lesions had more PR or CR than other types. NOS was most common in children, and showed similar outcomes as in adults.
Conclusions: Our data support that different histopathologic variants have different clinical presentation and also different prognosis, regardless of age of patients.
Category: Kidney (does not include tumors)
Wednesday, March 24, 2010 1:00 PM
Poster Session VI # 244, Wednesday Afternoon