Focal segmental glomerulosclerosis: presenting features, treatment and outcomes in far north Queensland adults

Aim: To describe the clinical features, treatment and outcomes in adult patients with Focal Segmental Glomerulosclerosis (FSGS) presenting in Far North Queensland. \ud \ud Background: FSGS is a common cause of end stage kidney disease (ESKD) understudied within the Australian population. The incidence of ESKD attributed to FSGS is increasing worldwide. \ud \ud Methods: A retrospective review on 47 patients with biopsy confirmed FSGS between January 1997 and December 2013 at Cairns Hospital renal unit. Patients were located through the local biopsy database of 790 biopsies. Presenting features, secondary causes, prognostic factors, treatment modality and outcomes (doubling of serum creatinine, ESKD, death) were examined. \ud \ud Results: Patients were followed on average for 4.8 years (range 2 months -16 years). The mean age was 45 years with 50% of Aboriginal and Torres Strait Islander origin. Secondary causes included obesity (45%), hepatitis (6.8%) and additional glomerulonephritis (27%). FSGS not otherwise specified (NOS) was the most common variant (83%). Renin angiotensin system inhibitors and immunosuppression were commonly given (70% and 29% respectively). Of those given steroids, 45% responded and the remainder had an equal distribution of dependence or resistance. Eight patients died (17%), 14 doubled their serum creatinine (30%) and 13 progressed to ESKD (30%) within an average of 4.3 years. The number of patients was too few to make any statistically significant conclusions regarding prognostic factors. \ud \ud Conclusions: FSGS holds serious implications for patients with 40% progressing to the combined outcome. The Aboriginal and Torres Strait Islander community is over-represented in this population. Rates of immunosuppression treatment suggest either a significant proportion of secondary FSGS or under-treatment of primary FSGS in the region. Detailed description of prognostic factors remains to be clarified