title: Clinical presentation, treatment and outcome of focal segmental glomerulosclerosis in Far North Queensland Australian adults.
abstract: OBJECTIVE
  The aim is to describe the clinical features, treatment and outcomes in Australian adults with focal segmental glomerulosclerosis and identify predictors of disease progression and all-cause mortality.
  
  
  METHODS
  The study included all patients with biopsy confirmed focal segmental glomerulosclerosis between January 1997 and June 2014 at participating hospitals. Clinical factors, histopathological findings, biochemical markers and treatments were analysed and potential predictors of doubling serum creatinine, end stage kidney disease or death identified.
  
  
  RESULTS
  A total of 98 patients were included with a median follow up of 4.3 years. Thirty-four (35%) patients were Aboriginal or Torres Strait Islander. Focal segmental glomerulosclerosis not-otherwise-specified was the most common variant. Seventeen (59%) patients initially treated with immunosuppression experienced an improvement in renal function. At the end of follow up, 43 (44%) patients had progressed to the composite outcome. Baseline tubulointerstitial scarring and lower haemoglobin predicted shorter time to doubling serum creatinine. Dual diagnosis, higher serum creatinine, lower estimated glomerular filtration rate and doubling creatinine were associated with shorter time to end stage kidney disease with remission the only protective factor. Age was the only variable associated with all-cause mortality.
  
  
  CONCLUSIONS
  Focal segmental glomerulosclerosis holds serious implications for patients. Concomitant diabetic nephropathy, higher serum creatinine and lower estimated glomerular filtration rate at renal biopsy were associated with poorer renal prognosis. Indigenous people had a female predominance and are over-represented in relation to their population size, however, were not associated with poorer prognosis. Remission was the only modifiable variable and thus should be at the forefront of patient management goals and future studies.
authors.affiliation: College of Medicine and Dentistry, James Cook University, Cairns, Queensland, Australia.|Department of Renal Medicine, Cairns Hospital, Cairns, Queensland, Australia.
authors.forename: Alice M
authors.initials: AM
authors.lastname: Greenwood
authors.affiliation: College of Medicine and Dentistry, James Cook University, Cairns, Queensland, Australia.|Research and Development Unit, Primary Health Care and Dental Care, Southern Älvsborgy, Västra Götaland, Sweden.|Department of Public Health and Community Medicine, Institute of Medicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
authors.forename: Ronny
authors.initials: R
authors.lastname: Gunnarsson
authors.affiliation: College of Medicine and Dentistry, James Cook University, Cairns, Queensland, Australia.|The George Institute for Global Health, Royal Prince Alfred Hospital, Camperdown, NSW.
authors.forename: Brendon L
authors.initials: BL
authors.lastname: Neuen
authors.affiliation: Pathology Queensland, Queensland Health, Brisbane, Queensland, Australia.
authors.forename: Kimberley
authors.initials: K
authors.lastname: Oliver
authors.affiliation: Department of Renal Medicine, Cairns Hospital, Cairns, Queensland, Australia.
authors.forename: Stella J
authors.initials: SJ
authors.lastname: Green
authors.affiliation: Department of Renal Medicine, Cairns Hospital, Cairns, Queensland, Australia.
authors.forename: Richard A
authors.initials: RA
authors.lastname: Baer
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers / Q000097:blood; D003404:Creatinine / Q000097:blood; D018450:Disease Progression; D005260:Female; D005919:Glomerular Filtration Rate; D005923:Glomerulosclerosis, Focal Segmental / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007668:Kidney / Q000187:drug effects* / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D011793:Queensland; D020127:Recovery of Function; D012074:Remission Induction; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D015854:Up-Regulation; D055815:Young Adult
chemical_list: D015415:Biomarkers; D007166:Immunosuppressive Agents; D003404:Creatinine
keywords: adult; end stage kidney disease; focal segmental glomerulosclerosis; nephrotic syndrome; prognosis