Dr Adrienne Cohen1, Dr ESWARI VILAYUR1,2
1John Hunter Hospital, New Lambton, Australia, 2School of Medicine and Public Health, University of Newcastle, Newcastle, Australia
Background: Fibrillary and immunotactoid glomerulonephritis are uncommon. Fibrillary glomerulonephritis (FGN) is often renal-limited, whereas immunotactoid glomerulopathy (IT) is more associated with haematological disorders. There is limited literature on the natural history of these glomerulopathies in Australia.
Aims: We aim to study the prevalence, clinicopathological correlation and outcomes of FGN and IT in our regional centre.
Methods: A local renal biopsy database was interrogated for cases of fibrillary and immunotactoid glomerulopathies from 2000-2020. Data collected includes demographics, serum creatinine, haematuria status, proteinuria, comorbidities, and histopathological findings.
Results: Out of 4083 native and transplant kidney biopsies, we had 14 cases of FGN (biopsy incidence 0.34%) and 2 cases of IT (biopsy incidence 0.04%). The mean age at presentation was 59.8yrs. 42.9% were males. No patients with FGN had dysproteinaemia, whereas both patients with IT had chronic lymphocytic leukaemia. 75% of patients with FGN and all patients with IT had haematuria. All patients had proteinuria. Mean presenting albuminuria was 254mg/mmol for FGN and 604mg/mmol for IT. Mean presenting serum creatinine and eGFR were 149umol/L and 48mL/min respectively for FGN and 95umol/L and 72mL/min for IT.
The prognosis of FGN was poor with limited response to immunomodulatory therapy; six patients (46.2%) reached end-stage kidney disease (ESKD) after a median of 42 months (1-96 months). High-grade presenting proteinuria led to poor renal outcomes. Patients with IT had disease remission with treatment of their underlying haematological disease.
Conclusions: FGN is rare, with poor response to immunomodulatory therapy. It carries poor renal prognosis. Less proteinuria at diagnosis may predict a more benign disease course. IT is associated with haematological malignancy and carries better prognosis and response to treatment.
Biography:
Adrienne is a second year Nephrology Advanced Trainee, currently at John Hunter Hospital in Newcastle. She is passionate about optimising the quality of care we deliver to our patients.