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Background In Australia, Lupus Nephritis (LN) affects a culturally diverse population of patients. We established the Lupus Nephritis Australian Registry (LUNAR) to better understand the population of patients receiving Myfortic and other immunosuppressants, and to analyse the safety and efficacy of the treatments they received. Methods A non-interventional, multicentre, registry of patients with biopsy-proven ISN/RPS class III, IV or V LN, treated with Myfortic or other immunosuppressants, was established. We collected baseline demographic and 6-monthly follow-up data over a 5-year period (2013-2018), including clinical data, laboratory tests and safety outcomes. Results 149 patients were enrolled in LUNAR across 8 sites, with 83.7% female and a mean age of 38.8 years. Most patients were Caucasian (45.2%) - patients of Asian ethnicity (29.6%) and Aboriginal/Torres Strait Islander or Maori/Pacific Islander descent (10.4%) were significantly over-represented compared to the general population. The mean and median duration of SLE was 8.6 and 6 years, respectively (range 0-42 years), and of LN was 5.4 and 3.9 years, respectively (range 0-30 years). Most patients had class IV LN on their initial kidney biopsy (59.3%), with 21.5% having class III and 11.1% having class V LN. Immunosuppressants used prior to screening included corticosteroids (68.9%), mycophenolate mofetil ((MMF) 61.5%), Myfortic (41.5%) and cyclophospha-mide (32.6%). At enrolment or during the study, most patients were treated with Myfortic (76.3%), MMF (65.2%) and/or hydroxychloroquine (67.4%). Rituximab and azathio-prine were each started in 8.9% of patients during the study period. Kidney function was stable/improved for most patients over the study period and mycophenolate-based therapy was well-tolerated. Conclusions LUNAR is the first study outlining the demographics, outcomes and practice patterns in the management of patients with LN in Australia.

Background In Australia, Lupus Nephritis (LN) affects a culturally diverse population of patients. We established the Lupus Nephritis Australian Registry (LUNAR) to better understand the population of patients receiving Myfortic and other immunosuppressants, and to analyse the safety and efficacy of the treatments they received. Methods A non-interventional, multicentre, registry of patients with biopsy-proven ISN/RPS class III, IV or V LN, treated with Myfortic or other immunosuppressants, was established. We collected baseline demographic and 6-monthly follow-up data over a 5-year period (2013-2018), including clinical data, laboratory tests and safety outcomes. Results 149 patients were enrolled in LUNAR across 8 sites, with 83.7% female and a mean age of 38.8 years. Most patients were Caucasian (45.2%) - patients of Asian ethnicity (29.6%) and Aboriginal/Torres Strait Islander or Maori/Pacific Islander descent (10.4%) were significantly over-represented compared to the general population. The mean and median duration of SLE was 8.6 and 6 years, respectively (range 0-42 years), and of LN was 5.4 and 3.9 years, respectively (range 0-30 years). Most patients had class IV LN on their initial kidney biopsy (59.3%), with 21.5% having class III and 11.1% having class V LN. Immunosuppressants used prior to screening included corticosteroids (68.9%), mycophenolate mofetil ((MMF) 61.5%), Myfortic (41.5%) and cyclophospha-mide (32.6%). At enrolment or during the study, most patients were treated with Myfortic (76.3%), MMF (65.2%) and/or hydroxychloroquine (67.4%). Rituximab and azathio-prine were each started in 8.9% of patients during the study period. Kidney function was stable/improved for most patients over the study period and mycophenolate-based therapy was well-tolerated. Conclusions LUNAR is the first study outlining the demographics, outcomes and practice patterns in the management of patients with LN in Australia.

Background: Primary oxaloses are rare genetic disorders that result in hepatic overproduction of oxalate. Primary hyperoxaluria type1(PH1) is themost common and severe form. Clinical manifestations range from renal calculi to progressive renal impairment and systemic deposition of oxalate. Supportive measures may slow kidney disease progression but many cases develop end stage renal disease(ESRD). Combined liver-kidney transplantation may be curative. Case Report: A 65yo male had developed ESRD in 1990 and underwent deceased-donor kidney transplantation in Holland in 2000. The transplant failed within a week, histology revealed oxalate crystals and a diagnosis of PH1 was made by genetic analysis revealing compound pyridoxine-sensitive mutations in the AGXT gene. The patient returned to haemodialysis for 8 years then presented to our unit for simultaneous kidney-liver transplantation( SLK). Aiming to prevent early disease recurrence, Continuous Renal Replacement Therapy(CRRT) was performed during surgery followed by daily CRRT for nine days. Liver allograft functionwas achieved, however the kidney experienced delayed graft function. A renal biopsy showed only acute tubular necrosis and intermittent haemodialysis was continued for a further week by which time kidney function was evident. The patient was discharged on day 26, creatinine 178umol/L. On day 29, creatinine was 202umol/L and a renal biopsy revealed focal oxalate crystals affecting 10% of kidney. A Repeat biopsy at day 39 showed progressive oxalate deposition. Daily dialysis was re-established followed by slow recovery of kidney allograft function. Conclusion(s): Data surrounding transplantation for primary hyperoxyluria are limited to small case series. Risk of disease recurrence caused by renal excretion of residual systemic oxalate may occur despite SLK preventing further production. More data on how to assess oxalate burden and prevent recurrence are required.

Background and Aims: BK virus is an important cause of renal allograft loss in the renal transplant population. There is limited data regarding intermediate to long-term outcomes in patients with BK viraemia. Effects of routine screening are not well established, with prevalence in prospectively screened cohorts ranging from 11 to 43%. Our aim was to describe the burden of BK viraemia (BKV) and BK nephropathy (BKN) in a large single transplant centre. Method(s): We conducted a retrospective cohort study of 526 patients transplanted between 2008 and 2015, when routine screening (at 3 and 12 months post transplantation) was established. Result(s): 71 patients (13%) developedBKVand 20 (4%) developed BKN during the study period. The median follow-up was 50.9 months (IQR 28.4-82.2) with a minimum follow-up of 12 months. More than 95% of patients were screened for BK at 3 months. All but two patients with BKN had intermediate or high levels of BKV>1000copies/ml. Themajority of patients had basiliximab induction (73%) and maintenance tacrolimus, mycophenolate and prednisolone (94%). Race was strongly associated with BKV (p<0.001), with Asian/ Indian and Aboriginal/Pacific Islander groups both having an increased risk compared to Caucasian (OR Asian/Indian compared to Caucasian 2.49; 95%CI 1.20-5.16). Therewas a trend toward higher rates ofBKV with increasing HLA mismatch (p=0.065). Acute rejection and thymoglobulin use were not associated with BKV (OR 1.16, p=0.751; OR 0.71, p=0.349 respectively). Conclusion(s): BKV was detected in 13%of our cohort and Asian and Indian race was associated with a significantly increased risk. Routine screening for BKV is effective and enables optimal management of immunosuppression to minimise progression.

Background: Primary oxaloses are rare genetic disorders that result in hepatic overproduction of oxalate. Primary hyperoxaluria type1(PH1) is themost common and severe form. Clinical manifestations range from renal calculi to progressive renal impairment and systemic deposition of oxalate. Supportive measures may slow kidney disease progression but many cases develop end stage renal disease(ESRD). Combined liver-kidney transplantation may be curative. Case Report: A 65yo male had developed ESRD in 1990 and underwent deceased-donor kidney transplantation in Holland in 2000. The transplant failed within a week, histology revealed oxalate crystals and a diagnosis of PH1 was made by genetic analysis revealing compound pyridoxine-sensitive mutations in the AGXT gene. The patient returned to haemodialysis for 8 years then presented to our unit for simultaneous kidney-liver transplantation( SLK). Aiming to prevent early disease recurrence, Continuous Renal Replacement Therapy(CRRT) was performed during surgery followed by daily CRRT for nine days. Liver allograft functionwas achieved, however the kidney experienced delayed graft function. A renal biopsy showed only acute tubular necrosis and intermittent haemodialysis was continued for a further week by which time kidney function was evident. The patient was discharged on day 26, creatinine 178umol/L. On day 29, creatinine was 202umol/L and a renal biopsy revealed focal oxalate crystals affecting 10% of kidney. A Repeat biopsy at day 39 showed progressive oxalate deposition. Daily dialysis was re-established followed by slow recovery of kidney allograft function. Conclusion(s): Data surrounding transplantation for primary hyperoxyluria are limited to small case series. Risk of disease recurrence caused by renal excretion of residual systemic oxalate may occur despite SLK preventing further production. More data on how to assess oxalate burden and prevent recurrence are required.

Background and Aims: BK virus is an important cause of renal allograft loss in the renal transplant population. There is limited data regarding intermediate to long-term outcomes in patients with BK viraemia. Effects of routine screening are not well established, with prevalence in prospectively screened cohorts ranging from 11 to 43%. Our aim was to describe the burden of BK viraemia (BKV) and BK nephropathy (BKN) in a large single transplant centre. Method(s): We conducted a retrospective cohort study of 526 patients transplanted between 2008 and 2015, when routine screening (at 3 and 12 months post transplantation) was established. Result(s): 71 patients (13%) developedBKVand 20 (4%) developed BKN during the study period. The median follow-up was 50.9 months (IQR 28.4-82.2) with a minimum follow-up of 12 months. More than 95% of patients were screened for BK at 3 months. All but two patients with BKN had intermediate or high levels of BKV>1000copies/ml. Themajority of patients had basiliximab induction (73%) and maintenance tacrolimus, mycophenolate and prednisolone (94%). Race was strongly associated with BKV (p<0.001), with Asian/ Indian and Aboriginal/Pacific Islander groups both having an increased risk compared to Caucasian (OR Asian/Indian compared to Caucasian 2.49; 95%CI 1.20-5.16). Therewas a trend toward higher rates ofBKV with increasing HLA mismatch (p=0.065). Acute rejection and thymoglobulin use were not associated with BKV (OR 1.16, p=0.751; OR 0.71, p=0.349 respectively). Conclusion(s): BKV was detected in 13%of our cohort and Asian and Indian race was associated with a significantly increased risk. Routine screening for BKV is effective and enables optimal management of immunosuppression to minimise progression.