J LAM 1, D LANGSFORD , T PIANTA
1Northern Health, Epping, Australia
Aim: To analyse the long-term outcome of endovascular interventions on arteriovenous dialysis access in a nephrology led interventional service.
Background: IgG4 retroperitoneal fibrosis and related disease (IgG4-RD) obstructive uropathy is a rare cause of acute kidney injury (AKI). Positron emission tomography (PET) is reported to assist diagnosis of IgG4-RD yet its use to monitor disease progress has not been well described.
Case report:We describe a patient with an atrophic left kidney who developed IgG4-RD resulting in AKI from right sided obstructive uropathy. Five years earlier she had presented to another service with left renal obstruction caused by biopsy proven IgG4-RD. This was managed with JJ stent alone. She presented to our service with severe right hydroureter and hydronephrosis and serum creatinine 1067umol/L, having been 89umol/L 4 months previously. PET demonstrated markedly FDG-avid soft tissue posteriorly abutting the right ureter and a right lateral perinephric collection. With normal serum IgG4 levels, eosinophil count, complement C3 and C4, and fluctuating erythrocyte sedimentation rate and C-reactive protein there was no obvious reliable biomarker of disease activity.
A multidisciplinary approach to AKI involved ureterolysis with omental patch and initiation of weaning prednisolone. Creatinine stabilised at 93umol/L, however, four weeks after cessation of prednisolone creatinine was 142umol/L with recurrent FDG avidity on PET. Retreatment with weaning prednisolone followed by mycophenolate 1g BD was commenced and 6 months later there was no evidence of FDG avidity with creatinine of 103umol/L.
Conclusions:Our case study highlights the utility of serial PET to monitor patients with retroperitoneal IgG4-RD particularly when there are no reliable serum biomarkers
Joey is a general medical advanced physician trainee at Northern Health and has a keen interest in nephrology.