Abstract: SA-PO262

A Two-Centre Cohort Experience of Anti-GBM Disease

Session Information

Category: Glomerular

  • 1005 Clinical Glomerular Disorders

Authors

  • Antonelou, Marilina, UCL centre for Nephrology, London, United Kingdom
  • Oliveira, Benjamin A., UCL centre for Nephrology, London, United Kingdom
  • Baumann, Astrid, Royal Free Hospital, London, United Kingdom
  • Blunden, Mark, Royal London Hospital, London, United Kingdom
  • Harber, Mark, UCL centre for Nephrology, London, United Kingdom
Background

The important determinant for the response of therapy and long term prognosis in anti-glomerular basement membrane (GBM) disease is early diagnosis. The aim of this study is to identify possible delays in the recognition and treatment of the disease and determine renal outcomes.

Methods

Retrospective review of cases of all patients identified as anti-GBM positive presenting in two tertiary referral centres and associated district hospitals between 1978 and 2016. Case notes, pathology archives and laboratory results were reviewed to collect demographic and clinical data at presentation and last follow-up.

Results

Forty nine patients presented across both sites with anti-GBM disease (28 at the Royal Free and 21 at the Royal London Hospital), 17 (34.7%) of which initially presented to a district hospital prior to transfer. Twenty five (51%) were male. Their median age was 58 (10-82) years and GBM titre 134(31-779) U/ml. Thirty (61%) had a renal biopsy. Seven (14%) had pulmonary haemorrhage at presentation. Figure1 shows different time interval points where delays can occur from presentation to treatment. The median follow up was 4 (0.5-37.9) years. 70% became dialysis dependent within a month of presentation. At last follow-up (n=41), 12(29.3%) were renal replacement therapy (RRT) dependent, 12(29.3%) had received a renal transplant and three (7.3%) were RRT independent.Fourteen (34,1%) patients died.

Conclusion

Patients with (GBM) disease are at increased risk of morbidity and mortality. Local review of clinical practice is crucial to avoid delays in establishing a diagnosis and initiating treatment.