Dr CHATHRI RATNAYAKE1, Dr THEODORE CROMBIE1, Dr MARGARET FRAENKEL1, Proferssor LAWRENCE P. MCMAHON1,2, Dr LIMY WONG1,2
1Department of Renal Medicine, Eastern Health, , Australia, 2Department of Renal Medicine, Monash University Eastern Health Clinical School, , Australia
Background
Double-seropositive vasculitis is defined as coexistence of both anti-neutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane (anti-GBM) antibodies. Consistent with the low recurrence rate of anti-GBM disease, seropositive relapses are nearly always related to the ANCA-associated vasculitis (AAV). The role of plasmapheresis (PLEX) in the management of AAV remains controversial given the recent landmark PEXIVAS trial demonstrating no benefit of routine use in AAV patients. We describe a rare case of AAV relapse five years post-kidney transplant, which was successfully treated with PLEX.
Case Report
A 64-year-old Caucasian man presented to the emergency department with productive cough, haemoptysis and worsening dyspnoea. He had been diagnosed with double-seropositive anti-GBM and anti-myeloperoxidase (MPO) glomerulonephritis seven years ago. He was dialysis-dependent then with 100% glomerular crescent formation on kidney biopsy but no lung involvement. Both PLEX and cyclophosphamide treatment were deemed futile and discontinued. Accordingly, he remained dialysis-dependent and subsequently had a deceased donor kidney transplant two years later.
On arrival, he was noted to be febrile and in respiratory distress. Initial investigations revealed severe anaemia, with elevated inflammatory markers and acute kidney injury. Chest imaging showed extensive pulmonary infiltrates suggestive of pulmonary haemorrhage, infection or inflammation. He was started on methylprednisolone, broad-spectrum antibiotics and PLEX. He required a short period of invasive ventilatory support following bronchoscopy, which confirmed diffuse alveolar haemorrhage. Anti-MPO antibodies were positive. He was commenced on cyclophosphamide and mycophenolate mofetil was ceased. He completed ten sessions of PLEX and was discharged home one week later with no further haemoptysis and improved kidney function.
Conclusions
Relapse of AAV post-kidney transplant is uncommon and a selective group of AAV patients might benefit from PLEX treatment.
Biography:
Chathri is a renal advanced trainee from Victoria who maintains a keen interest in all aspects of nephrology. She also takes an interest in mentoring and educating the junior medical doctors.