Introduction:
Acute Glomerulonephritis due to anti GBM disease is a rare disease with overall incidence of 1 case per million case of GN. Almost one third of these patients have concurrent ANCA positivity. Isolated ANCA associated vasculitis is equally rare with 33 case per million case of GN and around 10% having dual positivity for anti GBM as well. Here is an 8 case series of dual positive ANCA anti GBM disease with special focus on their clinicopathologic characteristics and outcome over a period of 10 years.
Methods:
Available records of all ANCA associated vasculitis patients was assessed retrospectively to look for double positivity for ANCA and anti GBM disease between 2015 to 2024 being treated at our institution.
Results:
Among these 8 patients, 6 were females and 2 males; average age 44.8 years; mean serum Creatinine at presentation 8mg/dl; proteinuria: 2.5g; 3 had diffuse alveolar hemorrhage and 4 were dialysis requiring; 5 patients were positive for both ANCA (2 positive for PR3 and 6 positive for MPO) and anti GBM serologically while 3 were single positive only for MPO but had linear anti GBM IgG deposit on renal biopsy. Renal biopsy was done in 6 out of 8 patients. 5 patients recieved plasmapheresis, 5 Cyclophosphamide and 1 Rituximab therapy.
Conclusions:
Double positivites are mixed phenotype that needs to be treated aggressively for the best possible outcome.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process