Introduction:
Coexistence of anti-glomerular basement membrane (anti-GBM) disease with anti-neutrophil cytoplasmic antibody (ANCA) in a case of glomerulonephritis is often identified as a 'double-positive' disease. The majority of 'double-positive' ANCA cases are associated with myeloperoxidase (MPO)-ANCA. Proteinase 3-ANCA (PR3-ANCA) positive diseases are less common in this context but have been reported. Patients positive for all three antibodies, known as 'triple-positive' patients, are extremely rare.
Methods:
We report a case of triple positivity hospitalized in the nephrology department of CHU Sahloul
Results:
A 29-year-old woman, with no significant medical history, working in a factory for 9 years specializing in the production of wires and cables for the mobile industry, presented with an intermittent cough unresponsive to symptomatic treatment, complicated by general malaise, acute kidney injury with creatinine at 1200 μmol/L, nephrotic syndrome: plasma protein at 53, albumin at 26.2, and proteinuria at 3g/24h, along with massive hemoptysis (hemoglobin 5.8 g/dl) and severe hypoxemia requiring invasive mechanical ventilation. Immunological testing revealed: Anti-MPO AC +++, Anti-PR3 AC+, Anti-MBG AC+, AAN- and native DNA-.
The patient required three sessions of hemodialysis, three boluses of 1g solumedrol followed by dexamethasone 1mg/kg/day, 6 boluses of cyclophosphamide, and eight plasmapheresis sessions. The evolution was rapidly favorable with a decrease in oxygen requirements without recurrence of alveolar hemorrhage. Extubation was performed on day 6 without incident. She presented as side effects thrombocytopenia at 53,000 elt/mm3 controlled at 157,000 and the last one done on 21/03/2024. The patient presented leukopenia at 2000 elt/mm3, the occurrence of a seizure at the end of the 5th plasmapheresis session with hypotension and increased oxygen requirements, explored by brain CT scan and MRI which returned without abnormalities and placed on Depakine.
Kidney biopsy showed extracapillary proliferation with fibrocellular and cellular crescents. There was a significant biological improvement: creatinine decreased from 1200 to 168 μmol/L, proteinuria negativization to 0.3g, and radiological clearance on the respiratory level. Maintenance treatment chosen was IMMUREL.
Conclusions:
This case highlights the natural course and management of a patient with crescentic glomerulonephritis and seropositivity for MPO-ANCA, PR3-ANCA, and anti-GBM antibody (triple-positive) who was successfully treated with methylprednisolone, cyclophosphamide and plasma exchange.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.