Jamal Eddine NAJI, Hicham RAFIK, Mohamed Reda ELFAROUKI, Imane ELABOUDY, Mohamed HASSANI
Journal: Medpeer Publisher
ISSN: 3066-2737
Volume: 2
Issue: 2
Date of Publication: 2025/02/08
Introduction : Neutrophil cytoplasmic antibody (NCA)-associated vasculitides are rare systemic disorders characterized by pauci-immune necrotizing glomerulonephritis with extracapillary proliferation. IgA nephropathy is characterized by the presence of mesangial IgA deposits, which can lead to rapidly progressive glomerulonephritis. The association of the two entities is rarely described.
Observation : We report the case of a 65-year-old patient admitted for generalized petechial purpura with macroscopic hematuria and renal failure, who underwent renal biopsy revealing IgA mesangial deposits nephropathy at an advanced stage of sclerosis. The patient was put on chronic hemodialysis. Six years later, he was hospitalized for the therapeutic management of pulmonary tuberculosis. After two months of antibacillary treatment, the patient presented with hemoptysis associated with extensive intra-alveolar hemorrhage. Immunological tests revealed highly positive p-ANCA with MPO specificity (>135 IU/mL). The patient was put on corticosteroids and Cyclophosphamide with complete remission, followed by Azathioprine as maintenance therapy.
Discussion: ANCA-positive patients with IgA nephropathy have a more severe clinical presentation and histological lesions, but a better response to treatment. The therapeutic management of this association has not been codified.
Conclusion: The association of IgA nephropathy and ANCA vasculitis is exceptional. One hypothesis suggests the presence of IgA-isotype ANCA, responsible for degranulation of polynuclear cells and interaction with mesangial cells. As the treatment of this association has not been codified, most of the protocols used are similar to those for ANCA vasculitis.
IgA Nephropathy, ANCA Vasculitis, Association
Download PDF