Imane ELABOUDY, Mohamed Reda EL FAROUKI, Saida EL ASRI, Jamal Eddine NAJI, Mohamed EL HASSANI
Journal: Medpeer Publisher
ISSN: 3066-2737
Volume: 2
Issue: 5
Date of Publication: 2025/05/06
Introduction: Extracapillary glomerulonephritis (ECGN), or rapidly progressive glomerulonephritis (RPGN), is a major nephrological emergency associated with severe renal and vital prognosis, defined by rapid deterioration of renal function and the presence of glomerular crescents. Its classification is based on immunofluorescence (IF) (Type I anti-GBM, Type II immune complex, Type III pauci-immune/ANCA+). Prognosis depends on early diagnosis and immunosuppressive treatment. This study describes the characteristics and outcomes of ECGN in our center.
Methods: A retrospective single-center study was conducted at the Moulay Ismail Military Hospital in Meknes (Morocco). Nine consecutive adult patients diagnosed with ECGN between January 2022 and December 2024 were included. Descriptive analysis of demographic, clinical, biological, immunological, histological, therapeutic, and outcome data was performed.
Results: Nine patients (4 men, 5 women), mean age 68.9 years. The predominant etiology was pauci-immune ECGN (ANCA+) in 7 patients (78%; 4 Granulomatosis with Polyangiitis (GPA), 3 Microscopic Polyangiitis (MPA)/limited Vasculitis), followed by anti-Glomerular Basement Membrane (anti-GBM) antibody disease (Type I) in 2 patients (22%). Pulmonary-renal syndrome was frequent (56%). Initial renal failure was very severe: mean creatinine 670 µmol/L (75.9 mg/L), mean eGFR 7.2 mL/min/1.73m² (CKD-EPI), anuria in 33%. Renal biopsy (7/9 patients) showed an average of 66% crescents. Induction therapy included corticosteroids (9/9) and cyclophosphamide (8/9). No patients received plasma exchange (unavailability). Initial hemodialysis was required for 44%. Outcomes included 3 early deaths (33%, sepsis/respiratory distress), 4 progressions to ESKD on chronic dialysis (44%), 1 CKD stage 3b (11%), and 1 complete remission (11%). The combined rate of death or ESKD was 78%.
Conclusion: This series illustrates the severity of ECGN in our setting, affecting elderly individuals with very severe initial presentation and a predominance of pauci-immune forms. The high morbidity and mortality observed, despite treatment with corticosteroids and cyclophosphamide, highlight the potential impact of therapeutic limitations, notably the absence of plasma exchange. Early diagnosis and access to the full range of therapeutic resources are crucial to improve prognosis.
Extracapillary glomerulonephritis, Rapidly progressive glomerulonephritis, ANCA, Anti-GBM antibody, Vasculitis, Acute kidney injury, Renal biopsy, Morocco.
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