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Kidney Week

Abstract: SA-PO0866

Clinical Features, Histopathology, and Outcomes of Fibrillary Glomerulonephritis: A Spanish Multicenter Cohort

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Tatis, Efrain, Hospital Universitari Vall d'Hebron, Barcelona, CT, Spain
  • Maskani, Ines, Fundacio Puigvert, Barcelona, CT, Spain
  • Domínguez Guasch, Anna, Fundacio Puigvert, Barcelona, CT, Spain
  • Moliz, Candela, Hospital Regional Universitario de Malaga, Málaga, AL, Spain
  • González Cabrera, Fayna, Hospital Universitario de Gran Canaria Dr Negrin, Las Palmas de Gran Canaria, CN, Spain
  • Cases Corona, Clara Maria, Hospital Universitario Fundacion Alcorcon, Alcorcón, Community of Madrid, Spain
  • Ferri, Josefa Belen, Hospital Clinico Universitario Virgen de la Arrixaca, El Palmar, Región de Murcia, Spain
  • Cabello Pelegrin, Sheila, Hospital Universitari Son Espases, Palma, Mallorca, Spain
  • Cannata-Ortiz, Pablo J., Hospital Universitario Fundacion Jimenez Diaz, Madrid, Community of Madrid, Spain
  • Osma, July Vanessa, Hospital Arnau de Vilanova, Valencia, Valencian Community, Spain
  • Santos Collado, Natalia, Hospital Universitario Virgen del Rocio, Seville, AL, Spain
  • De La Flor, José C., Hospital Central de la Defensa Gomez Ulla, Madrid, Madrid, Spain
  • Huerta, Ana, Hospital Universitario Puerta de Hierro Majadahonda, Majadahonda, MD, Spain
  • Poma Saavedra, Fabrizzio, Hospital Comarcal de Vinaros, Vinaròs, Valencian Community, Spain
  • Rodriguez Magariños, Catuxa, Complexo Hospitalario Universitario A Coruna Biblioteca, A Coruña, GA, Spain
  • Carbayo, Javier, Hospital General Universitario Gregorio Maranon, Madrid, Community of Madrid, Spain
  • Soler Romeo, Maria Jose, Hospital Universitari Vall d'Hebron, Barcelona, CT, Spain
Background

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease with poor renal prognosis and limited therapeutic evidence. This study analyzed clinical and histopathological features, predictors of renal progression, and the impact of rituximab (RTX) in a Spanish multicenter cohort.

Methods

Retrospective multicenter study including 98 patients with biopsy-proven FGN from 15 Spanish hospitals. Outcomes were ESKD, mortality, and a composite of ESKD or ≥50% eGFR decline. Cox regression identified predictors of progression.

Results

Mean age was 60.3±10.9 years, median eGFR 40.5 mL/min/1.73 m2, and median proteinuria 3.25 g/day. During 32.2 months of follow-up, 36.7% progressed to ESKD and 15.3% died. RTX was associated with lower risk of ESKD (HR 0.36, 95% CI 0.16–0.80) and the composite renal outcome (HR 0.49, 95% CI 0.25–0.96), while higher creatinine, nephrotic-range proteinuria, and tubular atrophy predicted worse renal outcomes.

Conclusion

Baseline renal dysfunction, nephrotic-range proteinuria, and tubular atrophy were associated with poor prognosis in FGN. RTX therapy was associated with reduced renal progression, supporting its potential role in the management of this disease.

Baseline characteristics according to rituximab treatment
VariableAll (n=98)RTX (n=48)No RTX (n=50)p-value
Age (years), mean ± SD60.3 ± 10.962.3 ± 11.558.4 ± 10.00.077
Male, n (%)49 (50.0)26 (54.2)23 (46.0)0.683
Hypertension, n (%)75 (76.5)38 (79.2)37 (74.0)0.715
Nephrotic syndrome, n (%)22 (22.7)9 (19.1)13 (26.0)0.574
Serum creatinine (mg/dL)1.60 (1.06–2.00)1.54 (1.16–2.00)1.65 (1.00–2.22)0.867
eGFR (mL/min/1.73m2)40.5 (28.3–65.8)40.0 (30.0–63.0)41.5 (26.3–70.0)0.962
Proteinuria (mg/day)3250 (1231–5925)3200 (1235–5440)3357 (1230–6000)0.855
Serum albumin (g/dL)3.9 (3.1–4.5)4.1 (3.4–4.9)3.7 (2.9–4.2)0.017
Hematuria, n (%)60 (61.9)32 (66.7)28 (57.1)0.449

Abbreviations: SD = standard deviation; eGFR = estimated glomerular filtration rate; Continuous variables are presented as mean ± SD or median (P25–P75), as appropriate. Categorical variables are presented as n (%).