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Abstract: PO1839

Rituximab in IgA Vasculitis with Aggressive Glomerulonephritis: A Real-Life Experience

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Fenoglio, Roberta, 1Nephrology and Dialysis Unit & CMID (Center of Research of Immunopathology and Rare Diseases), Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital of Turin, and Department of Clinical and Biological Sciences, University of Turin, Turin, Italy, Turin, Italy, Italy
  • Sciascia, Savino, 1Nephrology and Dialysis Unit & CMID (Center of Research of Immunopathology and Rare Diseases), Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital of Turin, and Department of Clinical and Biological Sciences, University of Turin, Turin, Italy, Turin, Italy, Italy
  • Roccatello, Dario, 1Nephrology and Dialysis Unit & CMID (Center of Research of Immunopathology and Rare Diseases), Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital of Turin, and Department of Clinical and Biological Sciences, University of Turin, Turin, Italy, Turin, Italy, Italy
Background

IgA-vasculitis (IgAV) is a systemic small vessels vasculitis characterized by deposition of underglycosylated IgA1 immune complexes. Presently, no treatment is specifically recommended in IgAV Glucocorticoids (GC) have been traditionally thought to be effective in tempering systemic symptoms, but did not show long-term benefits either in reducing flares or progression of kidney disease. Recently Rituximab (RTX) has been proved to be effective in a few case series of adults with IgAV. Aim of the study: to evaluate the effectiveness of RTX as first line therapy in induction and maintenance of remission of adults with IgAV with biopsy-proven crescentic glomerulonephritis.

Methods

We reviewed the clinical records of patients (pts) with adult-onset IgAV treated with RTX at our Center. Patients included 8 males and 4 females, mean age 45 years with mean follow-up duration of 31 months. All pts had a biopsy proven IgAV- severe nephritis. Pts received 4 weekly doses of RTX given alone (8 pts) or in combination with CS (4 pts). Disease activity was evaluated by BVAS version 3 at the onset and at 1, 6 and 12 months and at the end of follow up. Complete remission (CR) was defined as BVAS of 0.

Results

Eleven pts (91.7%) achieved a clinical response at 6 months. 10 pts had a CR while 1 pt had a partial response and was given an additional dose of RTX after 12 months from induction due to persistent proteinuria (1gr/24 hrs), despite systemic remission. He achieved a CR 6 months later. One patient did not respond to RTX and was switched to MMF. Among the 10 pts with CR, 1 patient needed maintenance doses of RTX every 6 months due to relapse of palpable purpura; 1 relapsed after 15 months and received a new induction course showing a CR again.
Significant decrease in 24-hour proteinuria, BVAS,and CRP level was detected. RTX was generally well tolerated. One patient, who had a CR with RTX alone died after 6 months of follow-up for cardiovascular cause.

Conclusion

This extended experience confirms our initial results supporting the use of RTX in the treatment of IgAV with severe renal involvement. Indeed, RTX proved to be effective and safe for induction and maintenance of long-lasting remission. Present data also suggest that RTX is not only effective for severe and refractory IgAV, but can be also proposed as a first line therapy.