IgA nephropathy and granulomatosis with polyangiitis-overlap: a rare coexistence of two glomerular nephropathies with remission after steroids and rituximab

• Published in: Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia Vol. 40; no. 1
• Main Authors: Londrino, Francesco, Giuliani, Giovanni, Santostefano, Marisa, Baraldi, Olga, Mattiotti, Maria, Mangiulli, Marco, Tatangelo, Paola, Gambardella, Giorgia, Dominijanni, Sara, Napoli, Marianna, La Manna, Gaetano, Palumbo, Roberto
• Format: Journal Article
• Language: Italian
• Published: Italy 27.02.2023
• Subjects: Adult; Antibodies, Antineutrophil Cytoplasmic - therapeutic use; Glomerulonephritis; Glomerulonephritis, IGA - complications; Glomerulonephritis, IGA - drug therapy; Granulomatosis with Polyangiitis - complications; Granulomatosis with Polyangiitis - drug therapy; Humans; Kidney Glomerulus - pathology; Male; Rituximab - therapeutic use; Steroids; granulomatosis with polyangiitis; IgA nephropathy and granulomatosis with polyangiitis-overlap; ANCA-associated vasculitis; rituximab
• ISSN: 1724-5990

Granulomatosis with polyangiitis (GPA) is an ANCA-positive systemic vasculitis that mainly involves lungs and kidneys. This condition rarely overlaps with other glomerulonephritides. A 42-year-old man with constitutional symptoms and haemophtoe was admitted to the Infectious Diseases department, where he was subjected to fibrobronchoscopy with BAL (broncho-alveolar lavage) and lung transbronchial biopsy that showed histological signs of vasculitis. The association with severe acute kidney injury with urine sediment alterations (microscopic haematuria and proteinuria) led the consultant nephrologist to a diagnosis of GPA. Thus the patient was transferred to the Nephrology department. During the hospitalization, the worsening of the clinical course and the development of alveolitis, respiratory failure, purpura, and rapidly progressive kidney failure (nephritic syndrome - serum creatinine 3 mg/dl) required the start of steroid therapy, according to EUVAS. The presence of florid crescents in 3 out of 6 glomeruli in the renal biopsy and the IgA positive immunofluorescence allowed to make a diagnosis of overlap of GPA and IgA nephropathy. Rituximab (RTX 375 mg/m² per week for 4 weeks) and plasma exchange (7 sessions) were added to steroid therapy. During follow-up, partial functional recovery was achieved after 4 months, whereas total regression, i.e. the absence of protein and red blood cells in urine sediment, was reached during the 4-years follow-up. The main therapy during the first 2 years of follow-up was RTX, followed by mycophenolate mofetil for the remaining 2 years.