José Hernández-Rodríguez1, Cristina Carbonell2, José-A Mirón-Canelo3, Sandra Diez-Ruiz4, Miguel Marcos5, Antonio J Chamorro6. 1. Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic of Barcelona, University of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain. 2. Conective Tissue Diseases Unit, Department of Internal Medicine, Hospital Clínico Universitario de Salamanca, Salamanca, Spain. 3. Department of Preventive Medicine and Public Health, Faculty of Medicine, University of Salamanca, Salamanca, Spain; Institute of Biomedical Research of Salamanca (IBSAL), Salamanca, Spain. 4. Department of Medicine, Faculty of Medicine, University of Salamanca, Salamanca, Spain. 5. Conective Tissue Diseases Unit, Department of Internal Medicine, Hospital Clínico Universitario de Salamanca, Salamanca, Spain; Institute of Biomedical Research of Salamanca (IBSAL), Salamanca, Spain; Department of Medicine, Faculty of Medicine, University of Salamanca, Salamanca, Spain. 6. Conective Tissue Diseases Unit, Department of Internal Medicine, Hospital Clínico Universitario de Salamanca, Salamanca, Spain; Institute of Biomedical Research of Salamanca (IBSAL), Salamanca, Spain; Department of Medicine, Faculty of Medicine, University of Salamanca, Salamanca, Spain. Electronic address: ajchamorro@usal.es.
Abstract
BACKGROUND: Immunoglobulin A vasculitis (IgAV) is a systemic small vessel vasculitis for which treatment of severe cases is usually based on glucocorticoids and other conventional immunosuppressive drugs. The role of rituximab for resistant or refractory cases has been explored in isolated case reports and small series. AIMS: To perform a literature review of all pediatric and adult patients with IgAV treated with rituximab (RTX) and to assess disease characteristics, RTX efficacy and safety. METHODS: We conducted a systematic literature review according to PRISMA guidelines by selecting articles with information on IgAV and RTX up to October 2019. We extracted data on patient characteristics, disease course, RTX efficacy and tolerance. The resulting database was analyzed with statistical software package SPSS v 22.0. RESULTS: Among the initial 161 articles found, 20 studies including 35 well-characterized IgAV patients treated with RTX were finally analyzed. Distribution by sex was similar, and the median age at diagnosis was 26 (range: 2 months to 70 years). Patients included were equally diagnosed at pediatric age and in the adulthood. Almost 90% of patients had renal involvement before RTX treatment and resistant or refractory disease to glucocorticoids or other immunosuppressive agents, mainly with renal impairment, was the reason for RTX administration in 85.7% of patients. RTX was used because of contraindication to these previous agents in 8.6% of patients, and as first line therapy in 5.7% of them. With regard to RTX response, 94.3% of patients presented clinical improvement of any type and 74.3% achieved sustained remission at the end of follow-up. Among the 13 (37.1%) patients who experienced a disease relapse, 11 (31.4%) were treated with a new RTX dose, with good disease control in all cases. In terms of treatment requirements, glucocorticoids and additional immunosuppressants were significantly lower after RTX administration. No deaths were observed and the rate of minor RTX-associated adverse effects was of 8.6%. CONCLUSION: RTX seems to be a safe and useful agent in inducing disease remission and reducing previous immunosuppressive treatment in IgAV pediatric and adult patients resistant or refractory to glucocorticoids or other immunosuppressive drugs, and in those patients in whom these agents are contraindicated. Nevertheless, controlled clinical trials in are still warranted to clarify the role of RTX in IgAV.
BACKGROUND: Immunoglobulin A vasculitis (IgAV) is a systemic small vessel vasculitis for which treatment of severe cases is usually based on glucocorticoids and other conventional immunosuppressive drugs. The role of rituximab for resistant or refractory cases has been explored in isolated case reports and small series. AIMS: To perform a literature review of all pediatric and adult patients with IgAV treated with rituximab (RTX) and to assess disease characteristics, RTX efficacy and safety. METHODS: We conducted a systematic literature review according to PRISMA guidelines by selecting articles with information on IgAV and RTX up to October 2019. We extracted data on patient characteristics, disease course, RTX efficacy and tolerance. The resulting database was analyzed with statistical software package SPSS v 22.0. RESULTS: Among the initial 161 articles found, 20 studies including 35 well-characterized IgAV patients treated with RTX were finally analyzed. Distribution by sex was similar, and the median age at diagnosis was 26 (range: 2 months to 70 years). Patients included were equally diagnosed at pediatric age and in the adulthood. Almost 90% of patients had renal involvement before RTX treatment and resistant or refractory disease to glucocorticoids or other immunosuppressive agents, mainly with renal impairment, was the reason for RTX administration in 85.7% of patients. RTX was used because of contraindication to these previous agents in 8.6% of patients, and as first line therapy in 5.7% of them. With regard to RTX response, 94.3% of patients presented clinical improvement of any type and 74.3% achieved sustained remission at the end of follow-up. Among the 13 (37.1%) patients who experienced a disease relapse, 11 (31.4%) were treated with a new RTX dose, with good disease control in all cases. In terms of treatment requirements, glucocorticoids and additional immunosuppressants were significantly lower after RTX administration. No deaths were observed and the rate of minor RTX-associated adverse effects was of 8.6%. CONCLUSION: RTX seems to be a safe and useful agent in inducing disease remission and reducing previous immunosuppressive treatment in IgAV pediatric and adult patients resistant or refractory to glucocorticoids or other immunosuppressive drugs, and in those patients in whom these agents are contraindicated. Nevertheless, controlled clinical trials in are still warranted to clarify the role of RTX in IgAV.
Authors: I Hernanz; A B Larque; L F Quintana; G Espigol-Frigole; G Espinosa; A Adan; M Sainz-de-la-Maza Journal: Am J Ophthalmol Case Rep Date: 2021-04-22