A Report on the Clinical Efficacy of Rituximab Administration in Patients  with Inborn Errors of Immunity and Autoimmune/Autoinflammatory Manifestations

Samin Sharafian; Mahya Mohammadi; Samin Alavi; Mehrnaz Mesdaghi; Reza Shiari; Bibi Shahin Shamsian; Peyman Eshghi; Hedieh Haji  Khodaverdi Khani; Hassan Abolghasemi; Abdollah Karimi; Nasrin Behniafard; Parastoo Mollaei Tavana; Mohammad Mehdi Nasehi; Mozhgan Hashemieh; Mehran Khodashenas; Mahnaz Jamee; Zahra Chavoshzadeh; Narges Eslami

doi:10.18502/ijaai.v24i5.19743

Samin Sharafian Department of Allergy and Clinical Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Mahya Mohammadi Student Research Committee, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Samin Alavi Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Mehrnaz Mesdaghi Department of Allergy and Clinical Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Reza Shiari Department of Pediatric Rheumatology, Shahid Beheshti University of Medical Sciences, Mofid children hospital, Tehran, Iran
Bibi Shahin Shamsian Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Peyman Eshghi Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Hedieh Haji Khodaverdi Khani Department of Immunology, Faculty of Medical Science, Shahed University, Tehran, Iran
Hassan Abolghasemi Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Abdollah Karimi Pediatric Infections Research Center (PIRC), Research Institute for Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Nasrin Behniafard Children Growth Disorder Research Center, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
Parastoo Mollaei Tavana Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Mohammad Mehdi Nasehi Department of Pediatric Neurology, Pediatric Neurology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Mozhgan Hashemieh Department of Pediatrics Hematology/Oncology, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Mehran Khodashenas Student Research Committee, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Mahnaz Jamee Pediatric Nephrology Research Center, Research Institute for Children’s Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Zahra Chavoshzadeh Department of Allergy and Clinical Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Narges Eslami Department of Allergy and Clinical Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

DOI: https://doi.org/10.18502/ijaai.v24i5.19743

Keywords: Autoimmune diseases; Primary Immunodeficiency diseases; Rituximab

Abstract

It can sometimes be very difficult to control the manifestations of autoimmunity and lymphoproliferation in patients with primary immunodeficiency diseases, and there is no adequate response to first-line treatments. Rituximab (RTX), as a second-line treatment, is efficacious and well-tolerated for the management of these clinical manifestations.

This retrospective study was conducted to analyze the clinical, immunological, and genetic
findings together with the response rate to RTX therapy in subjects with inborn errors of immunity (IEI) and autoimmune or autoinflammatory manifestations. In this study, 23 individuals with IEI and autoimmune or lymphoproliferation manifestations who received RTX between April 2008 and 2021 were evaluated.

Fifteen out of the 23 patients were female. The median age of cases was 12 years. The moderate and severe adverse reactions, including fever, diarrhea, and anaphylaxis shock, were manifested during RTX infusion in 5 patients. In total, 86.9% of patients responded to rituximab (complete response: n=14, partial response: n=6) while three failed to respond. The median response time to RTX treatment was 50 days. All patients were given monthly intravenous immunoglobulin (IVIG) therapy. Pneumonia and candidiasis occurred in one patient a week after receiving the second injection of RTX. Eight patients expired during follow-up.

In conclusion, the response rate of RTX could be improved through administering monthly IVIG for hypogammaglobulinemia treatment following RTX infusion. Early use of rituximab leads to a better response rate in comparison with late use of rituximab in multitreated refractory patients. The efficient cumulative dose of rituximab remains undefined.