RESUMO
INTRODUCTION: IgG4 related disease is a fibroinflammatory condition characterised by lymphoplasmocytic infiltration with predominance of IgG4+ plasma cells, fibrosis, and in most cases elevated IgG4 serum levels. It can affect any organ and result in varying clinical manifestations. Steroids are the cornerstone of treatment, however there is a high percentage of relapse. Recent studies have demonstrated favourable effects with rituximab. OBJECTIVE: To evaluate effectiveness related to the response to treatment with rituximab in patients with IgG4 related disease refractory to steroids and other immunosuppressant therapies. MATERIALS AND METHODS: We undertook a systematic search of the specialist databases EMBASE, LILACS, PUBMED and OVID-Cochrane for publications up until December 2017. RESULTS: After the quality analysis, we selected 27 articles (264 patients in total) for the final review, of which 23 were case reports and case series (105 patients), 3 were observational follow-up cohort studies (129 patients), and there was one clinical trial (30 patients). IgG4 related disease presents predominantly in male patients aged between 50 and 70 years on average. Multiple organs are compromised with an average of 3.5 compromised organs. Orbital, glandular and lymph-node compromise is most frequent. Patients in the different studies we included had received various treatments prior to starting rituximab, including glucocorticoids and disease-modifying anti-rheumatic drugs. There was 90.7% response in the cases where rituximab was used as second line therapy; rituximab was used as first line treatment for 10% of the patients with a 100% response rate. CONCLUSION: The use of rituximab for patients refractory to first-line treatments was associated with a high response percentage and less dependence on glucocorticoids.