Risk of intracranial hemorrhage in anti-neutrophil cytoplasmic antibody-associated vasculitis treated with glucocorticoids: a systematic review of case reports.

The aim of our study is to investigate risk factors, clinical characteristics, laboratory findings, treatment strategies, and outcomes of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) presenting with central nervous system (CNS) involvement, with a focus on intracranial hemorrhage (ICH). A systematic review was conducted using PubMed, Cochrane, and EMBASE databases (through October 2024). Data from 47 patients with AAV-associated ICH (derived from 46 reports), were pooled and analyzed. The results were stratified by AAV subtype and outcome, and logistic regression analyses were used to identify determinants of outcome. Among the 47 patients with AAV-associated ICH, eosinophilic granulomatosis with polyangiitis was the most common AAV subtype (48.9%), followed by granulomatosis with polyangiitis (29.8%) and microscopic polyangiitis (14.9%). Intraparenchymal hemorrhage was the most frequent presentation (55.3%). Most patients (85.1%) were diagnosed with AAV at the time of ICH presentation, and 57.4% had not received prior immunosuppressive therapy. Glucocorticoid use before ICH was associated with higher mortality (P < 0.001), while treatment with cyclophosphamide or rituximab improved survival (P < 0.001). Age was identified as a significant predictor of mortality (P = 0.022). In conclusion, glucocorticoid use before the occurrence of ICH may worsen bleeding severity in patients with AAV. These findings highlight the importance of glucocorticoid-sparing strategies in managing CNS involvement in AAV, with cyclophosphamide or rituximab showing potential improved survival.