Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China

Abstract

Background Central nervous system (CNS) involvement is extremely rare in eosinophilic granulomatosis with polyangiitis (EGPA), but is associated with a poor prognosis in the five-factor score. This study aims to elucidate the clinical features and independently associated factors of EGPA with CNS involvement. Results CNS involvement was observed in 17.3% (19/110) of EGPA patients from Peking Union Medical College Hospital between 2007 and 2019. We retrospectively reviewed their clinical data and analyzed the independently associated factors. Their mean age was 51.7 ± 11.56 years with no male/female predominance. Ischemic lesions were the most common manifestations, accounting for 63.2% of the 19 cases, followed by posterior reversible encephalopathy syndrome (36.8%), spinal cord involvement (15.8%), medulla oblongata involvement (15.8%), and intracranial hemorrhages (15.8%). Compared to the control group, patients with CNS involvement were of older age (51.7 ± 11.56 vs. 43.7 ± 13.78 years, p = 0.019) and had a higher ratio in the digestive tract involvement (52.6% vs. 28.6%, p = 0.042). Further multivariate analysis revealed that age, disease duration, and fever were the potential independent risk factors for CNS involvement of EGPA. Glucocorticoids combined with cyclophosphamide were the strategic therapy (94.7%). Intrathecal injections of dexamethasone and methotrexate were administered to 21.1% of the patients. Although seven patients relapsed during glucocorticoid reduction, seventeen patients finally achieved clinical remission. One patient died of acute intracerebral hemorrhage within one month, and another died of gastrointestinal perforation. Outcomes and cumulative survival show no significant differences between the two groups. Conclusions CNS involvement is uncommon in EGPA with various manifestations, and ischemic lesions are the most frequent. Age, disease duration, and fever are independent factors associated with CNS involvement in EGPA. The therapy of glucocorticoids combined with cyclophosphamide and intrathecal injections yields favorable responses. Acute intracranial hemorrhage and gastrointestinal perforation may be the principal causes of death.

Eosinophilic granulomatous polyangiitis with central nervous system involvement in children: a case report and literature review

“…Suying Liu et al conducted a study on 110 EGPA patients aged between 19 and 80, and found that 17.3% had involvement of the CNS. The most common manifestation was ischemic lesions (63.2%), followed by reversible posterior encephalopathy syndrome (PRES) (36.8%) (12). And age, disease duration, and fever are potential independent risk factors for CNS involvement.…”

“…Suying Liu et al. discovered that individuals with damage to the CNS are more prone to experiencing digestive tract involvement ( 12 ). The microbiota-gut-brain axis theory offers a potential explanation for this connection ( 25 ).…”

A fatal eosinophilic granulomatosis with polyangiitis case presenting intracerebral hemorrhage and thrombocytopenia

“…Peripheral nerve damage due to the disease is quite common, affecting 50% to 98% of patients, whereas central nervous system (CNS) involvement is rare, with an prevalence of only 4% to 17%. 4 The lesions predominantly present as cerebral infarction, reversible posterior circulation syndrome, spinal cord, or medulla involvement, and ischemic stroke is the most prevalent type. Intracerebral hemorrhage is extremely, rare with some isolated case reports; however, it is the leading cause of death due to CNS lesions in EGPA.…”

Hypertrophic Pachymeningitis Development in Eosinophilic Granulomatosis with Polyangiitis at Relapse of Disease: A Case-Based Review

“…The English literature describing EGPA complicated with HP is scarce, with eleven patients in four retrospective studies and four case reports. We excluded 2 articles because of inadequate information (André et al 2017;Liu et al 2021). In addition, we found 10 articles in the Japanese literature, and have summarized the 18 patients in Table 2 (Lio et al 2001;Maki et al 2001;Kanesaka et al 2005;Ito 2012;Furuya et al 2015;Iizuka et al 2015;Shibata et al 2018;Toda et al 2018;Aoyama et al 2019;Matsuura et al 2019;Nakano et al 2019;Sakairi et al 2019;Kobayashi et al 2020;Kurihara et al 2020;Shiraishi et al 2021;Izuka et al 2022).…”