Clustering plus eosinophilia, fibrosis predict relapse in eosinophilic fasciitis patients

Patients with eosinophilic fasciitis (EF) can be categorized into homogenous clusters that help predict relapse and sequelae, together with fibrosis and eosinophilia, suggests a study.

A retrospective multicentre study of EF patients was conducted from 2013 to 2019 to further explore the feature of EF. The authors used multivariate correspondence analysis to cluster patients and then sought prognosis factors.

Of the 128 patients who met the inclusion criteria, 69 (50 percent) had skin sclerosis and 71 (55 percent) had increased eosinophil count. On multivariate correspondence analysis, the authors identified three clusters, namely “mild,” “late-onset and hypereosinophilic,” and “fibrotic.”

One hundred nine patients were followed up for more than 1 year, of whom 49 (45 percent) presented a relapse and 48 (44 percent) had sequelae.

Multivariate analysis further showed that eosinophilia (hazard ratio [HR], 1.56; p=0.02) and fibrosis (HR, 4.02; p=0.002) significantly predicted relapse, while oedema (odds ratio [OR], 0.31; p=0.03), relapse (OR, 3.00; p=0.04), and fibrosis (OR, 1) significantly predicted sequelae.

Treatment changes after relapse included an increase in glucocorticoids in 40 patients (82 percent) and the addition of methotrexate in 31 (63 percent). These modifications resulted in clinical improvements and glucocorticoid withdrawal in 37 (76 percent) and 22 patients (45 percent), respectively.

The study was limited by its retrospective design.

“EF is an extremely rare disease with polymorphic presentation and prognosis,” the authors said.