Cluster analysis reveals eosinophilia and fibrosis as poor prognostic markers in 128 patients with eosinophilic fasciitis.

BACKGROUND

Eosinophilic fasciitis (EF) is an extremely rare disease with polymorphic presentation and prognosis.

OBJECTIVE

To further investigate EF features.

METHODS

We performed a retrospective multicentre study of EF patients from 2013 to 2019, clustered patients using multivariate correspondence analysis, and sought prognosis factors.

RESULTS

One hundred twenty-eight patients were included. Sixty-nine (50%) patients had skin sclerosis, and eosinophil count was increased in 71 (55%) patients. Multivariate correspondence analysis identified 3 clusters: a "mild," a "late-onset and hypereosinophilic," and a "fibrotic" cluster. Of 109 patients who followed up for more than 1 year, 49 (45%) presented a relapse, and 48 (44%) had sequelae. Multivariate analysis revealed that eosinophilia (hazard ratio = 1.56; P = .02) and fibrosis (hazard ratio = 4.02; P = .002) were predictive factors of relapse, whereas edema (odds ratio = 0.31; P = .03), relapse (odds ratio = 3.00; P = .04) and fibrosis (odds ratio = 1) were predictive factors of sequelae. Following relapse, treatment modifications consisted of an increase in glucocorticoids in 40 (82%) patients and the addition of methotrexate in 31 (63%) patients. These modifications led to clinical improvement and glucocorticoid withdrawal in 37 (76%) and 22 (45%) patients.

LIMITATIONS: Retrospective study.

CONCLUSION

EF patients can be divided into 3 homogenous clusters, which, along with fibrosis and eosinophilia, are prognosis factors of relapse and sequelae.