Characteristics and factors associated with treatment response among patients with eosinophilic fasciitis: a systematic review and meta-analysis.

Background/objectives: Eosinophilic Fasciitis (EF) is a rare connective tissue disorder characterized by skin thickening. Few studies explored its characteristics and factors associated with treatment response.

Methods: PubMed, CENTRAL, Web of Sciences, and Scopus databases were searched in July 2023 to identify all published case reports of EF without restrictions on publication year or language. We extracted patients' demographics, clinical symptoms, laboratory findings, biopsy results and treatment outcomes.

Results: We analyzed 476 published case reports with an aggregate number of 597 patients. The mean age of the patients was 44.52, with a 1:1 male-to-female ratio. Comorbid autoimmune diseases were present in 13.3% of patients. The most common reported skin manifestation was induration (80.7%), with the most commonly affected body part being the legs (77.1%). Only 1.6% of patients had Raynaud's phenomena and 3.1% of patients had Dysphagia. Hypereosinophilia was present in 90.2% and hypergammaglobulinemia was present in 65.9%. Corticosteroids were received by 89.7% of patients. The majority of patients (82.2%) exhibited a clinical response to treatment, but 24.5% of patients who initially responded to treatment relapsed. Patients who received oral corticosteroid monotherapy were significantly more likely to respond to treatment compared to those who received other treatments (56.2% Vs. 39.4%). Patients who had forearm skin involvement (OR = 3.459; 95% CI: 1.334-8.966) had significantly higher odds of clinical response to treatment.

Conclusions: EF is a complex disease with non-specific symptoms. Our study offers comprehensive insights into patient characteristics and treatment outcomes, aiding clinicians in enhancing their approach.