Background/Purpose: Eosinophilic fasciitis (EF) is a rare disease and there have been few published series that include clinical, pathological and serological data. These studies have provided disparate information on clinical course, with high rates of complete resolution and recurrence reported. Furthermore, no clear direction has emerged on the prognostic significance of features initially reported to be characteristic for EF.

Methods: We conducted a retrospective review of all EF patients evaluated at our institution from 1/1/1997 to 12/30/2016. Clinical records were reviewed to confirm the diagnosis of EF. Baseline demographic data, clinical, laboratory features at time of diagnosis and information on clinical course were collected. Kaplan-Meier methods were used to determine response and recurrence rates over time.

Results: We identified 83 patients with eosinophilic fasciitis seen within the study period. The median age of diagnosis was 53 years and the female to male ratio was 1:1. Majority of patients (90%) were diagnosed with combination of clinical features and biopsy, 6 (7%) were diagnosed based on clinical presentation alone, and 2 were diagnosed by clinical and imaging (MRI) features. Mean time to diagnosis from symptom onset was 7.9 months (range 1 – 45). Twenty-one patients (25%) reported exercise as a possible trigger. Groove sign was noted in only 24 (29%). Active inflammatory arthritis was noted in 9 patients at the time of initial EF evaluation, and only one of these had a known diagnosis of rheumatoid arthritis. Muscle weakness was described in 8 (10%), and EMG was interpreted as indicative of inflammatory myopathy in 6/17 (35%). The median absolute eosinophil count was 0.4-X 10⁹/ml and peripheral eosinophilia was noted in 37/74 patients (50%). Elevated erythrocyte sedimentation rate was seen in 16/70 (23%) and elevated CRP in 33/56 (59%). CK was elevated in 2/40 (5%), but aldolase was elevated in 9/33 (27%). High titer (≥1:320 or ≥ 3 units by ELISA) positive ANA was seen in 5/66 (8%). The median follow up was 2.3 years (range 0.2 – 18.5) among 46 patients with at least one follow up visit. Of those, 35 were initially seen within 1 year of diagnosis, and were included in prognosis analysis. By 3 years, 57% (95% confidence interval [CI] 32 -73%) had achieved a complete response with resolution of skin thickening. The rate of recurrence was 47% (95% CI 20 – 65%) at 3 years.

Conclusion: In a cohort of patients with EF defined by clinical diagnosis, rates of classic features in addition to, complete response and recurrence rates, are similar to those in previously published reports. To our knowledge, this is the largest retrospective cohort study of patients with EF.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/eosinophilic-fasciitis-baseline-retrospective-evaluation-of-clinical-characteristics-and-prognosis-in-a-cohort-of-83-patients/