Baseline characteristics and long‐term outcomes of eosinophilic fasciitis in 89 patients seen at a single center over 20 years

• Published in: International journal of rheumatic diseases Vol. 23; no. 2; pp. 233 - 239
• Main Authors: Mango, Robert L., Bugdayli, Kubra, Crowson, Cynthia S., Drage, Lisa A., Wetter, David A., Lehman, Julia S., Peters, Margot S., Davis, Mark D., Chowdhary, Vaidehi R.
• Format: Journal Article
• Language: English
• Published: England Wiley Subscription Services, Inc 01-02-2020
• Subjects: Adolescent; Adult; Aged; Arthritis; Azathioprine; Biopsy; Blood diseases; Child; Delayed Diagnosis; Diagnosis, Differential; eosinophilia; Eosinophilia - diagnosis; Eosinophilia - drug therapy; eosinophilic fasciitis; Fasciitis; Fasciitis - diagnosis; Fasciitis - drug therapy; Female; Follow-Up Studies; Forecasting; Hematological diseases; Humans; Hydroxychloroquine; Hypergammaglobulinemia; Immunosuppressive agents; Immunosuppressive Agents - therapeutic use; Inflammation; Leukocytes (eosinophilic); Male; Methotrexate; Methotrexate - therapeutic use; Middle Aged; Mycophenolate mofetil; Mycophenolic acid; Patients; Response rates; Retrospective Studies; sclerosing disorder; thickened skin; Treatment Outcome; Young Adult; eosinophilia; eosinophilic fasciitis; thickened skin; sclerosing disorder
• ISSN: 1756-1841; 1756-185X
• DOI: 10.1111/1756-185X.13770

Aim
Eosinophilic fasciitis (EF) is a rare, fibrosing disorder of skin and subcutaneous tissue. This study was undertaken to describe its clinical and laboratory features and identify prognostic factors associated with outcome.
Methods
We conducted a retrospective review of all EF patients evaluated at our institution from 1 January1997 to 30 December 2016. Kaplan‐Meier methods were used to determine treatment response rates over time. Potential associations between baseline characteristics and complete response were examined using Cox models adjusted for age and sex. Time‐dependent covariates were used to examine treatment effects.
Results
We identified 89 EF patients, with a female‐to‐male ratio of 1:1. Clinical features included groove sign in 26 (29%), peau d'orange/dimpling in 22 (25%), inflammatory arthritis in 9 (10%) and muscle weakness in 9 (10%). Aldolase was elevated in 11/36 (31%). Complete response rate was 60% (95% confidence interval [CI]: 35‐75) at 3 years. Diagnostic delay was inversely associated with treatment response (hazards ratio: 0.84 per 1 month increase; 95% CI: 0.73‐0.98). No baseline characteristics correlated with treatment response, but a trend toward positive association of elevated aldolase, hypergammaglobulinemia and presence of hematologic disorders was noted. Methotrexate was the most commonly used immunosuppressant in 79%, hydroxychloroquine in 45%, mycophenolate mofetil in 18% and azathioprine in 8%. No single immunosuppressant agent was associated with a superior response during treatment.
Conclusions
EF is characterized by relatively high response rates. Consensus diagnostic criteria, standardized management algorithms, and large prospective multi‐center cohorts are needed to develop an evidence‐directed approach to this challenging condition.