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

Abstract：

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.

journal_name

Int J Rheum Dis

journal_title

International journal of rheumatic diseases

authors

Mango RL,Bugdayli K,Crowson CS,Drage LA,Wetter DA,Lehman JS,Peters MS,Davis MD,Chowdhary VR

doi

10.1111/1756-185X.13770

subject

Has Abstract

pub_date

2020-02-01 00:00:00

pages

233-239

issue

eissn

1756-1841

issn

1756-185X

journal_volume

pub_type

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