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.

Autoimmune comorbidities and antibody markers associated with eosinophilic fasciitis

“…Mango et al 3 review of 89 patients with EF revealed 5.6% of patients with autoimmune comorbidities, similar to the 5.3% shown in our review. In comparison, while Raynaud's phenomenon was significantly reported in patients with EF (23.8%); we found autoimmune thyroid disease to be the most reported associated comorbidity (27.4%).…”

UVA‐1 phototherapy as adjuvant treatment for eosinophilic fasciitis: in vitro and in vivo functional characterization

“…Some authors considered EF as a deep form of localized scleroderma (LS): nevertheless, 28–65% of EF patients develop LS lesions 7,8,21,22 . The mRSS has been traditionally used to monitor the disease activity in SSc and LS patients and occasionally for EF patients 7–11,23–25 . However, the mRSS has multiple limitations, including the impossibility to identify slight alterations in skin thickness and to take into account high intraobserver/interobserver variability.…”

“… 7 , 8 , 21 , 22 The mRSS has been traditionally used to monitor the disease activity in SSc and LS patients and occasionally for EF patients. 7 , 8 , 9 , 10 , 11 , 23 , 24 , 25 However, the mRSS has multiple limitations, including the impossibility to identify slight alterations in skin thickness and to take into account high intraobserver/interobserver variability. We thus decided to perform both mRSS and LoSCAT indices at each examination: the LoSCAT appeared to be sensitive to patient improvements, whereas the mRSS did not exhibit a clear correlation.…”

“…Densitometric analysis of zymography gels. Supernatants protein content was evaluated with the Bradford method; 25 7.5% sodium dodecyl sulfate (SDS)-polyacrylamide gel (PAG) was copolymerized with 0.1% gelatin and 20 mg of protein of each sample was loaded into each well under nondenaturing conditions and run under a constant current (25 mA). After electrophoresis, the gel was rinsed in 2.5% Triton X-100 and 50 mmol/l Tris-HCl (pH 7.5) and then incubated overnight in an activation buffer (50 mmol/l Tris, pH 7.5, 0.15 mol/l NaCl, 10 mmol/l CaCl 2 , and 1% Triton X-100).…”

Eosinophilic Fasciitis of Wrists with a Positive Prayer Sign

“…In a new retrospective study of 89 patients, methotrexate was the most commonly used immunosuppressant in 79%, hydroxychloroquine in 45%, mycophenolate mofetil in 18%, and azathioprine in 8%. The response rate was similar for a single immunosuppressant agent [18]. The therapeutic response and prognosis are generally favorable.…”