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

Robert L. Mango, Kubra Bugdayli, Cynthia S. Crowson, Lisa A. Drage, David A. Wetter, Julia S. Lehman, Margot S. Peters, Mark D. Davis, Vaidehi R. Chowdhary

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalInternational Journal of Rheumatic Diseases
DOIs
StateAccepted/In press - Jan 1 2019

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Fructose-Bisphosphate Aldolase
Immunosuppressive Agents
Confidence Intervals
Mycophenolic Acid
Hydroxychloroquine
Hypergammaglobulinemia
Therapeutics
Subcutaneous Tissue
Muscle Weakness
Azathioprine
Proportional Hazards Models
Methotrexate
Arthritis
Eosinophilic Fasciitis
Skin

Keywords

  • eosinophilia
  • eosinophilic fasciitis
  • sclerosing disorder
  • thickened skin

ASJC Scopus subject areas

  • Rheumatology

Cite this

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

In: International Journal of Rheumatic Diseases, 01.01.2019.

Research output: Contribution to journalArticle

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. / Baseline characteristics and long-term outcomes of eosinophilic fasciitis in 89 patients seen at a single center over 20 years. In: International Journal of Rheumatic Diseases. 2019.
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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.",
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T1 - Baseline characteristics and long-term outcomes of eosinophilic fasciitis in 89 patients seen at a single center over 20 years

AU - Mango, Robert L.

AU - Bugdayli, Kubra

AU - Crowson, Cynthia S.

AU - Drage, Lisa A.

AU - Wetter, David A.

AU - Lehman, Julia S.

AU - Peters, Margot S.

AU - Davis, Mark D.

AU - Chowdhary, Vaidehi R.

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Y1 - 2019/1/1

N2 - 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.

AB - 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.

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KW - sclerosing disorder

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